Medicine Quiz 1 


Medicine Quiz 1 


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Answered Review 


1. Question 


1 points 










An 85-year old woman who lives in a small rural Midwest town has always kept active despite her 
age, singing in her church choir, baby sitting her great-grandchildren, etc. However, for the past 
several months she has not felt well, not even able to muster sufficient energy to go to church on 
Sunday mornings, let alone go to choir practice. Also, she has problems sleeping, is extremely 
nervous with hand tremors, constantly anxious, sweats more than usual and can’t stand having the 
heat on, has chronic diarrhea, and has the sensation that her heart races. When she mentions 
some of these observations to her local primary care provider, he basically shrugs them off, saying 
that no one of her age can expect to feel as they did when younger. He was worried however by 
the fact her systolic pressure rose from an average of 125 mm Hg to 185 mm Hg at the time of her 
visit; he prescribed hydrochlorothiazide and suggested she return in 3 months for a follow-up 
examination. However, in the meantime, she started to have trouble with her eyes: she had double 
vision and they appeared to her to be reddish and swollen; they also were itchy. Consequently her 
granddaughter took her to an optometrist who practiced in a nearby town. After her initial 
examination, the optometrist became alarmed to such a degree that she arranged a visit to the 
Mayo Clinic in Rochester Minnesota. After giving her a thorough physical, the physicians there 
started her on a therapy with the aim of alleviating the underlying cause of her symptoms, including 
the problem with her eyes. Which of the following choices most probably describes an aspect of the 
treatment used to alleviate the underlying cause of her symptoms? 


1. Prescription of levothyroxine 

2. Injection of interferon-(31 a 

3. Watchful waiting 

4. Prescription of zolpidem 

5. Prescription of propylthiouracil >/ 


INCORRECT X 

The correct answer is 5. 

This lady has Graves’s disease and the optometrist recognized signs of Graves’s’ 
ophthalmopathy. Graves’s disease is a form of hyperthyroidism caused by an autoimmune 
reaction of unknown etiology. The core of treatment is to reduce the production of thyroid 
hormone by the gland. This can be done by antithyroid drugs, radioactive iodine treatment, or 
surgery. Usually, the first line of treatment is by drugs either propylthiouracil or methimazole 
is used. The problem with this lady’s eyes also needed to be treated with surgery to relieve 
the pressure behind the eyes caused by the infiltrating exophthalmos, which causes the 
swollen, bulging eyes very often associated with Graves’s disease. It is thought that this 
exophthalmus is due to common antigens shared by the tissues behind the eye and the 
thyroid gland; as a result exophthalmus is an almost 100% specific symptom for Graves’s 
disease. It is estimated that the incidence of Graves’s disease is about 30-80 cases per 
100,000 individuals, and it affects seven to eight females for every one male. Typically, the 
incidence peaks between the ages of 20-40 years. It is likely that this lady’s primary health 



care provider did not think Graves’s disease because she was so far outside of the “typical” 
range. Had the disease not been discovered and treated it would have continued to ravage 
her body, possibly even leading to irreversible blindness and/or death. 

(Choice 1) is incorrect. A prescription of levothyroxine would be used to treat a case of 
hypothyroidism, not hyperthyroidism. The most common cause of hypothyroidism is 
Hashimoto’s thyroiditis; in adult women, the incidence is about 3.5 per 1,000 persons per 
year, and in men it is about 0.8 cases per 1,000 persons per year (but the incidence in both 
sexes is much higher in the Appalachian region). Like Graves’s disease, Hashimoto’s 
thyroiditis is an autoimmune disease, but it causes hypothyroidism rather than 
hyperthyroidism; however, short intervals of hyperthyroidism may intervene, particularly at 
the initiation of the disease. It is usually diagnosed by the presence of signs and symptoms 
of hypothyroidism (such as weight gain, sluggishness, sensitivity to cold, and depression), 
and confirmed by measuring the thyroid-stimulating hormone (TSH) level and presence of 
autoantibodies against various thyroid antigens. Chronic Hashimoto’s can also cause goiter. 
Interferons are a class of cytokines produced by many cells in response to double stranded 
RNA. They may aid the immune response by inhibiting viral replication within infected cells; 
they may also activate natural killer cells and macrophages and facilitate antigen 
presentation to lymphocytes. 

(Choice 2) is incorrect. Although interferons (3 la and (3 1b have anti-autoimmune activity 
that has made them useful in treating multiple sclerosis they have no known function in 
treating Graves’s disease. 

(Choice 3) is incorrect. Watchful waiting is defiantly not an acceptable treatment for 
Graves’s disease; morbidity will only progress with time, perhaps even resulting in death. 
(Choice 4) is incorrect. Zolpidem is used to treat insomnia. Although it might be used to treat 
the insomnia associated with hyperthyroidism, it will not alleviate the underlying cause of her 
symptoms. 


2. Question 1 points 

A 67-year-old man presents with headache, vomiting, blurred vision, difficulty with speaking and 
swallowing, and loss of balance. He also complains of numbness in his face. His past history is 
significant for diabetes mellitus and hypertension. The patient presently is on metformin for the 
former and a combination of an angiotensin converting enzyme (ACE) inhibitor and diuretic for the 
latter. The acute problem occurred approximately 2 hours prior to being seen in the emergency 
room. His blood pressure is 150/100 mm Hg, his pulse rate is 80/min with a few irregular beats, and 
his temperature and respiratory rate are normal. The patient is conscious, the left pupil is smaller 
than the right, and the eyeball appears to be sunken into the orbit. He also has partial closure of the 
left upper eyelid. He has no pain sensations over the left side of the face to pinprick. However, 
touch is preserved. His gag reflex is depressed, and the palate is noticeably lowered on the left 
side, with the uvula being pulled to the right when he was asked to say, “aaah.” There was a 
noticeable nystagmus to the left, and he has poor coordination of his left upper and lower 
extremities. His gait is ataxic. When tested with a pin, he cannot feel pain in the right upper and 






lower extremities. However, the sensation of touch is preserved. A noncontrast computerized 
tomography (CT) of the brain was normal. Which of the following is the best means of immediate 
medical management in this patient? 


1. Heparinization and observation 

2. Right carotid endarterectomy 

3. Left carotid endarterectomy 

4. Thrombolytics 

5. Antiplatelet agents 


INCORRECT X 

The correct answer is 4. 

This patient most likely has had an infarction involving the posterolateral portion of the 
medulla oblongata as a result of blockage of the posterior inferior cerebellar artery (PICA) on 
the left side. The condition is also known as Wallenberg syndrome, which might also be 
caused by occlusion of the vertebral artery. The computerized tomography scan shows no 
evidence of hemorrhage, and therefore the diagnosis of ischemic infarction is appropriate. 
Since his condition has been present for less than 3 hours, it is prudent to use a thrombolytic 
agent (Choice 4) as the initial medication. In the thrombolytic system, plasminogen is 
activated into plasmin by tissue plasminogen activator (tPA); plasmin then further decreases 
fibrin into degraded peptides. Plasminogen activator must be administered within 3 hours of 
the onset of the acute event to be efficacious. It is contraindicated if the blood pressure is 
sustained above 185/110. No other antithrombotic treatment should be instituted for 24 hours. 
(Choice 1) followed by warfarin is appropriate in the management of a patient with a non- 
hemorrhagic stroke and would have been the choice had it been more than 3 hours after the 
onset of the acute event. 

(Choices 2 & 3) Right or left carotid endarterectomy are incorrect, because the problem 
involves the vertebral circulation. 

(Choice 5) Antiplatelet agents, such as aspirin and clopidogrel, are not used in the initial 
management of infarctions. They have a role to play in the prevention of strokes. 


3. Question 1 points 

A 60-year-old man presents to the emergency room with a history of vomiting, increasing 
confusion, hearing voices, and blurred vision. The patient provided a history of congestive cardiac 
failure and reported he is being treated with an unnamed diuretic, enalapril, carvedilol, and digoxin. 
His blood pressure is 140/95 mm Hg; pulse, 88/min irregular; respirations, 22/min; and body 
temperature is normal. Physical examination confirmed altered mental status, an irregular cardiac 




rhythm to auscultation, and basilar rales on the left, but there is no cyanosis or pallor. The 
electrocardiogram shows left axis deviation and paroxysmal atrial tachycardia with block. A chest 
x-ray film confirms left ventricular hypertrophy with blunting of the left costophrenic angle and 
diffuse shadowing at the base. Effective management of cardiac dysfunction would include which 
one of the following? 


1. Raising the serum sodium level 

2. Raising the serum magnesium level 

3. Lowering the serum magnesium level 

4. Raising the serum calcium level 

5. Raising the serum potassium level / 


INCORRECT X 

The correct answer is 5. 

This patient has symptoms of digitalis toxicity in addition to cardiac failure. Digitalis toxicity 
leads to vomiting, increasing confusion, hallucinations, photophobia, yellow vision, and 
cardiac arrhythmias that could be potentially dangerous if left untreated, because 
supraventricular tachycardia (SVT) and atrioventricular (AV) block could supervene, or even 
worse, ventricular tachycardia and fibrillation. The presence of SVT and AV block signifies 
cardiotoxicity. Digitalis toxicity may be precipitated by hypokalemia, and therefore, the serum 
potassium level should be increased, maintaining it between 4.0 and 5.0 mmol/L. There is a 
strong possibility the patient is on a loop diuretic (such as furosemide), which restricts 
sodium and chloride reabsorption in the proximal part of the ascending loop of Henle, with 
resultant excretion of sodium, water, chloride, and potassium. Digitalis administration should 
be discontinued temporarily. The serum digitalis level does not correlate with digitalis toxicity. 
(Choice 1) will have no effect on the heart. Sodium has its primary effects on the brain and 
not the heart. 

(Choice 3) would aggravate the problem further, as one of the causes of digitalis toxicity is 
hypomagnesemia. 

(Choices 2 & 4) Raising the serum calcium level would be disastrous, as hypercalcemia 
promotes digitalis toxicity. Although hypomagnesemia could result from use of loop diuretics, 
this effect is less common than that of hypokalemia; therefore, raising the serum magnesium 
level is not indicated. Other causes of digitalis toxicity include hypothyroidism, myocardial 
ischemia, advancing age, renal insufficiency, volume depletion, and concomitant use of 
drugs that delay elimination of digitalis, such as amiodarone, verapamil, (3-blockers, such as 
carvedilol (which has been prescribed to this patient) and quinidine, a cinchona alkaloid that 
is used to treat cardiac arrhythmias. Although with coadministration of angiotensin-converting 
enzyme (ACE) inhibitors, such as enalapril, digitalis toxicity can potentially induce 
hyperkalemia, in the case presented, the electrocardiogram did not show evidence of 
hyperkalemia (peaked T waves); if it did, one would consider lowering the serum potassium 
level. 



4. Question 1 points 

A 69-year-old man with a known history of coronary artery disease (CAD), atrial fibrillation, and 
hypertension presents with sudden onset of right facial weakness and numbness. He also 
complains of a roaring in the right ear. On examination, he has some difficulty with speech. He does 
not have a pronator drift, and his grip strength is normal. However, there is weakness of the face on 
the right side, including the orbicularis oculi. He is unable to appreciate taste on the anterior tongue 
on the right side. He has normal sensations on the face to touch and pinprick. Which of the 
following would best explain these findings? 


1. Upper motor neuron lesion 

2. Brainstem glioma 

3. Left middle cerebral artery embolus 

4. Hemorrhage within the left internal capsule 

5. Lower motor neuron lesion V 


INCORRECT X 

The correct answer is 5. 

This patient has a lower motor neuron lesion affecting cranial nerve VII—the facial nerve (CN 
VII) on the right side—and is also known as Bell’s palsy. Facial palsy is the most common of 
the cranial neuropathies. Most cases are due to infection with herpes simplex virus and are 
not idiopathic, as was believed earlier. The facial nerve innervates the muscles of the face 
and the stapedius muscle in the ear and conveys taste fibers to the anterior two-thirds of the 
tongue, via the chorda tympani nerve. The roaring in the ear (hyperacusis) and lack of taste 
in the anterior portion of the tongue are due to involvement of the aforementioned innervation. 
The problem with speech is due to dysarthria, resulting from paralysis of the muscles around 
the mouth. The inability to close his eye can put his cornea, and indeed the eye, in danger. 
Hence, the eyelid should be taped shut or covered with an eye patch. The facial nerve also 
supplies the lacrimal gland; absence of tears could result in xerophthalmia (corneal drying) 
and attendant complications, hence artificial tears are necessary. Corticosteroids are helpful. 
The disorder usually resolves over time. Other causes of lower motor neuron facial paralysis 
include lesions in the brainstem, cerebellopontine angle, middle ear infection, multiple 
sclerosis, human immunodeficiency virus (HIV) infection, Lyme disease, parotid tumor 
tumors, diabetes mellitus, and trauma to the facial nerve. 

(Choice 1) An upper motor neuron lesion of the facial nerve will spare the upper half of the 
face because of the bilateral innervation to that region. An upper motor neuron VII nerve 
paralysis is due to involvement of the contralateral motor neurons of the descending 




corticobulbar pathway or of the pathway used en route to the seventh cranial nerve nucleus 
in the pons. 

(Choice 2) is unlikely, given the sudden nature of the event, lack of symptoms such as 
headache, nausea and vomiting, and bulbar signs involving the lower cranial nerves. These 
patients could have ataxia as well. 

(Choice 3) could very well occur given his history of atrial fibrillation. However, embolism 
involving the middle cerebral artery would be associated with headache, confusion, right 
hemiparesis or hemiplegia, and an upper motor neuron facial paralysis on the right as well. 
(Choice 4) could occur, given the history of hypertension in this patient. However, the patient 
would have severe headache, obtundation, and a dense right hemiplegia, together with a 
right upper motor neuron facial nerve paralysis. 


5. Question 1 points 

A 55-year-old woman presents to the emergency room with a history of severe crushing 
retrosternal chest pain while she was moving some furniture around the house. 

Electrocardiography shows elevated ST segments in leads II, III, and aVF, consistent with an 
inferior myocardial infarction. This diagnosis is further supported by cardiac enzymes that test 
positive. The patient is hemodynamically stable. She is moved to the intensive care unit. A few 
hours later, she developed tachyarrhythmia. Treatment with intravenous (IV) boluses of lidocaine 
controls the arrhythmia only transiently—the arrhythmia disappears within 1 minute only to 
reappear within 4 minutes of each bolus dose. Plasma levels of lidocaine measured soon after each 
injection remained within the therapeutic range (1-5 mg/L). Which of the following statements is 
most accurate? 


1. Rapid metabolism of lidocaine is responsible for the short duration of its 
antiarrhythmic action after the bolus administration. 

2. Laboratory determinations of blood levels of lidocaine after IV administration are 
frequently in error. 

3. The elimination half-life of lidocaine is approximately 2 minutes. 

4. Lidocaine rapidly redistributes from blood to other tissues. 

5. Cardiac cells rapidly develop tachyphylaxis to lidocaine. 


INCORRECT X 

The correct answer is 4. 

Rapid (but temporary) control of arrhythmia follows achievement of a therapeutic blood level 
of lidocaine. The temporary nature occurs because lidocaine rapidly redistributes from blood 
to other tissues. In this situation, the reappearance of the arrhythmia reflects the rapid 





distribution of lidocaine from the blood to highly perfused body tissues, resulting in a 
decrease in plasma concentration of the drug below therapeutic levels. A similar process, 
involving redistribution of thiopental from the brain to other highly perfused tissues, is 
responsible for termination of the anesthetic effects of the intravenous (IV) barbiturate. 
(Choices 1 & 3) Although the liver metabolizes lidocaine extensively, the elimination half-life 
of the drug is 1-2 hours (not 2 minutes), which cannot account for such a rapid reappearance 
of the arrhythmia. 

(Choice 2) While the blood levels following IV administration of lidocaine may be variable, 
this is due to redistribution processes and not to errors in laboratory determination of drug 
levels. 

(Choice 5) There is no evidence that cardiac cells are capable of rapid (and reversible) 
changes in sensitivity to any antiarrhythmic drug. Conversely, the lidocaine level may be 
increased in patients taking cimetidine, and the dosage needs to be reduced in patients with 
congestive cardiac failure or liver disease. 


6. Question 1 points 

A 58-year old male computer programmer presented to his physician for a routine check-up after 
his wife nagged him into it. This was his first visit to a physician in at least a decade. After work, he 
would come home, eat dinner, watch television, and go to bed. He spent his weekends watching 
sports on television. While doing so, he often ate peanuts, buttered popcorn, or potato chips and 
downed it with a couple of beers. When asked if this had always been his lifestyle, he replies that 
prior to turning 40 he played sports and enjoyed dancing with his wife. His subsequent physical 
examination and laboratory work did not uncover any abnormalities with his heart, lungs, thyroid 
hormone levels, or kidney function. On the other hand, three different blood pressure readings 
taken by both the physician and his assistant obtain similar results, namely, an average reading of 
178/92 mm Hg. He is 5 feet, 9 inches (1.75 m) tall and weighs 204 pounds (92.5 kg). A fasting 
blood sugar and lipid profile show a glucose level of 124 mg/dl_, total cholesterol (TC) level of 234 
mg/dL, and low-density lipoprotein (LDL) level of 158 mg/dl_. The most appropriate first line of 
management in this patient would be which of the following? 


1. Gastroplasty 

2. Prescription of an anorexic drug 

3. Prescription of gemfibrozil 

4. Persuade him to take his wife out dancing at least twice a week. 

5. Prescription of a dietary supplement containing ephedra 

6. Prescription of tolbutamide 







INCORRECT X 

The correct answer is 4. 

This patient is in the early stages of metabolic syndrome and increases insulin resistance, 
which, when fully developed, is characterized by type 2 diabetes, dyslipidemia, hypertension, 
and obesity. Although only tested once, at this time, it is highly probable that he is in a pre¬ 
diabetic state, which is defined as having fasting plasma glucose levels between 100 and 
126 mg/dL on two consecutive days. His fasting total cholesterol (TC) level is also in the 
borderline high range. (For TC, the normal value is defined as equal to or less than 200 
mg/dL [5.2 mmol/L], borderline is between 200 and 240 mg/dL [5.2-6.2 mmol/L], and elevated 
as equal to, or greater than 240 mg/dL [6.2 mmol/L]. Whereas the new American Heart 
Association definitions for LDL levels are: optimal less than 100 mg/dL [2.5 mmol/L], near 
optimal 100-129 mg/dL [2.5-3.3 mmol/L], borderline high 130-159 mg/dL [3.4-4.0 mmol/L], 
high value 160-189 mg/dL [4.1-4.8 mmol/L], and very high 190 mg/dL [4.8 mmol/L] and 
above.) In addition, he is definitely hypertensive (systolic/diastolic values reported as mm Hg 
are defined as normal, <120/80; pre-hypertension, 120/80 to 139/89; stage 1 hypertension, 
140/90 to 159/99; and stage 2 hypertension, > 160/100). In summary, he is well on his way to 
becoming a full-blown diabetic, with a high blood glucose level as well as high LDL and total 
cholesterol levels, and also is liable soon to be a stage 2 hypertensive and a candidate for 
severe coronary artery disease (CAD). The primary driving force for these conditions is his 
obesity, created by his lifestyle. His body mass index (BMI) is 30.2 kg/m 2 , calculated using 
the following formula: 

BMI = weight in kg divided by height in meters squared, where 1 kg = 2.2046 lb, and 1 m = 
39.37 inches. 

A BMI between 25 and 29.9 kg/m 2 is defined as overweight, and 30 kg/m 2 or greater as 
obese. The first step in managing obesity in this patient is to get him off his couch and 
moving about. Taking his wife out dancing at least twice a week, while not a solution in itself 
would be a step in the right direction, and it might be easier to persuade this obviously 
recalcitrant man to do this, which may also please his wife, than to initially recommend the 
more stringent dietary and exercise regimes that will be required. In the interim, it also may 
be possible to persuade him to return to treat his hypertension and incidentally better deal 
with his lifestyle issues, including the possibility of depression. 

(Choice 1) will cause him to lose weight. However, it requires surgery and has concomitant 
short and long-term risks. As a consequence, it should be reserved only for the morbidly 
obese. 

(Choice 2) Although short-term weight loss is enhanced by the prescription of an anorexic 
drug, patients taking such agents usually end up even heavier than before they started 
treatment because of a rebound effect. Management in this case clearly entails a lifestyle 
change, which may require long-term treatment and a great deal of patience. 

(Choice 3) One would prescribe gemfibrozil as a way to lower triglyceride levels only after 
they were acutely high and more conservative measures failed. 

(Choice 5) A dietary supplement containing ephedra would not be a wise move since 
several studies have shown that it can cause serious reactions including hypertension, 
which certainly would not be recommended in this case. Ephedra (also known as ma huang) 
contains the chemical ephedrine, which was used as an asthma medication until the 1980s, 
when it was taken off the market because of its dangerous effects on the heart and blood 


pressure. Tolbutamide is a first-generation sulfonylurea, an oral hypoglycemic agent. Not 
only are more effective agents available, but also tolbutamide, like all sulfonylureas, runs the 
risk of inducing hypoglycemia and promotes weight gain. 

(Choice 6) The physician would not likely prescribe tolbutamide. Until recently, prediabetes 
would only be treated by lifestyle changes, diet, and exercise. However, it now is deemed 
acceptable to be more aggressive and prescribe an oral hypoglycemic agent. Metformin is 
often recommended because it neither causes hypoglycemia nor promotes weight gain. 


7. Question 1 points 

A 44-year-old woman consulted her primary care physician because of a nagging feeling of fatigue, 
pain, and stiffness in both hands upon rising; the pain persisted for at least 1 hour after arising and 
was severe enough to make getting herself ready for the day’s work difficult. The physician found 
that she had a low blood count, an elevated sedimentation rate, and an elevated C-reactive protein 
level, but x-rays of her hands were essentially normal. Feeling that she may have some sort of 
autoimmune condition, he referred her to a rheumatologist, who immediately had her haplotype 
determined and ran immunological tests for rheumatoid factor and an anti cyclic citrullinated peptide 
antibody (ACPA) test, as well as testing for antinuclear antibodies (ANAs). Her haplotype serotype 
was HLA-DR4, and she tested positive for ANA and ACPA and negative for rheumatoid factor. 

Most likely, this lady suffered from which one of the following conditions? 


1. Systemic lupus erythematosus 

2. Multiple sclerosis 

3. Sjogren syndrome 

4. Diabetes mellitus type 1 

5. Rheumatoid arthritis >/ 


INCORRECT X 

The correct answer is 5. 

The lady has the early symptoms of rheumatoid arthritis (RA). RA is an autoimmune 
inflammatory condition principally involving the synovial membranes of multiple joints, 
typically in a symmetrical bilateral fashion starting with the smaller joints on the hands. As the 
disease progresses, typically with periods of remission, it affects the feet and bigger joints, 
and causes malaise, weight loss, and fever. Although in this case the lady’s symptoms and 
early laboratory studies are nonspecific, they are typical for some sort of inflammatory 
autoimmune condition. Moreover the HLA-DR4 haplotype serotype is strongly positively 
correlated with RA and a positive anticyclic citrullinated peptide (ACPA; also known as 
citrulline antibody, anticitrulline antibody, anti-CCP, and cyclic citrullinated peptide antibody) 







test. This test measures a form of serum citrulline, and some investigators hypothesize that 
the conversion of arginine to citrulline plays a role in the autoimmune inflammatory process 
associated with RA. It has a specificity of about 95% for RA, although it does provide false¬ 
negative results about 33% of the time; in addition, it often is present in the earliest phases of 
the disease, even before clinical signs are clearly manifested. In contrast, the negative 
rheumatoid factor result does not rule out RA because it is frequently negative in early 
cases, even after the first year. ANA is a relatively nonspecific test for autoimmune and other 
inflammatory conditions; therefore, a positive test is compatible with RA but also other 
disease states. The prevalence of RA is about 1 case per 100 persons; females are affected 
three times more often than males, and the onset in most cases occurs between the ages of 
25 and 50, peaking between the ages of 40 and 50 years. Recent treatments for RA have 
improved the lives of RA patents to a remarkable extent. Although the classic medications, 
corticosteroids and/or nonsteroidal anti-inflammatory drugs (including aspirin), are still 
employed to reduce pain and inflammation, so-called disease-modifying antirheumatic drugs 
(DMRDs) are now available and often prescribed early in the course of the disease to avoid 
disease progression to the point of causing irreversible damage. In addition, genetically 
engineered drugs called biologies have been developed that induce remissions in about two- 
third of cases. Unfortunately, these have to be administered by injection and are very 
expensive, thus they are often reserved for people in whom DMARDs are ineffective, or they 
are used to supplement DMARD treatments. Physical therapy is also an important adjunct 
used to help joint function. 

All the incorrect choices also are autoimmune diseases. 

(Choice 1) Systemic lupus erythematosus (SLE) is a chronic inflammatory systemic 
disease that can affect any organ system. ANAs are present in the serum of most patients 
with active SLE, and antibodies to native double-stranded DNA are essentially diagnostic for 
this disease. A genetic component is also likely, since HLA-DR2 and HLA-DR3 human 
leukocyte antigens are more common than in the general population. SLE is erratic in its 
manifestations in that it follows a relapsing and remitting course. The incidence is highest 
among women of childbearing age, and overall in adults, it is 10-15 times higher in women 
than in men. Cases of SLE vary from being relatively benign to being progressive and rapidly 
fatal. Prior to 1955, the 5-year survival was less than 5%, but now thanks to better medical 
care, the 10-year survival is about 90%. Nonetheless, 30% of SLE-related deaths occur 
before the age of 45 years. 

(Choice 2) Multiple sclerosis (MS) is a disease in which the immune system attacks the 
myelin sheets in the central nervous system, killing oligodendrocytes and causing 
demyelination. This may produce numerous physical and mental symptoms, depending on 
the nerves affected, and it often progresses to physical and cognitive disability. Disease 
onset usually occurs in young adults, and it is more common in women. It has a prevalence 
ranging between 2 and 150 per 100,000 depending on the country or specific population; it is 
more frequent among individuals of northern European descent. Although the condition is not 
curable, recently developed medications help mitigate symptoms; the type of medication 
used varies with the variant form of the disease. 

(Choice 3) Sjogren syndrome occurs when the immune system attacks and destroys 
moisture-producing organs, including the salivary and lacrimal glands and more rarely the 
lungs, kidneys, or gastrointestinal tract. It usually is characterized by dry eyes and mouth, 
and sometimes by enlargement of the parotid gland. More than 90% of individuals affected by 






Sjogren syndrome are women, and 90% of the time it is rheumatoid factor positive; it also 
may be associated with rheumatoid arthritis or other rheumatic diseases. Serum levels of 
ANA are also often elevated. Some people may experience only mild symptoms of dry eyes 
and mouth and are able to treat the disease symptomatically, while others have a more 
severe disease and must cope with blurred vision or recurrent oral infections and difficulties 
with swallowing and eating. Systemic problems can also occur including fatigue, joint pain, 
and even autoimmune tubulointerstitial nephritis leading to proteinuria, urinary concentrating 
defect, and distal renal tubular acidosis. 

(Choice 4) Unlike diabetes mellitus type 2, type 1 is a disease with an autoimmune 
component triggered by some still not clearly identified factor. Approximately 85% of patients 
have circulating islet cell antibodies, and the majority also has detectable anti-insulin 
antibodies before receiving insulin therapy. Most islet cell antibodies are directed against 
glutamic acid decarboxylase within pancreatic B cells. Some theorize that there is a virally 
induced etiology. The Coxsackie virus is commonly suggested, although the evidence is 
inconclusive. Since not everyone infected by this organism gets diabetes type 1, it also is 
suggested that, in addition, there is a genetic component; evidence for this is provided by the 
fact that up to 95% of patients with type 1 diabetes express human leukocyte antigen (HLA) 
DR3 or HLA-DR4; in fact HLA-DQs are considered specific markers of type 1 DM 
susceptibility. Others claim cow’s milk contains a triggering agent, which however never has 
been identified; this idea is derived from studies claiming a higher incidence among babies 
given cow’s rather than breast milk after birth. 


8. Question 1 points 

A man married a young woman who lost her vision at the age of 28 years. They had three children, 
two girls and one boy, all of whom started to become blind after the age of 20. All three of these 
children married sighted persons and had children. One daughter had two boys and a girl, all of 
whom became blind. The other daughter had a boy and girl; both of them lost their vision as well. 
The son had three boys and a girl, all of whom were sighted and had excellent vision as long as 
they lived. In the third generation, all the boys who remained sighted and those who lost their vision 
early married women who remained sighted until they died, and all their offspring also remained 
sighted well into old age. The daughter of the second-generation male who retained her sight 
married a normally sighted man, and their offspring could see throughout their lives as well. The 
second generation women who became blind married men who retained their sight but their 
offspring of either sex lost their sight at an early age. Which of the following inheritance patterns is 
most likely to be present? 


1. Autosomal dominant 

2. Autosomal recessive 

3. Mitochondrial 

4. X-linked dominant 


5. X-linked recessive 


INCORRECT X 

The correct answer is 3. 

In this pedigree, all affected females transmit a trait that causes early vision loss to offspring 
of both sexes. However, affected males and normal individuals of both sexes do not transmit 
loss of sight to their offspring. This non-Mendelian pattern of inheritance is characteristic of 
conditions caused by a mutation in a mitochondrial gene, due to the fact that only females 
transmit mitochondria to the ovum. This particular family has Leber’s hereditary optic 
neuropathy. Individuals who inherit this condition suffer precipitous vision loss, usually in 
their 20s, due to optic nerve degeneration. In different families with Leber’s hereditary optic 
neuropathy, 11 different missense mutations in three different mitochondrial genes encoding 
respiratory chain subunits have been described. Although the ultimate phenotype, loss of 
vision, remains consistent, the rate of vision loss and the age of onset vary considerably, 
even among members of the same family. Such variability in the phenotypic expression of 
conditions transmitted by mitochondrial mutations is characteristic of these conditions and is 
called heteroplasmy. It occurs because each cell carries multiple mitochondria, of which on 
average only half will be affected, but transmission of mitochondria with normal and mutated 
genes will occur in a random manner. Because mitochondrial-DNA (mtDNA)-encoded 
proteins are primarily associated with electron transport and adenosine triphosphate (ATP) 
synthesis, tissues that depend upon the electron transport system are affected most. 
(Choice 1) In an autosomal dominant disorder, either an affected male or female may 
transmit the mutant gene to a child of either sex, or the mutant trait will be expressed. 
(Choice 2) In an autosomal recessive disorder, both parents must transmit a mutant gene to 
the gamete for the trait to be expressed. An offspring of either sex who inherits one mutant 
gene from either parent will be a carrier. 

(Choice 4) In an X-linked dominant disorder, affected males will carry only one X 
chromosome, which will bear the mutated gene; this X chromosome will be transmitted to his 
daughters, in whom the trait is expressed since it is dominant. However, characteristically, 
the disease is more severe in hemizygous affected males than in heterozygous affected 
females. Since his sons only will inherit his Y chromosomes, they will not be affected, nor will 
they be carriers. Affected females almost certainly will have one X chromosome with the 
normal gene and one with the mutated gene; thus, they transmit the mutant gene to 50% of 
their daughters and to 50% of their sons. Since the trait is dominant, the mother as well as 
offspring of both sexes who inherit the affected chromosome will express the trait. 

(Choice 5) In an X-linked recessive disorder, males carrying an X chromosome with a 
mutated gene will express the trait since they only have one X chromosome; females 
carrying an X chromosome on the other hand will be carriers but will not expresses the trait 
since the recessive trait will not be expressed in the presence of an X chromosome carrying 
the normal gene. Sons of affected males will be neither carriers nor affected because they 
inherit a Y, not an X chromosome; however, 100% of the daughters will be carriers because 
they will receive the affected X chromosome from their father and a normal one from their 
mother. Fifty percent of the daughters of women who are carriers will become carriers 
themselves, since there is a 50% chance they will inherit the affected X chromosome. 


Although there is also a 50% chance that her son will inherit an X chromosome bearing the 
mutated gene, those who do will express the disease because that will be their only X 
chromosome. 


9. Question 1 points 

A 57-year-old woman has had a rather remarkable history. She was born to a poor immigrant family 
and worked her way through college as a bartender in a small night club where patrons enjoyed a 
“good smoke” along with their drinks. After college, she went to law school, passed the bar, got a 
job in a good law firm, and became a partner within a decade. Moreover, she married and has two 
children. All her life she has been active and full of energy, but about 6 months ago she began to 
fatigue early; 2 or 3 months ago, she noticed a shortness of breath and a loss of appetite and of 
weight. Several weeks ago, she developed a dry cough, and last week, she spat up sputum with 
streaks of blood. As a nonsmoker, she though herself essentially immune to lung cancer; however, 
the blood in her sputum alarmed her and she consulted a pulmonologist. He took a history, gave 
her a general physical examination, and took chest x-rays followed by a computed tomography 
(CT) scan. He reported back to her that he had both bad and good news: “The bad news is that you 
have lung cancer; the good news is that the cancer is located in a defined area near the center of 
the lung but only in the left lobe and at the root of the bronchus; this means it seems not to have 
spread and is a relatively slow-growing variant; this provides hope it can be successfully treated.” 
Which of the following lung cancer variants does this lady most likely have? 


1. Adenocarcinoma 

2. Squamous cell carcinoma 

3. Bronchoalveolar lung cancer 

4. Large-cell carcinoma 

5. Small (oat) cell cancer 


INCORRECT X 

The correct answer is 2. 

Lung cancer is the 5th most common cancer in Australia for both sexes. However, it is by far 
the leading cause of cancer death among both men and women. It is estimated that in 2011 
there were 10,511 new cases resulting in 8,137 deaths from lung cancer. More people will 
die from lung cancer than from colon, breast, and prostate cancers combined. Typically, lung 
cancer is divided into two major categories, small-cell and non-small cell. This is because 
small-cell cancers grow rapidly, metastasize readily, and are scattered throughout the lung, 
making them essentially impossible to treat by surgery or radiation. On the other hand, 
before they mature and spread, non-small cell cancers tend to remain concentrated in a 



localized area, making surgical or radiation treatment a possibility. Among the non-small cell 
lung cancers are adenocarcinomas, squamous cell carcinomas, bronchoalveolar lung 
cancers, and large-cell carcinomas. Squamous cell carcinomas are generally located in a 
defined area near the center of the lung, either on a bronchial branch or on a nearby major 
lobe; this matches the description of the cancer the case above. These cells are situated 
near the terminus of the bronchi; consequently, they are particularly susceptible to airborne 
irritants and carcinogens and cancers in this area are typically caused by smoking tobacco 
products. However, carcinogens do not have to be smoked in order to be breathed in, as for 
instance is the case of second-hand smoke, which likely happened to this lady while she 
worked as a bartender. Typically, the progression from early injury is one that takes years to 
achieve; cells progress from normal, to hyperplasia, through metaplasia and dysplasia, finally 
into a true neoplastic state, a carcinoma. In this lady, the original exposure caused a 
mutation, and the progression may well have been promoted over the years by further 
exposure, likely during conferences, etc. Prior to the popularity of filter-tipped cigarettes, 
squamous cancers were the most common type of lung cancer, and they still constitute 
25%-30% of all lung cancers. If caught in time, as it appears it may have been in the case 
described, squamous cell carcinomas are possibly treatable by surgery and/or radiation; 
however, they will grow and form bronchial cavities and thus become inoperable. It is the 
cancer type most often associated with hypercalcemia. 

(Choice 1) usually occurs in a peripheral location in the lung and has become the most 
common form of non-small cell carcinoma, accounting for 35%-40% of all lung cancers. 
When contained, it tends to respond better than other lung cancers to surgical removal. This 
type of cancer is the most frequent form found in nonsmokers, but the relatively recent 
increase in its incidence is primarily due to smokers of low-tar filter-tipped cigarettes. 
Apparently, microscopic fragments from the filters imbedded with carcinogen from the 
cigarette are responsible for this cancer variant, which also is responsible for the rapid 
increase of this cancer among women and in individuals younger than 45. 

(Choice 3) is a distinctive subtype of typical adenocarcinoma of the lung. A National Institute 
of Cancer survey found it differs from the typical adenocarcinoma in that it has a significantly 
higher 5-year survival rate among patients in all stages of the disease. Moreover, as 
compared to typical adenocarcinoma, bronchioalveolar carcinoma has a higher incidence of 
metastases within the lung but fewer to the brain. Bronchioloalveolar carcinoma also has 
been diagnosed with increasing frequency over the past few decades and now account for 
3%-4% of cases of non-small cell lung cancer;. A characteristic finding in persons with 
advanced disease is voluminous watery sputum. 

(Choice 4) cells are unusually large when seen under the microscope. In a chest x-ray, they 
usually appear in the central portion of the lung. They tend to be accompanied by extensive 
bleeding and tissue damage and to be undifferentiated, without the specific architecture found 
in other types of cancer cells. They grow quickly and metastasize at an earlier stage than 
other forms of non-small cell lung cancers. They also tend to induce paraneoplastic 
phenomena, including secretion of a hormone-like substance that causes gynecomastia in 
males. 

(Choice 5) differ from the non-small cell carcinomas in that the are more aggressive, grow 
rapidly, metastasize to distant sites earlier, and are more frequently associated with distinct 
paraneoplastic syndromes. Except in rare situations, surgery plays no role in their treatment; 
however, they are very sensitive to chemotherapy or radiation. 






10. Question 1 points 

A 56-year old woman presents at a family physician’s office complaining of a chronic cough. She 
says she has had a cough for about the past 10 years, but it usually has been only during a brief 
period after waking up, especially on cold damp mornings. She confides she also is prone to catch 
chest colds in the winter but recovers without any problem. However, for the past 6 months or so, 
the cough has gotten worse. It lasts well into midday or later, and she hacks so badly that she can 
hardly work. Her bosses at the fan manufacturing plant where she has worked for the past 25 
years have threatened to fire her unless she can get her all too frequent times of coughing under 
control; she adds “he also is pissed off because I miss so many days of work since I have so 
many colds.” She continues, “My coughing sometimes is dry but more often produces a thick 
snotty-like spit which sometimes contains pus.” When prodded, she admits she had been smoking 
since she was a teenager, and that for the last 10-20 years she probably smoked about two packs 
a day; she adds, “since cigarettes have became so expensive she has tried to cut back but can’t 
because that long puff feels so good.” 

On physical examination, her blood pressure was 165/85 mm Hg. Her pulse is 100 and regular. Her 
weight is 185 pounds (83.9 kg), height 5 feet, 6 inches (2.2 m), and her body mass index (BMI) is 
29.9. She wheezes while talking. On auscultation, adventitious breath sounds are heard in all lobes. 
X-ray demonstrates significant bronchial wall thickening and increased markings at the base of 
both lungs. Which is the most likely diagnosis in this patient? 


1. Emphysema 

2. Allergic bronchitis 

3. Asthma 

4. Chronic bronchitis V 

5. Smoker’s cough 


INCORRECT X 

The correct answer is 4. 

Chronic bronchitis is a recurrent problem defined by coughing up an abnormal quantity of 
viscous mucus nearly every day for at least 3 months of the year for two or more 
consecutive years, clearly this lady meets this definition. In chronic bronchitis, the 
mucociliary response that normally clears bacteria and mucus from the bronchia is inhibited 
by damage to the endothelium caused by pollutants, primarily those caused by cigarette 
smoke. The inability to clear bacteria efficiently makes these persons vulnerable to catching 
colds, as described for the case described. The inflamed endothelium, combined with loss of 
supporting alveolar attachments, allows airway walls to deform and narrow the airway lumen; 



this, plus the excess phlegm, obstructs the bronchia, causing the obstructive component of 
chronic bronchitis. The body responds by decreasing ventilation and increasing cardiac 
output, resulting in a rapid circulation of hypo-oxygenated blood. This produces hypoxia and 
eventually polycythemia, hypercapnia, and respiratory acidosis; these effects in turn cause 
pulmonary artery vasoconstriction, cor pulmonale, and signs of right heart failure. The 
difficulty these patients have in exhaling caused by the blockage of the bronchia tends to 
make these patients purse their lips, and the hypoxia makes the lips take on a blue-ish tinge. 
Consequently, persons with long-term chronic bronchitis are called “blue bloaters.” 

(Choice 1) is defined as an abnormal, permanent enlargement of the air spaces distal to the 
terminal bronchioles accompanied by destruction of their walls without obvious fibrosis. It is 
believed that noxious irritants found in cigarette smoke (and less often to other pollutants) 
activate polymorphonuclear leukocytes and macrophages, which release human leukocyte 
elastase and other proteases; these eventually overwhelm the anti-proteases of the lung, 
resulting in tissue destruction. As the alveolar septae and the pulmonary capillary bed are 
eroded, the ability to oxygenate circulating blood is diminished; the body compensates for 
these events by reducing cardiac output and hyperventilating. This results in a limited blood 
flow through a fairly well oxygenated lung. Because of low cardiac output and the collapse of 
the alveoli, the rest of the body suffers from tissue hypoxia and generalized cachexia. 
Eventually, these patients develop muscle wasting and weight loss and can be recognized 
as “pink puffers.” Both chronic bronchitis and emphysema lead to chronic obstructive 
pulmonary disease (COPD), an irreversible chronic disease state caused by obstructive air 
flow. Although most cases display characteristics of both chronic bronchitis and 
emphysema, it has been estimated that chronic bronchitis predominates in 85%-88% of the 
cases. 

(Choice 2) refers to short-term inflammations of the tracheobronchial tree caused by 
infections, allergens, or irritants. Although found in all age groups, bronchitis is diagnosed 
most frequently in children younger than 5 years. 

(Choice 3) In asthma, the airways narrow as they become inflamed, constrict, and produce 
excess mucus in response to a triggering stimulant such as an allergen, an irritant, 
temperature changes, exertion, or emotional stress. These responses of the airways cause 
symptoms such as wheezing, shortness of breath, chest tightness, and coughing. Between 
episodes, patients usually feel well but they may develop shortness of breath more rapidly 
while exercising and retain it longer after exercise than the non-asthmatic individual. 
Symptoms of asthma range from mild to life-threatening but most often can be controlled by 
use of bronchodilators, oral drugs, and/or environmental changes. Asthma can be mistakenly 
diagnosed as acute bronchitis if the patient has no prior history of asthma. In one study, one- 
third of patients who had been determined to have recurrent bouts of acute bronchitis were 
eventually identified as having asthma. 

(Choice 5) typically is an early morning cough demonstrated by most smokers. Nonsmokers 
clear harmful substances from their lungs via the action of cilia, which beat outward, thereby 
sweeping the bronchi clean. Smokers, however, both accumulate more toxic materials in 
their lungs than do nonsmokers and also cigarette smoke poisons the action of the cilia. 
Consequently, during the day, these toxic, irritating substances accumulate in smoker’s 
lungs. However, during the night, during which the smoker usually refrains from smoking, 






some cilia recover and begin working again and after waking up, the smoker coughs, trying 
to clear away the poisons that built up the previous day. Unfortunately, prolonged smoking 
leads to the permanent destruction of the cilia and chronic bronchitis. 


11 . Question 1 points 

A 22-year-old woman consulted a physician because she had been feeling poorly. She informed 
the physician that she is extremely tired despite going to bed early and sleeping well. This first 
became a problem almost a year ago, and more recently she also noted she became extremely 
weak after exerting herself physically or even after eating a carbohydrate-rich meal. Additionally, 
she was thirsty all the time, even though she also craved extra salt; understandably, she also 
needed to urinate excessively, even at night, thus interrupting her sleep. In response to the doctor’s 
questions, she also revealed that these spells of weakness involved all four limbs and were worse 
in and around her upper arms and shoulders, as well as in her hip muscles. This made walking 
almost impossible, while her weak arms caused her to drop and break things. She added that she 
also has been getting severe cramps and painful spasms in these muscles. In addition, she thought 
that she has more joint stiffness, some degree of numbness in her hands and feet, and sometimes 
she felt dizzy upon arising. She had no history of nausea, vomiting, or other gastrointestinal 
problems. She added that she does not smoke, only drinks alcohol at a few social occasions, and 
is not taking any prescription or over-the-counter drugs; no person in her family that she was aware 
of had similar problems; however, her parents are first cousins. A “weakness attack” was induced 
by making her lift weights while marching in place and then having her rest. During the ensuing 
attack, the muscles in all four limbs became flaccid and there were associated depressed tendon 
jerks. The muscles of her eyes, face, tongue, pharynx, larynx, diaphragm, and sphincters were not 
involved. Her blood pressure remained within the high normal limit. Laboratory studies showed her 
serum glucose was 132 mg/dL, sodium, 140 mmol/L (normal 136-145), serum calcium 2.5 mmol/L 
(normal 2.2-2.6), serum potassium ion value was 1.6 mmol/L (normal 3.5-5.0), serum magnesium 
ion level was 0.5 mmol/L (normal 0.8-1.2), and serum chloride ion value was 94 mmol/L (normal 98- 
106 mmol/L); the abnormal levels persisted even between the attacks. She also had metabolic 
alkalosis with a bicarbonate ion level of 33 mmol/L (normal 22-30 mmol/L) and a blood pH of 7.48 
(normal 7.35-7.45). A 24-hour urine analysis revealed alkaline urine with higher than normal 
amounts of potassium ion, 285 mmol/24h (normal 25-125 mmol/24h); chloride ion, 625 mmol/24h 
(normal 110-250 mmol/24h); and magnesium ion, 50 mmol/24h (normal 2-5 mmol/24h). On the other 
hand, the urinary calcium ion was lower than normal (1.5 mmol/24h, normal 2.5-7.5/24 hr). Thyroid 
function tests (T3, T4, and TSH) were normal. Which of the following does this lady most likely 
have? 


1. Thiazide diuretic toxicity 

2. Bartter syndrome 

3. Dent syndrome 

4. Andersen-Tawil syndrome 


5. Gitelman syndrome 


INCORRECT X 

The correct answer is 5. 

Gitelman syndrome is a disease of the kidney’s distal convoluted tubule that results in the 
loss of magnesium, sodium, potassium, and chloride ions into the urine. Most cases are 
either isolated or inherited in an autosomal recessive fashion, although there is at least one 
reported family in which the disease was reported to be inherited in an autosomal dominant 
manner. The most common cause of Gitelman syndrome is a mutation in SLC12 A3 gene, 
although a few cases have been attributed to aberrations in CLCKKB. Several different 
mutations have been described in SLC12 A3 gene; this may account for differences in 
symptoms reported in various families. Most cases are relatively benign but some are more 
severe. The product of SLC12 A3 gene functions in the distal convoluted tubule as part of the 
thiazide-sensitive NaCI co-transporter, and homozygous loss of function mutations in this 
protein induces a hypocalciuria, but there is excess urinary loss of sodium, potassium, and 
magnesium ions. Hydrogen ion is retained, causing an alkaline urine. These changes are 
reflected by low circulating levels of potassium and magnesium ions, which are responsible 
for the symptoms of fatigue, muscle weakness, spasms, and pain. As a rule, the disease is 
not recognized until adolescence and often not until middle or even old age. A high- 
carbohydrate meal precipitates an attack because increased levels of glucose increases 
insulin levels, and the resultant increase in glucose utilization lowers circulating potassium 
ion levels. Treatment usually consists of replacement of potassium and magnesium ion. 
Some investigators also recommend an anti-prostaglandin such as oral indomethacin, which 
was found to assist in returning potassium ion levels to normal in at least one case. 

(Choice 1) In so much as thiazide diuretics act on the same NaCI co-transporter as affected 
in Gitelman syndrome, it is not surprising that thiazide diuretic toxicity produces effects 
similar to Gitelman syndrome. However, in the case presented here, the patient states she 
does not take medication, consequently (Choice 1) is not correct. 

(Choice 2) refers to a group of autosomal recessive conditions, all of which, like Gitelman 
syndrome, cause the loss of excess potassium and magnesium ions into the urine. At one 
time, Gitelman syndrome was thought of as a mild form of Bartter. However, they clearly are 
distinct entities. Bartter syndrome usually makes its presence know in neonates or early 
antenatals and essentially always before the age of 6 years; in contrast, Gitelman strikes 
adults. Urinary excretion of magnesium is high in Gitelman syndrome and within the 
reference range in Bartter syndrome; conversely urinary calcium ion levels are high in 
Bartter syndrome and low or within the reference range in Gitelman syndrome. The final 
clinching difference is that different genes are involved. Recent genetic analyses have 
shown that there are five Bartter syndrome variants, as summarized in the following table. 

Bartter Type Gene Affected Site 


Type I 


SLC12A1 


The Na-K-2CI transporter in the distal convoluted tubule 






Type II 


R0MK1 Apical potassium channel 


Type III 


CLCNKB Basal chloride channel in the thick ascending limb of Henle 


Type IV 


BSND 


In barttina, a subunit protein of the basal chloride channel in 
the thick ascending limb of Henle 


Type V 


CLCNKA 


In barttin, a subunit protein of the basal chloride channel in 
thick ascending limb of Henle 


(Choice 3) is a rare X-linked recessive disorder sometimes called idiopathic hypercalcuria. It 
is caused by mutations in the renal chloride channel CLCN5, and it causes low-molecular- 
weight proteinuria, hypercalciuria, aminoaciduria, and hypophosphatemia. Affected males will 
usually show symptoms in early adulthood, including tubular proteinuria, hypercalcuria, 
calcium nephrolithiasis, nephrocalcinosis and hypophosphatemic rickets; a significant 
percentage will have end-stage renal failure before the age of 50. Because of these 
properties, it is one of the several diseases that cause Fanconi syndrome. 

(Choice 4) is an autosomal dominant disease characterized by a triad of symptoms: 

1. Episodic flaccid muscle weakness (i.e., periodic paralysis typified by weakness that 
occurs spontaneously following prolonged rest or after rest following exertion) 

2. Ventricular arrhythmias with a prolonged QT interval. 

3. Physical anomalies that may include low-set ears, eyes set far apart, small mandible, 
fifth-digit clinodactyly, syndactyly, short stature, scoliosis, and mild learning disability. 

Most commonly, serum potassium levels are reduced during periods of weakness. The 
disease is caused by mutations in KCNJ2, which codes for the inward rectifier potassium 
channel 2, known as Kir 2.1, which is involved in setting and stabilizing resting membrane 
potentials and is primarily expressed in skeletal muscle, heart, and brain. 

Phosphatidylinositol 4, 5 bisphosphate (PIP2) is an important regulator of Kir2.1 and many 
KCNJ2 mutations alter PIP2 binding. 


12. Question 1 points 

A 77-year-old man fractured his femur and was undergoing surgery to insert a rod to facilitate 
healing. After the fracture, the distal portion of the femur was pulled up toward the hip by the action 
of his muscles; this caused a lot of bleeding, fortunately, the femoral artery was not punctured. 

Now, during the surgery itself, more bleeding occurred, and it was decided to transfuse him with 





two units of blood. About 90 minutes after the start of the transfusion, it was noted that the patient 
developed chills; a quick check of his temperature showed it rose by 1.5°C from a presurgical 
value of 37.8°C. Which of the following reactions is most likely occurring? 


1. A hemolytic transfusion reaction 

2. A nonhemolytic febrile reaction 

3. A delayed hemolytic transfusion reaction 

4. An allergic reaction 

5. A reaction due to volume overload 

6. An acute lung injury 

7. A reaction caused by increased oxygen affinity 


INCORRECT X 

The correct answer is 2. 

A nonhemolytic febrile reaction happens during 3%-4% of transfusions, making it the most 
common type of transfusion reaction and thus the most likely reaction to have occurred in 
the case described. It is thought that the most likely cause of this reaction is the presence of 
antibodies against white blood cell human leucocyte antigen (HLA) in the donor blood. 
Another possibility is the release of cytokines from white cells during storage; however, this 
is most likely to happen in stored platelet preparations. Nonhemolytic febrile reactions occur 
most frequently in patients who have had blood transfusions before or in multiparous women. 
Such febrile reactions are successfully treated with acetaminophen, and some hospitals 
routinely give patients acetaminophen prophylactically prior to surgery and/or use pre¬ 
storage leukocyte-reduced blood components, at least for high-risk patients. Once fever is 
noted, the transfusion must be terminated and the blood rechecked to make sure that it truly 
is the proper type for the patient and the blood pack in question is destroyed. This is an 
expensive interruption, making it worthwhile to take such measures to avoid it when 
practical. 

(Choice 1) is the most dangerous type of reaction and approximately 20 patients die each 
year because of it; it occurs in about 1 in 40,000 transfusions. ABO incompatibility is most 
common, although antibodies against Rh or other blood group antigens do occur. Most 
common causes are: mislabeling of the recipient’s pre-transfer sample, giving the wrong 
blood to a patient with the same name, or not checking the patient’s blood bank identification 
tag with the identification number on the unit; a laboratory error in identifying the proper blood 
type very rarely occurs. The severity of the reaction depends upon the degree of 
incompatibility, the transfusion rate, the amount of blood product given, and the health of the 
recipient. Most often, the onset of symptoms is within an hour of initiation of the transfusion, 
but it can be delayed. If the patient is under general anesthesia, the only symptoms of the 
reaction may be hypotension, profuse bleeding from incision sites and mucous membranes 
caused by disseminated intervascular coagulation, and/or a dark urine caused by 





hemoglobinuria. If a hemolytic transfusion reaction is suspected, the transfusion must be 
stopped, the sample and patient identification rechecked, and supportive treatment begun. 
The initial goal of treatment is to maintain blood pressure and renal blood flow with IV isotonic 
saline and furosemide. 

(Choice 3) may occur if the patient has been exposed to an antibody level so low that it gave 
a negative pre-transfusion test. In this case, the reaction may occur as late as 1-4 weeks 
after the transfusion. Usually, the reaction is not very severe, often limited to the transfused 
erythrocytes causing a drop in the hematocrit. If a more severe reaction does occur, it 
should be treated as if it were a hemolytic transfusion reaction. 

(Choice 4) An allergic reaction to an allergen in the donor blood is not uncommon. Usually, 
the reaction is mild with urticaria, edema, and maybe dizziness during or immediately after 
the transfusion. An anaphylaxis reaction is rare, most often occurring in IgA deficient 
recipients. In the case of an anaphylactic reaction, transfusion should be stopped 
immediately and the reaction treated. Patients with IgA deficiency should be transfused with 
blood from an IgA-deficient donor. 

(Choice 5) might be induced in susceptible patients by the high osmotic load of blood 
products in the transfusion, which draws water into the intravascular space. To reduce this 
possibility, red blood cells should be infused slowly. Susceptible patients include those with 
renal insufficiency or cardiac problems. The patient should be observed carefully, and if 
signs of problems arise, the transfusion should be stopped and the patient treated; typical 
treatment is with a diuretic. 

(Choice 6) may be caused by anti-HLA or anti-granulocyte antibodies in the donor plasma 
that agglutinate and degranulate recipient granulocytes in the recipient’s lung. Such a 
reaction occurs in 1 case per 5,000 to 10,000 transfusions. Although many cases are mild, 
the reaction may cause severe acute respiratory symptoms, even death, making this the 
second most common cause of transfusion-related death following ABO incompatibility. In 
nonfatal cases, supportive therapy leads to complete recovery, but even mild incidents 
should be reported. Blood stored for more than 7 days has a marked decrease in the red cell 
2, 3-diphosphogylycerate (DPG) level, and after 10 days, the DPG level is essentially nil. 
(Choice 7) This absence enhances the affinity of hemoglobin for oxygen, slowing the release 
of oxygen to the tissues of a recipient. Such an effect can be significant in infants, sickle cell 
patients, and patients suffering a stroke or acute chest symptoms. 


13. Question 1 points 

A 65-year-old man sees his primary care physician because of pain in his chest that he thinks 
might indicate heart trouble. He describes the pain as a burning sensation in his chest that radiates 
into the neck and his left arm. He also reports that he regurgitates a sour-tasting liquid that leaves a 
feeling of “pins and needles” in his throat and mouth. These symptoms are not aggravated by 
exercise but are by drinking coffee or chocolate, or by eating fatty foods. Results of a physical 
examination that included his heart sounds and an electrocardiogram (ECG) taken as part of an 


exercise tolerance test are unremarkable. An upper gastrointestinal barium x-ray study shows the 
presence of a hiatal hernia. Which of the following is the primary mechanism for this patient’s 
complaints? 


1. Decreased acid production in the stomach 

2. Increased gastric emptying 

3. Decreased esophageal peristalsis 

4. Inappropriate relaxation of the lower esophageal sphincter (LES) 

5. Absence of ganglion cells in the myenteric plexus 


INCORRECT X 

The correct answer is 4. 

Although these symptoms clearly indicate that the patient has gastroesophageal reflux 
disease (GERD), it is always a wise move to rule out the possibility of cardioarterial disease 
(CAD), as was done by the exercise tolerance test. GERD is a chronic, recurrent disorder 
characterized by heartburn, belching, and epigastric pain. The primary mechanism is 
inappropriate relaxation of the LES. Agents that decrease LES pressure, such as chocolate, 
coffee, ethanol, fat, peppermint, anticholinergics, theophylline, diazepam, barbiturates, and 
calcium channel blockers, exacerbate GERD. About 1 in 10 nonpregnant adults complain 
about heartburn at least once a week, and prevalence of GERD increases with age, is found 
more commonly in older men than in older women, and is very common during pregnancy; 
approximately 1 in 4 pregnant women experience heartburn from acid reflux daily during 
pregnancy. This very high frequency is attributed to impaired LES competence coupled to 
increased abdominal pressure. 

Secondary factors that predispose to GERD include hiatal hernia; acid concentration of the 
refluxate; delayed acid clearance, which is associated with low-amplitude peristalsis and 
recumbency; and decreased gastric emptying, which aggravates reflux. The acid 
concentration of the refluxate is the most important factor in determining progression to reflux 
esophagitis. Complications associated with GERD are reflux esophagitis, stricture formation, 
and Barrett’s esophagus. Barrett’s esophagus is a premalignant condition characterized by 
glandular metaplasia of the distal esophagus. The risk of developing adenocarcinoma is 
2%-10% in the United States, and distal adenocarcinoma of the esophagus has replaced 
squamous cell carcinoma of the esophagus as the most common esophageal cancer. 

GERD can also precipitate an asthmatic attack by reflux vagal stimulation or aspiration. 
Approximately 80% of adults with bronchial asthma have GERD. This percentage appears to 
be independent of bronchodilator therapy. Chronic aspiration is also linked to pulmonary 
fibrosis and bronchiectasis. Ambulatory monitoring of esophageal pH for 24 hours, the gold 
standard, can detect GERD and provide information relative to the potential for chronic 
aspiration of acid reflux. The treatment of GERD involves reducing gastroesophageal reflux, 
neutralizing the acid reflux, enhancing esophageal clearance, and protecting the esophageal 
mucosa from ulceration. Diet modification, weight loss, postural therapy (elevation of the 



head of the bed), restriction of alcohol, and cessation of smoking, along with pharmacologic 
treatment, are used in a stepwise approach reflecting patient response. A recommended 
progression of drugs includes antacids and alginic acid —>■ histamine-2 (H 2 ) receptor 
antagonists at conventional doses —► H 2 receptor antagonists at high doses, omeprazole, or 
prokinetic agents (metoclopramide) —*■ antireflux surgery. 

(Choice 1) is noted in pernicious anemia (destruction of the parietal cells) and Helicobacter 
pylori-induced atrophic gastritis involving the antrum and pylorus. 

(Choice 3) is noted in both achalasia and systemic sclerosis. 

(Choice 5) is the key finding in achalasia. 


14. Question 1 points 

A 42-year-old woman complains of aching in the shoulder muscles, difficulty with climbing stairs, 
and difficulty with swallowing solids and liquids from her upper esophagus. Physical examination 
reveals atrophy of her shoulder and pelvic muscles. There is erythema over the bridge of her nose, 
with violaceous discoloration and edema of the upper eyelids. Erythematous papules are noted 
over the proximal interphalangeal joints, metacarpophalangeal joints, elbows, and knees. An 
electromyogram (EMG) reveals low-amplitude poly phasic potentials, and laboratory testing reveals 
an increased creatine phosphokinase (CPK). Which of the following is the most likely diagnosis? 


1. Polymyalgia rheumatica 

2. Rheumatoid arthritis 

3. Polymyositis 

4. Dermatomyositis 

5. Systemic lupus erythematosus (SLE) 


INCORRECT X 

The correct answer is 4. 

The patient has dermatomyositis (DM), which has some overlapping features with 
polymyositis (PM). Both disorders are a female-dominant type of myositis, with DM involving 
skin and muscle in adults and children, and PM involving only muscle in adults (Choice 3). 
Both disorders have muscle pain and atrophy, with the shoulders and pelvic muscles most 
commonly involved. The pelvic muscle weakness makes it difficult for patients to climb 
stairs. Dermatologic lesions associated with DM (not PM) include erythema over the bridge 
of the nose, with violaceous discoloration and edema of the upper eyelids; additionally, 
erythematous papular lesions may be noted over the dorsum of the proximal interphalangeal 
joints, knuckles, elbows, and knees. Dysphagia for solids and liquids is present, because of 
weakness of the striated muscle in the upper esophagus. Laboratory findings include a 



positive serum antinuclear antibody test result in 30% of cases. Anti Jo-1 and anti PM-1 
antibodies are present in a small percentage of cases. The serum creatine kinase is 
increased, and muscle biopsies show a lymphocytic inflammatory reaction with destruction 
of muscle fibers. The treatment for DM and PM is chronic prednisone therapy. If patients fail 
to respond to steroid therapy, they may be placed on immunosuppressive agents such as 
azathioprine, cyclophosphamide, or methotrexate. 

(Choice 1) may be associated with proximal muscle aching; however, serum creatine and 
the electromyogram (EMG) are normal. 

(Choice 2) Likewise, rheumatoid arthritis does not feature increased in serum creatine 
kinase or an abnormal EMG. 

(Choice 5) could easily explain the “butterfly rash” but would not explain the myopathic 
features expressed in the EMG and the increased muscle enzymes. 


15. Question 1 points 

A well-dressed but somewhat dishevel male presents at a walk-in clinic complaining of difficulty 
with breathing and a pain in his chest with a chronic cough. Upon providing a history, he says that 
he is a 

31-year-old Harvard MBA graduate and admits that, although he is the CEO of a small business, he 
has been burning the candle at both ends for at least the past 5 years and has not taken care of his 
health. The attending physician notices his foul-smelling breath before anything else. The patient 
also indicates he has not been feeling well for a couple of weeks and is bothered by profound 
sweating, an inability to sleep, and has lost 5 pounds during the past month. Upon further 
questioning, he admits he has a drinking problem and more than occasionally drinks until he 
passes out. Upon examination, the doctor notices his teeth are in sad shape, and he has evidence 
of gingivitis. Additionally, his temperature was 37.5°C (99.5°F) and he has decreased breath 
sounds and crackles over the area where the pain was most intense; there is also egophony, and 
dullness to percussion in the presence of effusion. A chest x-ray was performed; it showed 
consolidation with a single cavity containing an air-fluid level in the posterior segment of the upper 
right lobes. Among the follow- ing choices, which represents the best next step in the treatment of 
this man? 


1. A prescription for oral metronidazole 

2. A prescription for oral clindamycin 

3. A prescription for oral vancomycin 

4. A prescription for oral penicillin G 

5. A prescription for oral isoniazid 



INCORRECT X 

The correct answer is 2. 

The chest x-ray showed a fluid-filled cavity characteristic of an abscess. Characteristically, 
this is due to a necrotizing lung infection that produced a pus-filled cavitary lesion. Such 
abscesses are generally caused by aspiration of oropharyngeal material by patients who are 
unconscious. The risk is increased in patients with gingivitis or poor oral hygiene. This 
patient has poor oral hygiene and confessed to bouts of unconsciousness under the 
influence of alcohol. The foul smell of his breathe suggests anaerobic bacteria are at least 
partially responsible for the infection. In the absence of additional data, the best course of 
treatment is a broad-spectrum antibiotic such as clindamycin. Had he been sicker and if 
available in a walk-in clinic, he would most likely been given clindamycin IV. In any case, he 
should be advised to either see his own physician, or to return within a week; unfortunately, 
walk-in clinics are not designed to provide continuous, coordinated, or comprehensive care. 
(Choice 1) has activity against anaerobes and protozoa, but to be used in this situation it 
must be combined with an antibiotic such as penicillin to make sure the growth of aerobes is 
also inhibited. 

(Choice 3) A prescription for oral vancomycin would be used if infection by a methicillin- 
resistant Staphylococcus aureus (MSRA) organism were suspected; however, that is not 
the case, and it is best to reserve use of vancomycin for treatment of resistant bacteria, to 
minimize the development of vancomycin resistance. 

(Choice 4) A prescription for oral penicillin G would not effective because it would be 
degraded by the stomach’s acidity; consequently, it always is administered parentally. 
Moreover, it would not be effective against gram-negative bacteria. 

(Choice 5) A prescription for oral isoniazid would be used to treat tuberculosis, which would 
be a very unlikely possibility in this patient. 


16. Question 1 points 

A 30-year-old woman, who is a nonsmoker, presents with bilateral puffiness and swelling of the 
fingers and joint pains. Cold exposure and stress cause episodes of blanching of the fingers. After 
turning white, these digits take on a blue-ish tint and then a red one. She is not on any medications, 
and her liver aminotransferase enzyme activities have always been stable and within the normal 
range. Her thyroid function tests are also normal. The mechanism for this patient’s disease is most 
likely the result of which of the following? 


1. Vasospasm and thickening of the digital arteries 

2. Hyperviscosity due to cryoglobulins 

3. An immune complex vasculitis 

4. Thrombosis of the digital vessels 




5. An embolism to the digital vessels 


INCORRECT X 

The correct answer is 1. 

The patient has Raynaud’s phenomenon, which most commonly affects women aged 
between 20 and 50 years. The phenomenon is marked by the changing color of the digits, as 
described for this patient. The blanching is due to a diminishing blood supply; the blue tint 
followed by a reddish color is caused by prolonged hypo-oxygenation, followed by flushing 
when the blood vessels open again. The phenomenon is commonly seen in association with 
various rheumatoid conditions, including scleroderma, rheumatoid arthritis, and systemic 
lupus erythematosus. In these conditions, the symptoms of Raynaud’s phenomenon may 
precede the other symptoms of the underlying disease by years or even decades. This most 
notably occurs in sclerosis; it has been reported to be the presenting sign in 85% of the 
cases of systemic sclerosis and in 95% of the cases of localized scleroderma (aka, the 
CREST syndrome). Similar symptoms can be induced by other factors including 
hypothyroidism, carcinoid, frostbite, chronic use of vibrating tools, or a side effect of several 
medications, or the underlying factor(s) may be idiopathic. In idiopathic cases or those not 
involving generalized physical aberrations, in particular a rheumatoid disease, the multi 
coloration of the digits is often referred to as Raynaud disease rather than Raynaud’s 
phenomenon. This question asks which choice most likely describes the underlying factor 
causing Raynaud’s phenomenon in the patient described, and the data presented essentially 
eliminate most nonrheumatic causes of the phenomenon; in most, if not all, of the underlying 
rheumatoid conditions, the responsible factors playing critical roles responsible for initiating 
these changes are vasospasm and thickened digital vessels. 

(Choice 3) Although these rheumatoid conditions are autoimmune diseases, immune 
complexes are not present in the vessels. 

(Choices 2 & 4) Two other systemic causes of Raynaud’s phenomenon are thromboangiitis 
obliterans (Buerger disease), which is an inflammatory vasculitis producing thrombosis of the 
digital vessels in male smokers (patient is female and a nonsmoker); and cryoglobulinemia, 
with proteins that precipitate in cold temperatures. The latter condition is commonly 
associated with chronic hepatitis C (not present in this patient, as demonstrated by her 
normal, stable liver transaminase levels). In addition to affecting the digits, in cold weather 
this condition also produces cyanosis of the nose and ears that reverses to normal when 
returning to a warm environment. 

(Choice 5) Embolism to digital vessels in the hand is uncommon, and when it does occur, it 
is most commonly due to infective endocarditis or atrial fibrillation, neither of which is present 
in this patient. 


17. Question 


1 points 







A 33-year-old man presents as a new patient to a primary care physician. He tells the physician 
that he has had almost constant abdominal pain for at least the past month, and he lost his appetite 
and about 15 pounds during the past month; he also complains of being fatigued almost all the time. 
In addition, he has had frequent bouts of bloody diarrhea and the diarrhea and the concomitant need 
to wipe his bottom left his rectum sore. He adds that he had a similar sequence of events about 5 
years previously, and at that time, his physician told him he had a nervous stomach. He adds that 
his younger brother seems to be having similar problems. The physician notes that he looks pale, 
and upon examination, she finds he has diffuse abdominal tenderness with the right lower quadrant 
being the most sensitive area. His temperature is 99.5°F (37.5°C),blood pressure 98/78 mm Hg, 
pulse 90 and regular; a complete blood count reveals that his serum hemoglobin value is 9.8 g/dL 
and a peripheral blood smear reveals a decreased mean corpuscular hemoglobin count (MCHC) 
and a decreased mean corpuscular volume (MCV). Among the following choices, which most likely 
represents the disease that this man suffers from? 


1. Crohn’s disease >/ 

2. Ulcerative colitis 

3. Irritable bowel syndrome 

4. Lactase deficiency 

5. Nervous stomach 


INCORRECT X 

The correct answer is 1. 

Crohn’s disease is an inflammatory autoimmune condition with a significant genetic 
predilection; however, the course of the disease varies greatly from patient to patient. In 
many cases, attacks go into remission only to recur again years later; in other cases, the 
disease is consistent and persistent. The inflammation causes bowel cells to secrete large 
volumes of water with more salt than can be resorbed; consequently, it causes diarrhea. In 
mild cases, this may only be annoying but in severe cases patients may have dozens of 
bowel movements per day, interfering with daily activities and sleep. The inflammation also 
may cause thickening and scarring of the mucosa, which in turn causes cramping and pain. 
Again, the pain can vary from a minor annoyance to almost being intolerable. In addition, the 
inflammation can lead to ulceration and bleeding, which can induce a microcytic anemia, as 
in the case described, or less commonly it may interfere with vitamin B12 absorption in the 
terminus of the ilium and induce pernicious anemia, a macrocytic event. The pain and 
cramping inhibits appetite, causing weight loss. In some cases, the ulceration can be so 
severe that fistulas are formed and organs adjacent to the colon become affected, causing a 
host of potential secondary problems. The inflammation is often localized in the ilium (about 
50% of the time) or at the junction of the ilium and the colon (about 30% of the time), and in 
the body of the colon about 20% of the time, but it can occur at any place, varying from the 
mouth the rectum. In some patients, the affected area is a continuum, whereas in others it 
develops simultaneously in different areas, creating a patchwork effect. Ulceration can even 



happen outside the gastrointestinal system as, for example, in the mouth, skin, liver, joints, or 
eyes. What triggers the condition is unknown, but there appears to be a strong genetic 
component; approximately 20% of Crohn’s disease patients have a first-degree relative who 
also is affected. The onset of the disease can start at any age but most typically appears 
between the ages of 15 and 35 years. It affects men and women to an equal extent, and 
Caucasians are at greater risk than other ethic groups. 

(Choice 2) is the other inflammatory gastrointestinal disease. Unlike Crohn’s disease 
ulcerative colitis causes inflammation only in the colon or rectum, almost always only 
involves the superficial layer of the inner lining of the bowel, and the inflammation generally is 
diffuse and uniform rather than concentrated in some areas and pitted and nonhomogeneous. 
However, some relation must exist between the two diseases since having first-degree 
relatives with ulcerative colitis is a risk factor for developing Crohn disease. 

(Choice 3) is not a disease per se but a description of a group of functional disturbances that 
may cause symptoms such as bloating, flatulence, diarrhea and/or constipation, and mucus 
in the feces. Unlike Crohn disease or ulcerative colitis, the syndrome very rarely causes 
inflammation or any type of tissue damage. Irritable bowl syndrome is very common. There is 
no known cause, but individuals may have heightened sensitivity to certain foods or to 
stress. It is more common in young adults, particularly in females. 

(Choice 4) is defined as too little of the intestinal enzyme lactase used to digest the lactose 
(milk sugar) found in the normal diet. By definition, all mammalian species drink milk at birth 
and therefore must produce sufficient amounts of lactase to thrive through infancy. However, 
as they age, most species develop an intolerance to lactose-containing foods (dairy 
products), and they lose the ability to synthesize sufficient lactase. This intolerance causes 
bloating and flatulence upon ingestion of dairy products. Western Caucasian Europeans and 
their descendents are the major exceptions to the rule that lactase synthesis ceases with 
age. 

(Choice 5) There isn’t a specific diagnosis or a recognized disease called “nervous 
stomach”. Some doctors may use the term to generally describe symptoms of indigestion, 
bloating, or changes in bowel habits, especially after tests fail to reveal a specific cause. 


18. Question 1 points 

A 27-year-old Irish Catholic woman gave birth to a 7- pound 5-ounce baby boy. The vaginal birthing 
was normal and everything seemed fine, but 26 days after the baby was born the mother began to 
have heavy vaginal bleeding. Her husband called 911, and an ambulance brought her to a nearby 
trauma facility. They managed to stop the bleeding, but she had lost so much blood that they gave 
her two units of replace- ment blood and admitted her into the hospital. There, the attending 
physician noticed she had many bruises on her legs, arms, and trunk, making him think abuse. But 
he took a careful history, starting with the questions: How did you get these bruises? Have you or 
any of your family ever had a bleeding problem before? She replied, “The bruises just appeared, but 
I always did bruise easily. As to bleeding problems I can’t remember anything remarkable, except I 
used to have a bloody nose on a regular basis; so much so I some- times missed school; the 
school nurse told me to stop picking my nose, even though I didn’t think I did.” The physician also 


asked: “How about your menses?” She replied, “No real problem, they occurred on a regular basis 
with minimal cramping, but I did lose a lot of blood. However, my family’s doctor told me not to 
worry about it since my mother and grandmother also had heavy bleeding during their periods, thus 
it must simply be a familial trait.” On the basis of this information, which of the following conditions 
does this lady most likely have? 


1. Hemophilia A 

2. Hemophilia B 

3. Hemophilia C 

4. Bernard-Soulier syndrome 

5. von Willebrand disease >/ 


INCORRECT X 

The correct answer is 5. 

Von Willebrand disease is the most common of all the bleeding disorders; it affects both 
sexes equally, with a prevalence accounting for somewhat over 1% of the population. There 
are three major forms of the disease, types 1,2, and 3, with four subsets of type 2: namely, 
2A, 2B, 2M, and 2N. Patients with type 1 have less than the normal amount of von 
Willebrand’s factor (vWP), patients with type 2 disease have aberrant forms of vWP, and 
patients with type 3 disease have very little if any vWP. Types 1 and 2 are inherited as 
dominant traits; type 1 is the most common form, accounting for about 70% of case, and type 
2A is the next most common form, making up about 17% of the cases. The type 3 disease is 
inherited as a recessive trait, is the most severe form, and is also not commonly 
encountered. During pregnancy, the levels of vWP normally rise throughout the third 
trimester and then decrease, normally to baseline about a mouth postpartum. However, in 
patients with von Willebrand disease, the decrease is exaggerated and levels of vWP decline 
even below the pre-pregnancy levels, which were already less than optimal. Consequently, 
as in the case described, a hemolytic crisis may take place some weeks after the birth 
(usually about a month). Recent National, Heart, Lung and Blood Institute (NHLBI) guidelines 
state that health care providers should remain in close contact with women with von 
Willebrand disease during this period. Although von Willebrand disease affects males and 
females to an equal extent, females more commonly become aware of otherwise borderline 
cases because of their menstrual periods. As in the case described, it is not uncommon for 
generations of women to have abnormally heavy menstrual flow, and while true that it runs in 
families, that does not make it normal. The function of vWP is to bind to other factors 
involved in coagulation and bring them to wound sites, thus permitting them to work together 
to inhibit bleeding. The basic vWP subunit is a 2,050 amino acid protein with specific 
domains for coagulation factor VIII, heparin, collagen, and platelet receptors and hence 
platelets. These monomers are N-glycosylated and converted into multimers in a process 
involving cysteine cross-linking at the C-terminus. These multimers are very large, 
containing up to 80 monomers, and only these larger forms of vWP are functional. Pactor VIII 





is rapidly degraded when it is not bound to vWP, thus low circulating levels of vWP are 
accompanied by low levels of factor VI11. As mentioned, type 1 von Willebrand disease is due 
to reduced levels of functional vWP. Remember that, since this is a dominant condition, 
affected individuals all should have at least 50% of the normal levels of functional factor 
inherited from the normal parent. Variation in activity then depends upon residual activity 
remaining in the aberrant factor inherited from the other parent; thus, type 1 patients have half 
to near normal vWP levels and activity. In type 3 von Willebrand disease, a child inherits an 
aberrant factor from both parents; consequently, the type 2 diseases involve lack of 
functionality or hyper-functionality in the factor that is synthesized. 

(Choices 1 & 3) Hemophilia A and hemophilia B are both sex-linked recessive conditions 
and consequently are rarely expressed in females; thus, they are clearly incorrect choices. 
Hemophilia A is the most common form, with a prevalence of about 1 case per 5,000 male 
births and results in a deficiency of blood coagulation factor VIII; hence, an alternate name, 
factor VIII deficiency. Patients may have cases that vary from severe to minor bleeding 
problems. Hemophilia B results in a deficiency of coagulation factor IX, and is also called 
factor IX deficiency or Christmas disease, after the first patient in whom the disease was 
described. It is less common than hemophilia A, with a prevalence of about 1 case per 
25,000 male births. 

(Choice 3) causes a deficiency of coagulation factor XI and affects members of both sexes. 

It is inherited in a recessive fashion and is quite rare, affecting about 1 person among 
100,000 adults, almost only people of Ashkenazi or Iraqi Jewish decent, making it unlikely 
that this Irish Catholic lady suffers from this disease. 

(Choice 4) is a very rare condition, with an incidence of about 1 case per 1 million 
individuals; it is characterized by a thrombocytopenia. The biochemical cause is decreased 
expression of the glycoprotein complex Ib/IXA/ on the surface of platelets. This complex 
normally serves as a vWP receptor, and when it is deficient, platelet adhesion to wound sites 
is inhibited. In vitro, this can be demonstrated by the lack of aggregation of platelets in 
response to ristocetin, an antibiotic that normally induces platelets to aggregate. A deficient 
number of platelets at the wound site inhibits formation of the primary platelet plug and 
causes an increased tendency to bleed. The thrombocytopenia may be due to decreased 
platelet half life. 


19. Question 1 points 

A 26-year-old woman frantically presents to her physician showing him that she lost patches of 
scalp hair at various places. She screams, “I cannot continue living looking like this!” She blames 
her hair dresser, saying she left her under the dryer too long when she got a permanent and that 
she must have cooked her scalp. She is thinking of bring a lawsuit. She also says she expects the 
physician to provide treatment and to tell her if a suit is justified. Which of the following choices 
represents the physician’s best response? Tell her that: 


1. He can perform a hair transplant that will restore her hair within a few months but she 


doesn’t have sufficient grounds for a suit. 

2. He can perform a hair transplant that will restore her hair within a few months and he 
will testify on her account if she sues. 

3. He feels confident that it is most likely that her hair will grow back spontaneously, but 
he has medication that may accelerate the process. However, there are no guarantees and 
he doesn’t believe she has a case worth pursuing in court because the condition is an 
autoimmune reaction. V 

4. He knows of several similar cases all caused by permanents and the end result 
always is complete baldness; so she should certainly sue. 

5. He recommends shaving her head so the bald spots are no longer discernible; he will 
be glad to testify in court as to the extent of her loss. 


INCORRECT X 

The correct answer is 3. 

This lady has a case of alopecia areata. Alopecia is a general term for baldness; alopecia 
areata is a common type of specific hair loss that causes coin-sized areas of baldness, 
usually on the scalp, although it may occur any place on the face or trunk. It affects 1%-2% 
of the population, both males and females, most often teens or young adults, but may occur 
at any age. Hair generally grows back spontaneously, and it is thought to be an autoimmune 
reaction; thus, (Choice 3) is correct and permanents are not suspected as a cause (Choice 
4) is wrong). Person with this disorder sometimes have other autoimmune diseases, and 
there is a familial tendency to have the condition. As long as the loss is patchy and not all- 
inclusive, there is about an 80% chance it will grow back within a year. The early growth 
starts in the center of the bald patch; initially, it is fine and white, but with time, the hair usually 
regains its normal characteristics, although in older people it may not regain its color. The 
risk of not growing back is increased if there are large areas of hair loss, and in individuals 
with Down syndrome or eczema. If the hair loss on the scalp is total, resulting in baldness, 
the condition is called alopecia areata totalis; if the loss is all over the body, it is called 
alopecia areata universalis. These are relatively uncommon conditions accounting for less 
than 1% of all alopecia areata cases and in these cases regrowth usually does not happen. 
(Choices 1 & 2) Alopecia areata usually does not lend itself to hair transplantation, therefore 
these (Choices 1 & 2) are incorrect. Some physicians would rather not treat alopecia areata, 
since odds are the hair will grow back spontaneously, but as it says in (Choice 3), there are 
several treatments that may accelerate the rate of regrowth. Steroid 
creams, steroid injections or pills, dithranol treatment, and physical irritation all have been 
used and seem to jump-start hair growth, but often hair loss starts again once treatment 
stops. Steroids in particular may induce serious side effects. The least disruptive and 
probably the most effective treatment is minoxidil lotion. 

(Choice 5) Shaving her head is a counterproductive response, and is incorrect. 




20. Question 


1 points 


A 59-year-old man works as an accountant in a high- rise office building in Manhattan. Por many 
years, he habitually descended the 26 stories to the street at 10:30, noon, and 2:30 to smoke a 
cigarette and take a walk that lasted about a half hour during his lunch break. He figures walking is 
good for his health. However, starting about a month ago, he began to get pains in his calf that 
made walking difficult. These pains have become progressively worse, limiting his walks to just a 
few minutes before he had to find a place to sit and rest. He also found it hard to climb the stairs at 
home to go to bed; sometimes, he only managed three of four steps before he felt obliged to sit and 
rest. Worried, he consulted a physician who examined him and arrived at a tentative diagnosis. 
Which of the following choices best describes the first test the physician most likely preformed to 
verify this diagnosis? 


1. Peripheral Doppler ultrasound 

2. Peripheral angiography 

3. Computed tomography (CT) scan 

4. Ankle-brachial index (ABI) determination 

5. Magnetic resonance (MRI) scan 


INCORRECT X 

The correct answer is 4. 

This man most likely has peripheral artery disease (PAD), sometimes called peripheral 
vascular disease (PVD), of the legs. The simplest and usually first diagnostic screening test 
is determination of the ankle-brachial index (ABI). This is done by simply determining the 
brachial and ankle systolic blood pressures and then dividing the brachial pressure into the 
value for the pressure at the ankle. A ratio greater than 0.9 is considered normal; a ratio 
between 0.8 and 0.9 is consistent with mild PAD, between 0.5 and 0.8 moderate PAD, and 
less than 0.5 severe disease. If the ABI is abnormal, a test (or tests) to better determine the 
site and quantify the extent of the lesion causing the occlusion may also be run. 

(Choices 1,2 & 5) These imaging tests are likely to be peripheral Doppler ultrasound 
(Choice 1), peripheral angiography (Choice 2), or a computed tomography (CT) scan 
(Choice 3) would be non-contributory. Although a magnetic resonance imaging (MRI) scan 
(Choice 5) will provide an accurate image of the interior of the artery, it is less likely to be 
used because of the cost. In the United States, it is estimated that 10%-25% of the 
population over the age of 55 years has discernible PAD. Risk factors include smoking, 
diabetes, high blood pressure, dyslipidemia, obesity, male, African American descent, age 
older than 55, or having any other known vascular disease. Additionally, since the cause of 
PAD is most likely arteriosclerosis, having PAD suggests that a person is at risk of having 
other arterial diseases, such as coronary arteriosclerosis. Moreover, thrombi may break off 
from the arteriosclerotic regions in the leg and travel to the heart or lungs. The first step in 
treatment is smoking cessation. Subsequent steps are bringing other risks factors, such as 
diabetes or hypertension, under control. Accomplishing this is likely to require specific 





medication in addition to lifestyle modification. Chelation therapy is a widely advertised 
alternative treatment; however, there is no sound evidence to support the use of this 
modality, which at the least can be expensive and on occasion dangerous. If such 
treatments are not beneficial, surgery (stents, bypass surgery and angioplasty, etc.) might 
be attempted. An unfortunate last resort most commonly utilized for diabetics is amputation. 


21. Question 1 points 

A 29-year-old man visits his family physician with a history of pain in his legs. He states that the 
pain occurs in the evening and even at night, which results in disturbed sleep. He has even tried 
waking up and walking around to try to get rid of the pain. At times, he feels a tingling sensation in 
the legs, as if some- thing were crawling over them. Recently, he noticed a cramp like pain while he 
was driving, and he had to pull over. He had seen a psychiatrist recently and had been prescribed 
a neuroleptic medication. Based on the presentation, the most likely diagnosis to consider would be 
which of the following? 


1. Painful legs and moving toes syndrome 

2. Fibromyalgia 

3. Restless leg syndrome 

4. Cauda equina syndrome 

5. Akathisia 


INCORRECT X 

The correct answer is 3. 

Restless leg syndrome is a disorder of unknown etiology that affects both men and women 
equally. These patients complain of disagreeable sensations in the legs, cramp like pains, 
sometimes a crawling sensation, or even itching. These patients try to move their limbs to 
get rid of the pain, even getting up to walk or pace around. While the pain tends to disappear 
with movement, it returns during the resting phase. The pain can disturb sleep. It usually 
peaks by midnight and thereafter tends to improve by morning. The problem can occur 
during the day, either while being sedentary or while driving a car. It may be associated with 
iron deficiency anemia, diabetes, pregnancy, and chronic renal failure, especially in patients 
who are on dialysis. Some patient may go for months without symptoms. There are no 
clinical tests, and the diagnosis is based on a history of dysesthesia, motor restlessness, a 
desire to move the limbs, and worsening of symptoms at night or during periods of rest. 
(Choice 1) does not occur at night and so does not induce insomnia. These patients 
complain of pain in the legs and feet, and involuntary writhing movements occur during this 
time. The pain does not get worse at the end of the day or during the night. 





(Choice 2) usually involves a large area of the body, including the axial skeleton. Symptoms 
generally occur throughout the day, and movement does not improve it. 

(Choice 4) is usually associated with low back pain, unilateral motor weakness, and sensory 
loss, and there may be loss of anal sphincter tone and problems with urination. 

(Choice 5), an inner restlessness condition marked by an irresistible urge to move, is 
usually induced by medications such as phenothiazines or is a result of illness, such as 
Parkinson disease. These patients have restlessness that tends to affect the entire body, 
unlike restless leg syndrome, in which the problems are localized to the extremities. 
Symptoms occur less often at night, so insomnia is not a feature. 


22. Question 1 points 

An 87-year-old man diagnosed with type 2 diabetes 27 years ago also has systolic hypertension 
and a body mass index (BMI) of 32. He presently is taking hydrochlorothiazide, atenolol, and 
amlodipine to control his systolic blood pressure, which when measured at home still averages 
about 167 mm Hg, and at the doctor’s office, it ranges from 188 to 205 mm Hg. In addition, he also 
takes atorvastatin to help control his cholesterol level, which is now, with the help of this drug, at 
125 mg/dL. In reviewing the results from a basic metabolic panel taken the previous week, his 
physician noted his serum creatinine level was 1.20 mg/dL (normal 0.70-1.30) and his blood urea 
nitrogen (BUN) 23 mg/dL (normal 9-23). After reviewing his history, the physician recommends 
starting enalapril to lower the patient’s blood pressure. Three days after starting the enalapril, the 
patient checked himself into the emergency room (ER) complaining that he had hardly been able to 
urinate for the past 2 days. In addition, his arms and legs seemed to be swelling; he has abdominal 
pain, is unusually tired, and has problems concentrating. His serum creatinine level was now 1.80 
mg/dL and his blood urea nitrogen (BUN) 36 mg/dL. Which of the following choices correctly 
describes the cause of this patient’s symptoms? 


1. Vasodilation of the postglomerular efferent arterioles 

2. Vasoconstriction of the preglomerular arterioles 

3. Glomerulonephritis 

4. Acute interstitial nephritis 

5. A urethral stone 


INCORRECT X 

The correct answer is 1. 

This man is suffering from acute renal failure (ARP) marked by oliguria and retention of 
nitrogenous wastes. Causes of ARP are customarily divided into three categories: prerenal, 
renal, and postrenal. Prerenal causes are those occurring in the blood supply leading up to 


the glomerulus, renal are due to events in the body of the kidney, and postrenal are due to 
obstructions in the urinary tract leaving the kidney. In the case described, the precipitating 
factor was the ingestion of enalapril. As a general rule, angiotensin-converting enzyme 
(ACE) inhibitors such as enalapril or of angiotensin II receptor blockers (ARBs) are beneficial 
and lower morbidity and mortality. However, they can trigger ARP because they inhibit the 
production or utilization of angiotensin II, which will cause vasodilation of the postglomerular 
efferent arterioles; the result is a decrease of glomerular pressure, which decreases the 
filtration rate and the production of urine, leading to acute renal failure. This usually only 
happens in persons whose vascular system is stressed in some way. The man’s system in 
this case was stressed by old age, diabetes, and evidence of “inflexible” arteries as 
demonstrated by his resistant systolic hypertension. 

(Choice 2) Because the afferent preglomerular arterioles supply blood to the kidney, 
vasoconstriction of the preglomerular arterioles also may cause prerenal ARP. Non-steroid 
anti-inflammatory drugs (NSAIDs) may induce ARP via this mechanism because they inhibit 
cyclo-oxygenase (COX), and thus inhibit prostaglandin synthesis. 

(Choice 3) rarely causes ARP, but it represents a type of renal ARP if it is quickly induced. It 
will be accompanied by systemic manifestations such as fever, rash, and arthritis. 

(Choice 4) is another type of renal ARP. This condition often results from an allergic reaction 
to one of a host of drugs, including allopurinol, cephalosporins, cimetidine, ciprofloxacin, 
furosemide, NSAIDs, penicillins, phenytoin, rifampin, sulfonamides, and thiazide diuretics. 
Several diseases can also lead to development of acute interstitial nephritis. It again will be 
accompanied by a host of systemic manifestation. As indicated, obstruction of the outflow of 
urine from the kidney can also cause postrenal ARP. 

(Choice 5) is a cause easily visualized. Other causes include prostatic hypertrophy or 
cancer, catheters, and strictures. 


23. Question 1 points 

A 54-year-old man presents to his physician for a physical examination. He is 5 ft 11 in tall and 
weighs 229 lb (body mass index [BMI] 33 kg/m 1 2 ). His blood pressure is 165/80 mm Hg, pulse is 
75/min and regular. Otherwise, results of his physical examination are unremarkable. He was 
asked to have a fasting blood sample taken for a lipid profile. The next week, the following data are 
reported: total cholesterol, 225 mg/dl_; triglyceride level, 200 mg/dl_; low-density lipoprotein, 145 
mg/dL; and high-density lipoprotein, 40 mg/dL. Because his BMI defines him as being class I obese 
and because his total cholesterol is borderline high, the physician puts him on a diet and prescribes 
a cholesterol-lowering medication. Since starting the medication, the patient experienced problems 
with constipation and a bloating sensation that is relieved by increasing dietary fiber. Which of the 
following metabolic alterations is expected from the drug he is taking? 


1. Decreased activity of hydroxymethylglutary I (HMG)-CoA reductase 

2. Decreased synthesis of triglyceride in hepatocytes 


3. Increased activity of capillary lipoprotein lipase 

4. Increased excretion of free cholesterol in feces 

5. Increased synthesis of hepatocyte low-density lipoprotein receptors 


INCORRECT X 

The correct answer is 5. 

The patient is most likely taking a bile acid-binding resin (e.g., cholestyramine) to lower his 
cholesterol level. These resins work by binding bile acids in the intestine to form an insoluble, 
nonabsorbable complex that is excreted in the feces along with the resin. This action 
reduces circulating cholesterol levels and causes a compensatory increase in the synthesis 
of hepatocyte low-density lipoprotein receptors to more efficiently recover reduced levels of 
cholesterol from the blood. Recovered cholesterol is then converted to bile acids to replenish 
the bile acids that are lost in the stool. The most common complaints are constipation and 
bloating that are relieved by increasing fiber or mixing psyllium seed with the resin. Heartburn 
and diarrhea are occasionally reported. Problems may also arise from the resin forming 
complexes and promoting the excretion of other substances such as digitalis, folic acid, 
thiazides, and warfarin. 

(Choice 4) Normally, cholesterol is effectively reabsorbed from the intestine as part of the 
bile acids and is not excreted as the free compound. A bile acid-binding resin does not 
change this condition; however, now rather than being reabsorbed as an integral part of the 
bile acid, it is excreted as a component in a bile acid-bile acid binding resin complex, not as 
free cholesterol; increased excretion of free cholesterol in feces does not happen. 

(Choices 1,2 & 3) The “statin” drugs (e.g., lovastatin) inhibit HMG-CoA reductase (Choice 
1), the rate-limiting enzyme of cholesterol synthesis. This decreases the synthesis of 
cholesterol. Rare side effects of statin drugs include hepatic toxicity with an increase in 
transaminases, and a myopathy with an increase in creatine kinase; however, evidence 
suggests that the statins are also anti-osteoporotic, an unexpected boon. Fibric acid 
derivatives (e.g., gemfibrozil) and nicotinic acid lower serum triglyceride (TG) levels. Fibric 
acid derivatives decrease hepatic TG synthesis (Choice 2) and also interfere with the 
formation of very-low-density lipoproteins (VLDLs) in the liver. Complications that might be 
associated with fibric acid derivatives include rash and myopathy, the latter sometimes 
causing rhabdomyolysis with marked elevation of creatine kinase and myoglobinuria. 
Nicotinic acid is also used in the treatment of hypertriglyceridemia (also 
hypercholesterolemia). The drug interferes with the formation of VLDL in the liver. Flushing is 
the major side effect of nicotinic acid and is diminished by taking a nonsteroidal anti¬ 
inflammatory drug 30 minutes before taking nicotinic acid. Flushing is not present in this 
patient. Fibric acid derivatives and nicotinic acid both activate capillary lipoprotein lipase 
(Choice 3), promoting increased delivery of fatty acids to the adipose. 







24. Question 


1 points 


A 42-year-old man complains of headaches when he wakes up in the morning. He reveals that has 
had these headaches for maybe as long as a year, but they have been occurring more often and 
are getting progressively worse. He also feels as though he is losing his strength and that he 
sometimes has trouble placing his feet where he wants to without looking down at the ground. The 
physician observes him to have an unusually prominent jaw with spaces between the teeth, and 
large hands and feet. By asking him to press against his arm, the physician confirms muscle 
weakness. Upon physical examination, his blood pressure was found to be 135/95 mm Hg, and his 
non fasting blood glucose value was 195 mg/dl_. Which of the following would be expected in this 
patient? 


1. Chest radiograph with normal-sized heart 

2. Normal-sized sella turcica 

3. Lack of suppression of glucose with an oral glucose challenge 

4. Decreased concentration of insulin-like growth factor-1 

5. Decreased serum growth hormone 


INCORRECT X 

The correct answer is 3. 

The patient has acromegaly secondary to a benign pituitary adenoma with excess secretion 
of growth hormone {not decreased (Choice 5)} by the anterior pituitary and of insulin-like 
growth factor-1 {not decreased (Choice 4)} from the liver. If the condition occurs before 
fusion of the epiphysis, gigantism occurs. 

Clinical findings associated with acromegaly include: 

o Generalized enlargement of bone, cartilage, and soft tissue, resulting in large hands 
and feet, frontal bossing, a prominent jaw, an increase in hat size, spaces between the 
teeth, and hypertrophy of the left ventricle observed by chest radiography {not a 
normal-sized heart (Choice 1). This hypertrophy may lead to cardiomegaly and 
cardiomyopathy with congestive heart failure (the most common cause of death), 
o Diastolic hypertension (observed blood pressure is 135/95 mm Hg) 
o Muscle weakness (his perception confirmed by the physician) 
o Peripheral neuropathies (he can’t be sure where his feet are, loss of proprioception) 
o Diabetes mellitus from the gluconeogenic properties of growth hormone 
o Headaches and visual field defects from encroachment on the optic chiasm 
The laboratory findings include: 

o Hyperglycemia (40%) (his serum glucose level is 195 mg/dL) 
o Inability to suppress glucose with an oral glucose tolerance test (Choice 3) 
o A paradoxical increase of serum growth hormone concentration with injection of 
thyrotropin releasing hormone (TRH) 

o No stimulation of growth hormone release with L-dopa (normal persons have an 
increase in GH) 


o Before fusion of the epiphysis occurs, increased serum phosphate associated with 
growth spurts 

o Enlargement of the sella turcica in more than 90% of patients {not a normal-sized sella 
turcica (Choice 2)} 

Treatment consists of transsphenoidal Surgery or the use of a somatostatin analogue called 
octreotide, which produces clinical improvement in 70% of cases. 


25. Question 1 points 

For the past 6 months, a 55-year-old man with a 35year history of smoking cigarettes has 
complained of persistent frontal headaches. For several days, he has had projectile vomiting and 
bilateral blurry vision. Physical examination shows bilateral papilledema. A magnetic resonance 
imaging (MRI) study shows a mass lesion involving the corpus callosum that has spread to both 
cerebral hemispheres. The patient dies a few days later of a transtentorial herniation. Which of the 
following underlying conditions is the most likely cause of the lesion? 


1. Cerebral infarction 

2. Glioblastoma multiforme >/ 

3. Intracerebral hemorrhage 

4. Medulloblastoma 

5. Metastatic lung carcinoma 


INCORRECT X 

The correct answer is 2. 

The patient has a glioblastoma multiforme. This is the most common primary cancer of the 
brain in adults (peaks in the 40 to 70-year-old age bracket) and either arises de novo or from 
a preexisting low-grade astrocytoma. The tumors are located predominantly in the cerebral 
hemispheres and are classified as grade IV astrocytomas. There is no causal relationship 
with cigarette smoking. The distribution of this lesion and the history of headache, projectile 
vomiting, and bilateral papilledema are classic signs and symptoms of glioblastoma 
multiforme. The magnetic resonance imaging (MRI) scan shows a mass lesion extending 
across the splenium of the corpus callosum and into the adjacent cerebral hemisphere 
bilaterally. These tumors rarely metastasize out of the neuraxis. Radiation and chemotherapy 
are used for treatment. There is a 25%-40% 5-year survival rate. 

(Choice 1) are most commonly caused by a thrombus overlying an atheromatous plaque, 
which causes a pale infarction that extends to the periphery of the cerebral cortex. The 
lesion in this patient does not adhere to this distribution. 




(Choice 3) usually are due to hypertension (not recorded in this patient), which produces 
small-vessel aneurysms that result in rupture of the vessel and hemorrhage into the brain. 
The basal ganglia area is the most common site for the hemorrhage. 

(Choice 4) usually are located in the midline of the cerebellum in children. 

(Choice 5) Metastases to the brain usually are multifocal and occur primarily at the junction 
of the gray and white matter near the periphery of the brain. 


26. Question 1 points 

An 18-year-old man enlisted in the Marines. He appeared to be in good physical shape, but his 
buddies sometimes remark about his unusual thirst, and he was annoyed by the fact that he often 
became so thirsty at night that he had to get up and drink a couple of glasses of water. Not 
surprisingly, he also had a need to urinate frequently. Since he acquired these traits around the age 
of 10, he did not regard them as being abnormal, just slightly annoying; his megathirst apparently 
started shortly after he fell from a tree and was knocked unconscious for a short time. He did fine 
during basic training until his unit went on a forced march during a hot August week. By the first 
night, he had emptied his canteen and suffered such a severe thirst that he actually woke up his 
sergeant begging for extra water. Luckily, his sergeant was a compassionate man and recognized 
something was truly wrong. In addition to his desperate pleading, he seemed too weak to stand 
straight at attention, looked and felt feverish, and his eyes appeared to have sunk deep into his 
forehead. Consequently, he was excused from the march and sent to the sickbay. Once there, his 
symptoms were recognized as dehydration, and he was given water and sent to the base hospital 
for further evaluation. The medics there quickly made a diagnosis and prescribed which of the 
following treatments? 


1. Metformin 

2. Foscarnet 

3. Desmopressin 

4. Increasing his daily water intake 

5. Demeclocycline 


INCORRECT X 

The correct answer is 3. 

Clearly, this young man has a polydipsia/polyuria (excessive drinking/urinating) problem. 
Desmopressin is a vasopressin (also known as, the antidiuretic hormone) analog (1- 
deamino-8-D-arginine vasopressin) and is the only choice presented that would directly 
inhibit water loss into the urine. The word diabetes means to have an abnormal need to 
urinate. There are two basic types of diabetes, the more common “sweet” type, diabetes 




mellitus, and the insipid, or “tasteless” type diabetes insipidus. Physicians presented with 
such a patient would order an electrolyte panel to help diagnose his condition, but even 
without additional data, common sense indicates this patient does not have diabetes mellitus 
{and therefore does not need metformin (Choice 1) since he has had this condition for many 
years and is healthy except for his abnormal thirst; it follows he must have diabetes 
insipidus. However, there are five recognized types of diabetes insipidus: 

1. Central (also known as, neurogenic or pituitary), in which the hypothalamus has lost 
the capacity to synthesize vasopressin; this condition may be idiopathic or due to 
some sort of injury, physical or pathological (tumors or infection). 

2. Nephrogenic, in which the kidney tubules have lost functional binding sites for 
vasopressin; this may be caused by kidney disease, drugs that inhibit binding, or in 
very rare cases, it is inherited. 

3. Dipsogenic, in which the thirst-regulating part of the brain is damaged. 

4. Gestational, caused by the placenta’s destruction of vasopressin. As a rule, recovery 
is spontaneous within 24 days after birth, although an affected woman is at high risk to 
develop another case of diabetes insipidus during subsequent pregnancies. 

5. Psychogenic, in which a patient has an obsessive compulsion to drink large quantities 
of water. 

Usually, a physician will measure blood glucose, bicarbonate, and electrolyte levels, as well 
as urinary osmolarity, as the first step in confirming diabetes insipidus. Typically, urine 
os molarity and electrolyte levels are lower than normal and the degree to which 
hypernatremia occurs reflects the degree of dehydration. A fluid deprivation test helps 
determine if the diabetes is due to lack of vasopressin (central), to the kidney’s insensitivity 
to vasopressin (nephrogenic), or to excessive fluid ingestion (dipso or psychogenic). 
Normally, as fluid deprivation proceeds, dehydration starts to occur and the kidney 
compensates by excreting a lesser volume of more concentrated urine; consequently, the 
body retains a greater volume of water. Patients with diabetes insipidus fail to concentrate 
their urine; however, patients having central diabetes insipidus will start to concentrate their 
urine after the administration of desmopressin. This will not occur if the patient has 
nephrogenic, dipsogenic, or psychogenic diabetic insipidus. 

(Choices 2 & 4) Foscarnet, an antibiotic used to treat cytomegalovirus infection, has 
recently been shown to cause nephrogenic diabetes insipidus in certain patients and 
consequently will not likely be used to treat any form of diabetes insipidus. Increasing his 
daily water intake (Choice 4) also would be counterproductive and might lead to water 
intoxication caused by hyponatremia. 

(Choice 5) is a broad-spectrum tetracycline antibiotic and the only tetracycline with the 
property of competing with vasopressin for its binding sites on the renal tubules, thus it too 
may cause a transient diabetes insipidus and will not likely be used to treat diabetes 
insipidus. 


27. Question 


1 points 


While on a camping trip with his son in a remote location in Alaska a 69-year-old man accidently got 
a fish hook imbedded in the palm of his hand. They dug out the hook, washed the wound, and 
bandaged it. Apparently, they did not clean it out adequately because when they got back home a 
week later, it showed signs of infection. A doctor at the local ER cleaned it out thoroughly and gave 
him a shot of penicillin. The wound healed nicely but several weeks later, the man developed a mild 
fever, which persisted in the absence of any other symptoms. After a month, his temperature 
suddenly peaked and he developed excessive sweating, chills, fatigue, muscle and joint pains, 
night sweats, and painful lesions on the pads of the fingers. Which of the following is the most likely 
cause of this man’s symptoms? 


1. Infection by Staphylococcus aureus 

2. Infection by Streptococcus viridans 

3. Infection by Candida albicans 

4. Infection by Streptococcus bovis 

5. Infection by Clostridium septicum 


INCORRECT X 

The correct answer is 1. 

Bacteria that infected this man’s wound multiplied, entered his circulatory system, and 
localized in one of his heart valves. Initially, this caused a case of subacute endocarditis 
marked by a low-grade fever. The infection then fulminated, causing symptoms commonly 
associated with acute infectious endocarditis, including a higher temperature, excessive 
sweating, chills, fatigue, muscle and joint pains, night sweats, and painful lesions, called 
Osier’s nodes, on the pads of the fingers. Infections initiated by breaks in the skin are often 
caused by Staphylococcus aureus. In this case, because the shot of penicillin received at 
the ER did not prevent the development of endocarditis, it is likely that this was a methicillin- 
resistant strain. Unless faced with a massive infusion of organisms, skin breaks as a rule do 
not cause endocarditis because healthy valves are smooth and don’t provide a surface for 
organisms to adhere to. However, in older individuals, one or more valves are sometimes 
affected by arthrosclerosis and become roughened by calcification; this provides a place for 
organisms to anchor and proliferate, as presumably happened in this gentleman. This is the 
reason why the incidence of endocarditis is higher in the elderly. 

(Choice 2) Streptococcus viridans is an organism found in the mouth and is associated with 
endocarditis after dental work. Prior to October 2007, dentists were advised to administer 
penicillin to patients at risk for heart infection as prophylaxis to prevent endocarditis. 
However, at that time, the American Heart Association published an article in Circulation 
recommending that most of these patients no longer get short-term antibiotics as a 
preventive measure before their dental treatment. The consensus was that the risks of taking 
preventive antibiotics outweigh the benefits for most patients. These risks include adverse 


reactions to antibiotics that range from mild to potentially severe and, in very rare cases, 
death. Inappropriate use of antibiotics can also lead to the development of drug-resistant 
bacteria. 

(Choice 3) Infection by the yeast Candida albicans most commonly occurs in IV drug users; 
these infections commonly enter the right heart and often infect the tricuspid valve. 

(Choices 4 & 5) Streptococcus bovis and Clostridium septicum are part of the natural flora 
of the colon and cause endocarditis if the bowel is pierced or in colon cancers, rarely from 
skin punctures. 


28. Question 1 points 

An internist at a major medical center received a letter from a physician located far away from his 
place. The letter said: “With your permission, I am asking a former patient of mine to see you. He is 
an 18-year-old man suffering from occipital bone syndrome. Physically, he has the pathognomonic 
bilateral exostosis of the skull and pili torti, extremely loose skin and joints, long neck, 
dysautonomia, and vascular tortuosity, as well as a somewhat unusual appearance including an 
abnormally long neck and face, with a high forehead and an arched palate. He also suffers from 
seizures, which are presently under control by medication. He has a very low normal IQ of 88.1 am 
sure you will find his case interesting and his continued treatment challenging. If you accept him as 
your patient, I will send you his medical records.” The patient described in this letter has an 
aberration in the metabolism of which one of the following trace elements? 


1. Zinc 

2. Selenium 

3. Chromium 

4. Copper 

5. Cobalt 

6. Manganese 


INCORRECT X 

The correct answer is 4. 

There are three inborn errors of copper metabolism: Wilson disease, Menkes disease, and 
occipital horn syndrome. Both occipital horn syndrome (once known as Ehlers-Danlos 
syndrome IX and sometimes called X-linked cutis laxa) and Menkes syndrome (also known 
as, kinky hair syndrome) are caused by mutations in ATP7A located on Xq12-13. The 
normal gene has 23 exons and encodes a P-type copper transport protein with ATPase 
activity. Mutations in the splicing sites may result in insertions or deletions and nonsense 
rearrangements. Such errors result in greater distortion of the protein product in patients 


having Menkes disease than they do in patients with occipital horn syndrome; thus, boys 
afflicted with Menkes disease are by definition sicker than those with occipital horn 
syndrome. 

Male Menkes babies tend to be born a little prematurely; they seem normal at first, but then 
spiral into a tragic decline. Usually by about the third month, the child has an epileptic event 
and his appearance takes on the typical manifestation of the disease, which includes light, 
often steely colored, kinky hair that is sparse on the sides, plus an unusual appearance. 

Prom that time on, the child fails to thrive and degenerates neurologically and physically, 
eventually dying, generally by the third year. The normal Menkes gene product exists in both 
a long and short form, with different tissue distribution. The shorter form is expressed in 
occipital horn syndrome, and this probably accounts for the milder phenotype, which is 
similar to the one described in the case history. Formerly, most afflicted boys died in their 
early teens but now commonly live well into their 20s. 

The normal Menkes and Wilson disease genes have about 55% identity, and both code for 
copper-transporting proteins, but their distribution and the function of their gene products are 
different. The underlying function of the normal Menkes gene product is to bring copper ion 
into the body and distribute it among cells; in contrast, the function of the normal Wilson gene 
product is to remove excess copper ion from the body. Consequently, the normal Wilson 
disease gene is expressed predominantly in the liver, whereas the normal Menkes disease 
gene is not expressed to a significant extent in liver but is in most other tissues; moreover, 
serum copper ion levels are lower than normal in both Menkes disease and occipital horn 
syndrome, but raised in Wilson disease. 

(Choice 1) A rare autosomal recessive condition called acrodermatitis enteropathica (also 
known as, Brandt syndrome, Danbolt-Cross syndrome or congenital zinc deficiency) causes 
a congenital form of zinc deficiency. Zinc and copper are the only trace elements associated 
with known inborn errors of metabolism. Zinc is a cofactor for as many as 300 enzymatic 
reactions; hence, a deficiency can have profound effects. Patients having acrodermatitis 
enteropathica who are not treated by lifelong dietary zinc supplementation die within a few 
years. Acquired deficiencies are more common; the deficiency may be due to dietary 
insufficiency (either because of poor nutrition or to conditions such as anorexia nervosa or 
alcohol abuse), a decreased ability to absorb the ion (either because of intestinal disorders or 
sometimes associated with aging), and in patients on complete parenteral nutrition, 
insufficient zinc allowance. Deficiency states in developed counties are generally modest 
and the effects subtle. An obvious effect is in limiting the growth of children. Earlier studies in 
several U.S. communities showed that dietary supplementation with zinc induced growth 
spurts, thus indicating that these children had a nutritional deficiency. Dermatologic changes 
(e.g., rashes) are another readily recognized consequence of zinc deficiency. Loss of acuity 
of taste (dysgeusia) or smell (anosmia) caused by a reduced ability to absorb zinc has been 
observed among aged individuals. Chronic zinc deficiency also increases susceptibility to 
infection by reducing immunologic efficiency and also by reducing the efficiency of wound 
healing because it is a cofactor for collagenase, which is required to Resorb type III collagen 
so that it may be replaced by type I collagen. As a rule, these acquired deficiencies can be 
reversed by zinc supplementation, but with a note of caution: taking large quantities of zinc 
can reduce the bioavailability of copper because zinc induces the synthesis of a metallo 
thioneine that binds copper within intestinal cells and prevents its absorption. Thus, 
individuals taking zinc supplements are advised to also take a copper supplement. 






(Choice 2) is a cofactor for glutathione peroxidase (which neutralizes hydrogen peroxide and 
peroxide free radicals in cells), thioredoxin reductase (which indirectly reduces certain 
oxidized molecules), and three deiodinase enzymes (which interconvert forms of thyroid 
hormone). Its glutathione peroxidase activity helps reduce free radicals and is thought to act 
to reduce the risk of cancer. A frank selenium deficiency also predisposes patients to 
Keshan disease, an endemic viral cardiomyopathy primarily affecting children and young 
women. At high doses, selenium is toxic, causing hair loss, abnormal nails, dermatitis, 
peripheral neuropathy, nausea, diarrhea, fatigue, irritability, and a garlicky breath odor. 
(Choice 3) is another essential trace element required by humans. It is almost ubiquitous in 
nature, making deficiency states essentially unknown. Promotional literature from 
supplement distributors may claim chromium supplements can reduce serum cholesterol 
levels, prevent or help alleviate symptoms associated with diabetes, reduce body fat, and 
help build muscle, but there is little evidence to substantiate such claims. Nonetheless, 
chromium is a component of glucose tolerance factor, and consequently does help insulin 
make glucose available to cells by facilitating the binding of insulin to its receptors located in 
adipose and muscle. Thus, if a deficiency of chromium truly did exist, it might be associated 
with impaired glucose tolerance. 

(Choice 5) The human requirement for cobalt is not for the ionic form of the metal, but for a 
preformed metallo vitamin that cannot be synthesized from dietary metal. Therefore, it is the 
vitamin B12 content of foods in the diet that is of importance in human nutrition. 

(Choice 6) is a cofactor for arginase, pyruvate carboxylase, glutamine synthetase, and 
manganese superoxide dismutase (SOD), and is the preferred metal cofactor for 
glycosyltransferases. No deficiency states for manganese have been established. 


29. Question 1 points 

A poorly dressed 56-year-old lady enters an emergency department facility at a local hospital in an 
agitated manner saying she needs attention immediately because she just discovered she has 
colon cancer. The triage nurse calms her down a bit by asking her what makes her think that is 
true. She responds: “For the past week, each time I make number two, I get a sharp pain, and 
because the pain lasts for around a half hour after I wipe myself I know it must be coming from my 
insides. The worst was today when I saw bright red blood; I heard on the TV that blood when you 
go to the bathroom is a sign of cancer.” The nurse tells her that there are also many other additional 
causes for blood in the stool. She finds that the lady is hypertensive but has not seen a physician 
since she was pregnant at the age of 18 years; the nurse then arranges for the woman to see one 
of the ER doctors. When the doctor sees the patient, he arranges for her to see a primary care 
physician but also recommends treatment. Which of the following choices is the most probable 
treatment recommended? 


1. Removal of a mass that may be protruding from the patient’s anus 

2. Perform a sigmoidoscopy and during this procedure excise any polyps found 


3. Application of a local steroid cream twice daily, plus use of stool softeners and hot 
sitz baths V 

4. Prescription of antihypertensive drugs and antibiotics 

5. Cautery by argon plasma coagulation applied via an endoscope 


INCORRECT X 

The correct answer is 3. 

This lady most likely suffers from an anal fissure, for which the classical treatment is 
application of a local steroid cream twice daily, plus use of stool softeners and hot sitz baths. 
Such a fissure results from the passage of a large, hard bolus of stool and is a common 
cause of rectal bleeding. Severe pain is an important symptom, and the condition is 
confirmed by observation of a semi-epileptical defect in the anal skin running in a radial 
direction. The treatment described usually results in complete recovery. 

(Choice 1) A mass protruding from the patient’s anus is usually due to a prolapsed internal 
hemorrhoid and generally is caused by straining to defecate. The mass generally retracts 
spontaneously and is rarely accompanied by pain unless it is thrombosed. 

(Choice 2) There is no call for a sigmoidoscopy, and polyps cannot be removed during a 
sigmoidoscopy. 

(Choice 4) Prescription of antihypertensive drugs and antibiotics is a therapy used to treat 
relatively mild cases of ischemic colitis. Control of hypertension will facilitate blood flow into 
the colon, while the antibiotics will help prevent infection. 

(Choice 5) Idiopathic bleeding from the bowel due to dilated blood vessels in the mucosa is 
called angiodysplasia and may be initially treated by cautery employing argon plasma 
coagulation applied via an endoscope. 


30. Question 1 points 

A 79-year-old man with a long history of systolic hypertension was enrolled in a physical therapy 
session because of chronic back pain. For a reason that was unknown at the time, it was becoming 
increasing difficult for him to perform exercises involving raising his legs. As a result, the therapist 
decided to massage his legs and noted they were distended with edema. Consequently, he made 
an appointment with his primary care physician, who performed an electrocardiogram (ECG); this 
was essentially normal. The physician also prescribed a diuretic and several tests, and referred 
him to a cardiologist. Which one of the following tests will best provide quantitative evidence 
defining the severity of the problem suspected by the primary care physician? 


1. Atrial natriuretic peptide (ANP) 

2. Brain type (B-type) natriuretic peptide (BNP) 




3. C-type natriuretic peptide 

4. A chest x-ray 

5. A complete blood count (CBC) 


INCORRECT X 

The correct answer is 2. 

The onset of edema is a possible symptom of congestive heart failure, which in turn may be 
caused by longterm hypertension and/or arthrosclerosis. This probable diagnosis needs to 
be confirmed, as must the degree of the problem. Among the tests outlined, this can best be 
done by determining the level of brain type (B-type) natriuretic peptide (BNP) in the serum. 
The natriuretic peptides are a family of polypeptides that cause the excretion of sodium into 
the urine; that is, they promote natriuresis. The different forms of the natriuretic peptides are 
named by the tissues from which they were first isolated. Even though BNP was first 
isolated from brain, its medical significance lies in the fact that this peptide is released from 
the heart’s ventricles in response to excess stretching of the ventricular myocytes as often 
occurs in the stressed heart in congestive heart failure. In addition to stimulating natriuresis, 
this peptide also decreases systemic vascular resistance, thus causing a decrease in 
central vascular pressure, inducing and permitting a decrease in cardiac output. 

At first, it was thought that measuring the serum level of BNP would provide a quantitative 
estimate of the degree to which the heart is damaged, but at best it is semiquantitative and 
conditions other than congestive heart failure may also increase the activity. The most 
definitive test of heart function and thus the best test to estimate the extent to which the heart 
is damaged is a stress echocardiogram. However, the measurement of the serum level of 
the BNP can be done in the doctor’s office or at the bedside while an echocardiogram, in 
particular a stress echocardiogram, takes special equipment that is not always readily 
available, particularly in a primary care physician’s office or in remote areas and Third World 
countries. In addition to using BNP as a diagnostic aid, a recombinant human BNP, 
nesiritide, has been approved for use in the acute treatment of decompensated congestive 
heart failure caused by systolic dysfunction. Unfortunately, it must be administered IV and 
has a very short half-life. 

(Choice 1) acts similarly to BNP but is released from the atrium. However, its half-life in the 
serum is much shorter than that of BNP, which limits its usefulness as a diagnostic tool. 
(Choice 3) is also found in brain and may play a role similar to BNP but its medical value has 
not yet been established. 

(Choice 4) can show an enlarged heart but that in itself is not diagnostic of congestive heart 
failure nor does it provide even semiquantitative information concerning severity. 

(Choice 5) is irrelevant. 


31. Question 


1 points 







A 48-year-old patient presents to the emergency room with a history of tiredness, vomiting, and 
abdominal pain. He has a long history of hypertension, diabetes mellitus, and hyperlipidemia, and 
during this past year, he developed end-stage renal disease. He admits to being depressed of late 
and has not kept his appointment with the nephrologist for renal dialysis, which he used to go to 
three times a week. The patient is on medications for hypertension, diabetes, and hyperlipidemia, in 
addition to medications for renal failure. Which of the following is the initial medication that should be 
administered to reverse the clinical condition reflected by the accompanying electrocardiogram 
pattern? 


AMC Medicine MCQs 


(https://www.amcquestionbank.com/wp- 


content/uploads/2015/11/Picture-Test-6-Q08.png) 


1. Sodium bicarbonate 

2. Furosemide 

3. Calcium gluconate 

4. Glucose plus insulin 

5. Cation-exchange resin 


INCORRECT X 

The correct answer is 3. 

The electrocardiogram (ECG) pattern shown indicates that this patient has hyperkalemia. It 
reveals tall, slender, tented T waves in leads I, II, aVF, and V2 through V6. Other findings 
suggesting hyperkalemia include a widened QRS complex and even biphasic QRS-T 
complexes. Although hyperkalemia produces these characteristic patterns, the ECG is not a 
sensitive indicator of hyperkalemia, as almost 50% of patients with serum potassium levels 
above 6.5 mEq/L do not show any changes in the ECG pattern. This is because atrial cells 
are more sensitive than ventricular cells to elevated levels of potassium, permitting normal 
conduction even if atrial depolarization is inhibited: as a result, a junctional rhythm can result. 
To avoid dangerous arrhythmias and asystole, hyperkalemia above 6.5 mEq/L warrants 
immediate correction. In this case, hyperkalemia also is suspected clinically because the 
patient has end-stage renal disease, has not been undergoing dialysis, and has a history of 
tiredness, vomiting, and abdominal pain. Hyperkalemia could, in addition, have caused motor 
paralysis. Calcium gluconate 10% or 5% calcium chloride is the initial drug of choice. 
Approximately 5-30 mL of either administered intravenously provides calcium ion, which acts 
within minutes to antagonize cardiac conduction abnormalities: the effect lasts approximately 
an hour. In patients who are on digoxin, one must ensure that they do not have digoxin 
toxicity; if that were the case, calcium would only increase its toxic effects on the 
myocardium. 

(Choice 4) Although administering calcium has a rapid effect, it does not promote the shift of 
potassium into the cells; consequently, its effect is transitory. To secure a more permanent 
effect, one usually follows up with insulin and glucose. This drives potassium into the cells, 



thereby lowering serum potassium levels. Regular insulin (5-10 units) combined with 25 g of 
50% glucose is administered intravenously. Insulin takes about 15 minutes to an hour to act, 
and the action lasts anywhere from 4 to 6 hours. 

(Choice 1) Use of sodium bicarbonate is an alternative to insulin plus glucose. It creates a 
metabolic alkalosis, causing potassium ion to enter the cells in exchange for hydrogen ions. 
One or two ampules of sodium bicarbonate (approximately 44 mEq each) can be 
administered intravenously. Its effects begin in approximately 15-30 minutes and last 
anywhere from 1 to 2 hours. Thus, it is not as long lasting as insulin. 

(Choice 2) The use of furosemide is inappropriate in an emergency situation because it 
takes anywhere from 30 minutes to 2 hours to lower potassium levels, and one may very 
well not have the luxury of time. It is a loop diuretic that exchanges sodium for potassium, 
expelling the latter in the urine. 

(Choice 5) Like loop diuretics, sodium polystyrene sulfonate in 20% sorbitol, a cation 
exchange resin, may be used in nonemergency situations; 15-30 mL is administered orally or 
rectally. Potassium ions bind to the resin, lowering serum potassium levels. Due to the mode 
of administration, it takes much longer to act; its action lasts approximately 3 hours. 


32. Question 1 points 

A 40-year-old man presents to a physician complaining of erectile dysfunction. In taking a history, it 
is determined that he started drinking alcohol at the age of 14 years and has continued since. 
Presently, he starts his day by having a beer or two with his breakfast, and he carries a flask of 
whiskey with him to help him get thorough the day. In the evening, he often polishes off a pint of 
whiskey before going to bed. However, he also states that he has a “hollow leg,” and the alcohol 
doesn’t affect his functioning in any way. Physical examination reveals a distended abdomen and 
dependent pitting edema. He also has numerous radially oriented vessels around a central core on 
his face, neck, and upper trunk. Which additional physical finding in this patient has the same 
pathogenesis as the skin lesion? 


1. Ascites 

2. Asterixis 

3. Caput medusae 

4. Esophageal varices 

5. Gynecomastia 


INCORRECT X 


The correct answer is 5. 

The patient has cirrhosis of the liver (history of alcohol abuse, ascites, and dependent pitting 
edema). The skin lesions are spider angiomas, which have a central spiral arteriole with a 
group of small vessels radiating from the arteriole. Spider angiomas are associated with 
hyperestrinism, which is a complication of cirrhosis. In cirrhosis, the dysfunctional liver is 
unable to metabolize estrogen, which produces hyperestrinism. Hyperestrinism in men 
causes gynecomastia (development of breast tissue in males) and female secondary sex 
characteristics (palmar erythema, soft skin, and female hair distribution). 

(Choice 1) Although the distended abdomen is due to ascites, the question asks for 
additional symptoms having the same pathogenesis as the skin lesion. The factors that 
contribute to the development of ascites include portal hypertension (increase in hydrostatic 
pressure), hypoalbuminemia (decrease in oncotic pressure), secondary aldosteronism (salt 
retention), and increased lymphatic drainage into the peritoneal cavity. 

(Choice 2), or flapping tremor, refers to the inability to sustain posture. It is a sign of hepatic 
encephalopathy, which is caused by an increase in ammonia and false neurotransmitters 
(e.g., -aminobenzoic acid). 

(Choice 3) are dilated periumbilical veins that are associated with increased venous 
pressure caused by portal hypertension. 

(Choice 4) are dilated left gastric coronary veins, which normally drain the distal esophagus 
and proximal stomach and empty into the portal vein. An increase in portal vein pressure 
leads to dilation of the gastric veins (varices), which commonly rupture. 


33. Question 1 points 

A 23-year-old male prelaw student presents to the ER with the complaint of an itch and rash in his 
genital region. In providing a history, he states that the itching is worse at night, making avoidance 
of scratching almost impossible. He adds that the morning after his first night of discomfort, he 
noted a few small red blisters and bumps on his penis and while trying to treat the condition he 
bathed and applied hand lotion. The next night, the itching was even more intense, and in the 
morning, he noted the head of his penis was fully involved and the rash had spread to the 
surrounding genital area. This induced him to seek medical help. The attending physician asks if 
the student had had sex within the past 4-8 weeks? The student tells him that he did have sex with 
a young woman he met at a bar about a month ago; however, he felt he was protected since he 
wore a condom. The attending physician replies, “I am 99% sure I know what ails you, and I am 
going to write a prescription. If I am correct and you follow the directions, your condition should 
clear up within a few days. If it doesn’t, let me know; otherwise, there should be no problem. I would 
also like to talk to the young lady if that is possible.” Which of the following choices most likely 
describes the prescription given? 


1. Topical permethrin 5% 

2. Oral azithromycin 


3. Oral ivermectin 

4. Topical lindane (-benzene hexachloride) 1% 

5. Parental penicillin G 


INCORRECT X 

The correct answer is 1. 

The patient suffers from a case of scabies caused by a small, almost microscopic (0.3-0.9 
mm) mite called Sarcoptes scabei, variety hominis. The recommended treatment for adults is 
5% permethrin lotion, applied on a clean body from the neck to the toes and left for at least 8 
hours, after which it can be washed off. A second treatment is recommended a week later. 
To ensure that all eggs and mites are killed, all clothing, bedding, towels, etc. used by the 
infected person should be washed in hot water and dried in a hot dryer before treatment 
begins. No special cleaning is needed for rugs, floors, coats, and furniture because the mites 
cannot survive away from a human body. When patients are infested, pregnant female mites 
burrow into the stratum corneum and deposit eggs. In 3-10 days, the eggs hatch into larvae, 
which then molt and mature into a new generation of adults via a nymphal stage. The eggs 
produce an allergic reaction that is intensified by the action of the maturing mites; this 
induces the rash and the itch. A first-time infection may take as long as 8 weeks for the full¬ 
blown allergic reaction to develop. On the other hand, a person sensitized by a previous 
infection will develop symptoms much more rapidly. The mites, and consequently scabies, 
are transmitted readily by skin-to-skin contact with an infected person. Thus, clusters of 
cases often occur within a household or a health care facility. Obviously, sexual intercourse 
involves skin-to-skin contact, thus causing scabies to be classified as a sexually transmitted 
disease. It is quite possible that this patient’s partner picked up a few mites while working as 
a nurse and has already shown signs of scabies on her hands, a common site for early 
scabies to appear. Perhaps she only recognizes it as unusually red and rough hands and is 
spreading the disease among other patients; hence, the value of informing her that she is a 
vector. An epidemic of scabies in a health care facility can create havoc. Absolute diagnosis 
depends upon isolation and microscopic identification of active mites. However, it is more 
practical to make a tentative diagnosis based upon appearance and confirmed by response 
to treatment. Treatment as described above results in a 100% cure rate 
(Choice 2) is an antibiotic commonly used to treated chlamydial infections. Scratching 
scabies infection can sometimes lead to thick scaling, particularly in immunocompromised 
persons. Such a condition is called Norwegian scabies, and the extensive scaling makes 
topical medication ineffective. 

(Choice 3) Consequently, Norwegian scabies is treated with ivermectin, a broad-spectrum 
antiparasitic medication. 

(Choice 4) is an organochlorine insecticide once used as the primary treatment for scabies. 

It functions as a neurotoxin by interacting with -aminobutyric acid (GABA) A receptor. In 
humans, it has a dermal LD50 of 1,000 mg/kg and has also been reported to be a carcinogen 
in rodents. Because of potential toxicity, it is now banned in 50 countries and approved by 
the U.S. Food and Drug Administration only as a second-line treatment for scabies or for 
pubic (crabs) and head lice. 


(Choice 5) is an antibiotic often used to treat primary syphilis infections but has no role in 
treating primary scabies infections. 


34. Question 1 points 

For approximately the past two decades, a 62-yearold man had been a successful office manager 
at a new car dealership; for most of this time, he had his first alcoholic drink of the day upon arising 
and one or two more before arriving at work, then easily finishing off another pint of bourbon after 
leaving work and before going to bed. He felt alcoholic drinks had little effect on his ability to 
function and bragged about having a “hollow leg.” Until recently, he had never had anything to drink 
while at work, and despite his extreme drinking habits, his work had not suffered. Lately, however, 
he has had an irresistible urge to indulge in a drink or two or more, even during the workday, and he 
began to exhibit symptoms of impaired performance. Moreover, he had been caught telling untruths 
to cover up shortcomings in his performance. Consequently, the owner of the dealership threatened 
to fire him unless he admitted himself into an alcohol and drug rehabilitation center. As part of his 
admission to such a center, he underwent a thorough physical examination during which an 
abnormality present in most heavy drinkers one that may be reversed by abstinence was 
uncovered. This abnormality most likely was which one of the following? 


1. Alcoholic hepatitis 

2. Liver cirrhosis 

3. Portal hypertension 

4. An increase in the serum alanine aminotransferase (ALT) activity greater than an 
increase in the serum aspartate aminotransferase (AST) activity 

5. Wernicke disease (Wernicke’s encephalopathy) 

6. Korsakoff psychosis (Korsakoff syndrome) 

7. Fatty liver 


INCORRECT X 

The correct answer is 7. 

Alcohol abuse and alcoholism are major problems in the Australia, estimated to affect over 
8% of the adult population. Alcoholism, also known as alcohol dependence, is a disease that 
includes an almost irresistible craving for a drink; an inability to stop drinking once started; 
physical dependence marked by withdrawal symptoms such as nausea, sweating, shaking, 
and profound anxiety once drinking is stopped; and, a requirement to drink greater amounts 
to get a high as time progress, a phenomena called tolerance. Alcohol abuse is defined as 
drinking alcohol to such a degree that it causes personal harm, socially, physically, or with 


the law as in accumulating drinking under the influence of alcohol (DUIs); however, in 
contrast to true alcoholism, physical dependence is not a characteristic. Whether a person is 
afflicted with alcoholism or abuse, drinking large quantities of alcohol causes many different 
pathological changes in the drinker’s body. These abnormalities probably affect all heavy 
drinkers, although there is great variability regarding individual responses; thus, the pattern of 
symptoms shown varies among different individuals. Fatty liver is an abnormality found to be 
present in almost all heavy drinkers; fortunately, it frequently can be reversed by abstinence. 
Although fatty liver itself may not cause permanent damage, it is a symptom of abuse and a 
strong warning indicating a person should stop drinking before permanent damage is done. 
Generally, it is indicated by a slightly enlarged liver accompanied by higher than normal 
activity levels of liver enzymes in the serum. A computed tomography scan will show a liver 
that is less dense than normal, whereas an ultrasound test will produce a bright image in a 
ripple pattern. However, an absolute diagnosis requires a biopsy. 

(Choices 1 & 2) Up to 35% of chronic drinkers develop an inflammation of the liver know as 
alcoholic hepatis. This is caused by the oxidative metabolism of alcohol by the liver that 
produces free radicals as a side product and also by reaction to acetaldehyde, a toxic 
intermediate. Alcohol also permits the passage of bacterial endotoxins from the intestine to 
the small intestine via the portal vein. These toxins further damage liver cells causing the 
release of inflammation-promoting cytokines, which set up a vicious cycle by stimulating the 
release of additional cytokines. These inflammatory processes deplete oxygen levels, 
inducing cell death, and cause fibrous scarring, a condition known as cirrhosis. Early hepatis 
may be reversed by abstinence, but cirrhosis is not reversible and in 10%-20% of drinkers, 
these inflammatory processes lead to cirrhosis severe enough to eventually cause death if 
the patient is not given a liver transplant. 

(Choice 3) The fibrous scar tissue of cirrhosis also blocks the flow of blood through the 
branches of the portal vein that traverses the liver. This causes portal hypertension; when 
the portal pressure becomes high enough, blood is forced into other veins en route to the 
heart. These include veins in the esophagus, causing esophageal varices, and veins in the 
skin over the abdomen, leading to the appearance of spider veins. Esophageal varices are 
prone to bleeding, which may result in an anemia and may contribute to eventual death. Liver 
disease is almost always associated with leakage of enzymes from affected liver cells, 
causing an increase of these enzymes in the serum. Two of the liver enzymes are serum 
alanine aminotransferase (ALT) and serum aspartate aminotransferase (AST); however, in 
cases involving alcohol abuse, the increase in AST activity is always greater than that of 
ALT; thus (Choice 4) is incorrect. 

(Choice 5) Chronic excess alcohol consumption of course damages additional organs, the 
brain being one of the more critical. Wernicke disease affects nerves in both the central and 
peripheral nervous systems. The underlying cause is believed to be malnutrition, primarily a 
lack of thiamine. Heavy drinking is often associated with a poor diet, but individuals who 
maintain a balanced diet may still suffer from a lack of thiamine because alcohol inhibits 
thiamine absorption from the gut. Symptoms associated with Wernicke disease include 
confusion, nystagmus, ophthalmoplegia, anisocoria, ataxia, poor pupillary reflexes, and in 
extreme cases, coma and death. 

(Choice 6) often follows Wernicke disease, and although it too may involve thiamine 
deficiency, Korsakoff psychosis is associated with a direct toxic effect of alcohol on the brain 
that causes general cerebral deterioration, particularly affecting the medial thalamus and the 






mammary bodies of the hippocampus. The six major symptoms of Korsakoff’s psychosis 
are blackouts after a bout of drinking, known as anterograde amnesia; loss of memory of 
people and/or effects occurring prior to a bout of drinking, known as retrograde amnesia (this 
symptom often follows delirium tremens); invention of memories to fill in gaps associated with 
blackouts or other losses of memory, known as confabulation (confabulation may not be 
purposeful, and the false accounts are often believed by the teller); evasion of meaningful 
conversation; loss of insight; and increased apathy. These changes involve loss of neural 
function and bleeding within the mammary bodies. Although some improvement may occur 
during abstinence and treatment with thiamine supplementation, the condition cannot be 
reversed. It has been hypothesized that individuals genetically susceptible to symptoms 
associated with thiamine deficiency are more susceptible to acquiring the Wernicke and 
Korsakoff conditions. 


35. Question 1 points 

A 30-year-old man with a history of chronic diarrhea describes his stools as greasy and foul 
smelling. He recently developed pruritic vesicular lesions involving the elbows. A quantitative stool 
test for fat shows an increased amount of fat. An oral D-xylose absorption test reveals decreased 
reabsorption of xylose into the blood. Which of the following tests would be most useful in 
identifying the cause of the diarrhea? 


1. Antigliadin antibodies 

2. Antinuclear antibodies 

3. Fecal smear for leukocytes 

4. Stool for ova and parasites 

5. Stool osmotic gap 


INCORRECT X 

The correct answer is 1. 

The patient has celiac disease, an autoimmune disease with antibodies directed against the 
gliadin fraction in gluten, which is present in wheat products. The antibodies cause an 
inflammatory reaction in the villi, resulting in villous atrophy (flat mucosa) leading to 
malabsorption of fat (greasy stools), carbohydrates, and protein. In addition, this patient has 
an increased quantitative stool test result for fat and abnormal results in the D-xylose 
reabsorption test, which is an excellent screening test for documenting small bowel disease 
as a cause of malabsorption. The vesicular lesion on the patient’s elbow is dermatitis 




herpetiformis, which is an autoimmune skin disease that has an almost 100% correlation with 
underlying celiac disease. Other antibodies present in celiac disease include anti-endomysial 
and anti-reticulin antibodies. The treatment of choice is to eliminate gluten from the diet. 
(Choice 2) directed against nuclear proteins are not present in celiac disease. 

(Choice 3) is used for evaluating diarrhea that may be caused by invasive microbial 
pathogens (e.g., Campylobacter jejuni, Shigella sonnei). The presence of leukocytes 
presumes an invasive enterocolitis and would not be expected in celiac disease. 

(Choice 4) Testing the stool for ova and parasites is always recommended in the workup of 
a patient with chronic diarrhea. Giardiasis is the most common cause of chronic diarrhea 
associated with malabsorption; however, the presence of dermatitis herpetiformis excludes a 
parasitic cause of the chronic diarrhea. 

(Choice 5) A stool sample to calculate the osmotic gap is used for high-volume diarrheal 
states when a secretory or osmotic type of diarrhea is suspected rather than malabsorption. 
The stool osmotic gap is obtained by measuring potassium and sodium in the diarrheal fluid, 
adding them together, and multiplying the number by 2 (i.e., 2 X {[potassium] + [sodium]}). 
This value is then subtracted from 300 mOsm/kg, which represents the osmolality of plasma. 
Secretory diarrheas are characterized by isotonic diarrheal fluid; therefore, the osmotic gap 
in stool will be less than 50 mOsm/kg. Causes of a secretory type of diarrhea include certain 
types of laxatives and enterotoxigenic bacteria such as enterotoxigenic Escherichia coli and 
Vibrio cholerae. Osmotic diarrhea is characterized by a hypotonic stool due to the presence 
of osmotically active solutes drawing more water than electrolytes out of the enterocytes. 

The classic example of an osmotic diarrhea is lactase deficiency, leading to an increase in 
lactose, which is osmotically active. The osmotic gap in osmotic diarrheas exceeds 100 
mOsm/kg. 


36. Question 1 points 

A 65-year old man presents to a primary care physician for his pre-Medicare physical examination. 
He is 5 feet, 10 inches tall and weighs 225 pounds, making his body mass index (BMI) 32.3; 
consequently, he is classified as being overweight but not obese. His physical appearance shows 
that he has a belly. In addition, the following laboratory data were obtained: fasting blood glucose 
value 115 mg/dL; blood pressure 180/95 mm Hg; total cholesterol 275 mg/dl_, low-density 
lipoprotein 155 mg/dL, and high-density lipoprotein 25 mg/dL. The examining physician concluded 
that this man has metabolic syndrome and is likely to be pre-diabetic. Consequently, he subjected 
his patient to an oral glucose tolerance test by measuring the patient’s fasting serum level, giving 
him a standard glucose drink, and then determining his blood glucose 2 hours later; the 2-hour 
value was 175 mg/dL, resulting in a diagnosis of prediabetes. He informed his patient that clinical 
studies have shown that the best way to reverse such a pre-diabetic state is by choosing to 
undergo a lifestyle change that includes eating a carefully controlled diet and exercising regularly; 
however, since many patients quickly fall away from such a regime, he also prescribed a drug that 
had been shown help prevent progression to full-blown diabetes when given to pre-diabetic 
patients. Moreover, this drug will not cause hypoglycemia, promote weight gain, or increase the risk 
of heart failure. Which one of the following drugs did the physician choose? 




1. Sitagliptin phosphate 

2. Glyburide 

3. Metformin ✓ 

4. Rosiglitazone 

5. Nateglinide 


INCORRECT X 

The correct answer is 3. 

As indicated in the case study, this man shows the classic symptoms of metabolic 
syndrome (once called syndrome X). This includes a fasting glucose value higher than 
normal but below that required to diagnose diabetes (i.e., between 100 and 126 mg/dL), 
overweight, hypertension, and an abnormal serum lipid panel. Individuals having this 
syndrome feel well but are at high risk of developing full-blown diabetes. Even before 
becoming diabetic, they may be undergoing pathological changes that will lead to the typical 
complication of diabetes including retinopathy, nephropathy, and cardiovascular disease. 
Indeed, prediabetes likely should be considered a less severe form of diabetes rather than a 
unique condition in itself. The biguanide metformin is one of several oral diabetic drugs 
demonstrated to have the potential of reversing this pre-diabetic state; moreover, metformin 
operates primarily by inhibiting liver gluconeogenesis, as well as having a secondary ability 
to promote the uptake of glucose into muscle cells. In addition, it does not cause 
hypoglycemia or weight gain or increase the risk of heart failure, although it very rarely 
causes lactic acidosis. The potential ability of metformin to reverse the pre-diabetic state was 
evaluated in the Diabetic Prevention Program (DPP), a major clinical research program 
involving 27 research centers; in this study, overweight pre-diabetics persons were divided 
into three groups: the first group, called the lifestyle intervention group, received counseling, 
plus intensive training and supervised dietary and physical conditioning training as well as 
motivational therapy. The second and third group also received lifestyle counseling but not 
intensive training, supervision, or motivational therapy. The second group also received 850 
mg metformin twice a day and the third group a placebo. In comparison to the placebo group, 
diabetes development was reduced 58% in the lifestyle intervention group and 31% in the 
metformin group. 

(Choice 1) is the first drug in a relatively new drug class called dipeptidyl peptidase IV (DPP- 
4) inhibitors. DPP-4 normally breaks down the proteins that stimulate insulin production after 
a meal. Thus, a DPP-4 inhibitor permits insulin production to continue for a longer time, 
limiting the post meal increase in glucose. Sitagliptin phosphate does not cause 
hypoglycemia or weight gain; however, it has not yet been evaluated for its ability to reverse 
prediabetes, and sitagliptin may inflame the pancreas, conceivably worsening diabetes. 
(Choice 2) is one of the many sulfonylureas that act by stimulating the pancreatic (3 cells to 
excrete more insulin. This class of drugs is liable to cause weight gain as well as bouts of 
hypoglycemia. 




(Choice 4) and the other thiazolidinedione, pioglitazone, work by promoting the uptake of 
insulin by muscle cells, thereby decreasing insulin resistance in type 2 diabetics. It was 
reported at the 2008 meeting of the American Diabetic Association that pioglitazone lowered 
the progression from preto full diabetes by 81%; however, these thiazolidinediones promote 
water retention with concomitant weight gain and are suspected of inducing heart failure and 
therefore represent a false choice. 

(Choice 5) and repaglinide are the two medications presently approved for treatment of type 
2 diabetes. These drugs, like the sulfonylureas, stimulate the (3 cells to produce insulin; in 
contrast to the sulfonylureas, however, they only do so when blood glucose concentrations 
are elevated. Thus, they are taken usually three times a day, before a meal; as a result, they 
reduce the postprandial glucose high. Like sitagliptin phosphate, neither nateglinide nor 
repaglinide have yet been evaluated with respect to their potential for reversing prediabetes. 


37. Question 1 points 

A bereaved family just returned from a funeral for their 20-year-old son. In her grief, the mother 
began to reminisce to her mother. “He was such a healthy baby until he reached his third birthday. 
Then he stopped running and started walking like a duck. Next, he couldn’t stand without climbing 
up on somebody or something, and by his 11th birthday, we had to get him a motorized wheelchair. 
Even then he was doing well, finished high school, enrolled in City College, was a member of the 
chess team, and took an interest in politics. Then he caught what seemed just to be a cold and 
died.” The grandmother replied, “Yes, it is so tragic. I can’t understand what happened. Nobody 
else in the family ever had anything like his disease. Not his sisters, your sisters, or my sisters.” 
Which of the following diseases did this young man have? 


1. Becker muscular dystrophy 

2. Myotonic muscular dystrophy 

3. Facioscapulohumeral muscular dystrophy 

4. Limb girdle muscular dystrophy 

5. Welander distal myopathy 

6. Duchenne muscular dystrophy 


INCORRECT X 

The correct answer is 6. 

At least nine major variant types of muscular dystrophy exist, with several subtypes. The 
common feature is a progressive form of muscular weakness. The most common of these is 
Duchenne, the disease that killed the boy described in the vignette. It has an estimated 
incidence of 1 case per every 3,500 male live births and is due to a major deletion on the 


short arm of the X-chromosome (Xp21). It is inherited as a sex-linked recessive trait and 
results in the nonproduction of dystrophin, a very large protein (427 kDa) that is part of the 
contractile apparatus of muscle cells. In addition to making the muscles weak, the absence 
of a dystrophin molecule makes the muscle cell leaky, which leads to a marked increase in 
serum creatine kinase activity and contributes to the ultimate death of muscle cells. Affected 
boys seem normal until the age of 2-5 years, when they develop a peculiar duck-like 
waddling gait caused by weakness of the pelvic girdle muscles. The weakness then 
progresses throughout the legs, forcing them into a wheelchair by their teens, and then to the 
upper body. During the early period, their lower legs often increase in diameter, not from 
muscle, but from fat and connective tissue causing pseudo-hypertrophy. By their late teens, 
the respiratory muscles and/or heart also weaken, and these patients succumb to a 
respiratory disease or cardiac failure not long afterward. There is no treatment. The literature 
often reports mild retardation (an average IQ of 85), but there are many exceptions to this, as 
in the chess-playing boy described here. 

(Choice 1) also results from a mutation in the dystrophin gene, but the mutation permits 
production of a defective dystrophin protein that has limited function. The result is a muscular 
dystrophy similar to Duchenne but not as severe. First symptoms usually occur during the 
late teens or early 20s, and affected persons are able to walk into their 30s. However, they 
too will eventually die from either respiratory or cardiac failure. Duchenne muscular 
dystrophy is also inherited as an X-linked recessive disorder. 

(Choice 2) The incidence is about 1 case per 30,000 live male births. Type 1 myotonic 
muscular dystrophy is the most common adult dystrophy and takes its name from the 
tendency for affected persons have progressive muscle wasting and weakness, coupled 
with myotonia, the inability to relax muscles after use; thus, for example, a person may not 
be able to put down a fork after using it. At least two distinct forms of myotonic muscular 
dystrophy exist, type 1 (DM1) and type 2 (DM2); DM1 accounts for as many as 98% of the 
cases and affects about 1 in 8,000 individuals. Both forms are inherited as autosomal 
dominant diseases and DM1 in particular shows anticipation the disease appears earlier and 
become more severe with each successive generation. DM1 is caused by a trinucleotide 
repeat on chromosome 19 that result in a defective myotonin protein kinase, whereas DM2 is 
caused by a tetra-nucleotide repeat on chromosome 3. Additional poorly characterized 
variants of myotonic muscular dystrophy might also exist. Diagnosis of the myotonic 
muscular dystrophies is difficult to make because not only do symptoms vary from family to 
family, the range and severity of symptoms can vary greatly among patients even in the 
same family. At one extreme, a patient may only develop mild muscle weakness and/or 
cataracts late in life, whereas at the other extreme, a child might be born with the congenital 
form of the disease and have early-onset life-threatening disease. All patients with the 
condition are at risk of severe reactions to anesthesia and should be monitored carefully if 
subjected to anesthesia. 

(Choice 3) is an autosomal dominant condition that usually starts in the teens or early 20s. It 
first affects the face and shoulder girdle and then the pelvic girdle, legs, and abdomen. 
Clinically, its effects vary from very mild to severe; about 50% of patients can walk until they 
die at a normal age. 

(Choice 4) can be inherited in an autosomal dominant or more commonly in an autosomal 
recessive fashion. First symptoms usually start in the teens or early adulthood as weakness 
in the hips. It then slowly progresses, first to the shoulders, then to the arms and legs. Some 






20 years after the first onset of symptoms, walking becomes very difficult. 

(Choice 5) is a rare autosomal dominant condition most commonly is found among persons 
of Swedish or Finnish ancestry, having a prevalence of about 1 in 4,000 in mid-Sweden. It is 
one of a group of heterogeneous myopathies classified as distal myopathies, characterized 
clinically by muscular weakness and atrophy beginning in the hands and feet. The disease 
generally then progresses in severity and to muscles elsewhere in the body. As many as 
two dozen suspected distinct types of distal myopathies have been described (at least five of 
which have been identified as mutations in a specific gene), making diagnosis difficult. Most 
have adult onset, a major exception being Laing myopathy, which can be identified in infancy. 
In addition, individuals in the same family can be affected to markedly different degrees. One 
member may live a long, essentially normal life with little more than what appears to be 
arthritis of the hands, whereas another may be bedridden by mid-adulthood. 


38. Question 1 points 

A 74-year-old widow who lived alone fell at home and hurt her left wrist. She could immediately see 
that it was broken because of the way it dangled. Consequently, she called 911. The paramedics 
set her arm in splints and took her to the nearest emergency clinic. While the attending physician 
treated the fracture, he also took a history. Among the questions he asked was: “Why do you think 
you fell?” She responded that she became dizzy; she felt the room was spinning while she was 
standing in one place; consequently, she lost her balance. The physician probed further and 
subsequently discovered that she had been having similar spells of dizziness intermittently at 
various times both during the day or night for the past several months. The dizziness could occur 
while she was lying down, sitting, or standing without her changing her position. He further 
determined that her left ear often felt full and that she sometimes had a faint but annoying ringing in 
her ears. She denied ever vomiting during these dizzy spells but did say she often felt nauseated. 
Her dizziness was most likely caused by which of the following? 


1. Shy-Drager syndrome 

2. Meniere disease V 

3. Essential hypertension 

4. Orthostatic hypotension 

5. Benign positional vertigo 


INCORRECT X 

The correct answer is 2. 

Meniere disease is one of a host of conditions that can cause dizziness. Characteristics in 
the case history described that point to Meniere disease are the description of the room 


spinning while the patient stood still, tinnitus, and a sensation of fullness in one or both ears. 

It is also often also accompanied by nausea and vomiting. Since there is no specific test, it is 
diagnosed by clinical symptoms and response to treatment. Specifically, acute attacks 
usually respond to the antivertigo drug meclizine and/or benzodiazepines but not to treatment 
used to prevent other causes of dizziness. 

(Choice 1) Shy-Drager syndrome; aka, neurologic orthostatic hypotension or multiple 
system atrophy) is a rare disease that, among a host of other symptoms, causes a feeling of 
dizziness. The disease starts gradually, often manifesting with frequent falls caused by 
dizziness due to a drop in blood pressure upon standing up. It progresses into a 
parkinsonian-like condition that includes slowness of movement, muscle rigidity, bladder 
dysfunction, and poor balance; ultimately, it causes death. Commonly, it affects males more 
than females, and symptoms start at about the age of 50-60 years. The patient described is 
female, and by the age of 74 years, had she had Shy-Drager syndrome she would likely had 
symptoms more severe than dizziness. 

(Choice 3) should not of itself cause dizziness, nor does the case history mention 
hypertension. 

(Choice 4) is a common cause of dizziness provoked by rapidly standing, particularly from a 
supine position; typically, the patient feels faint. Moreover, the dizziness described by the 
lady in this question occurred while she was lying down, sitting, or standing, and was not 
provoked by a change in position. 

(Choice 5) is the most common cause of peripheral vertigo. Normally, tiny mineral particles 
help maintain balance by rotating within the semicircular canals. In benign positional vertigo, 
some of these get stuck in one place. Most patients get relief by body repositioning 
maneuvers that can be performed in the physician’s office or even at home. Benign positional 
vertigo is not associated with tinnitus (although a patient may have had tinnitus before being 
troubled by vertigo), or with a feeling of fullness in an ear; nor is it likely a patient will describe 
the room as spinning. A patient with benign positional vertigo is more likely to describe the 
dizziness as an unsteady feeling and/or faintness rather than a spinning room. 


39. Question 1 points 

A 40-year-old man came to see his primary care doctor as he had been feeling very fatigued of late. 
He stated that he was tired all the time, had difficulty walking the distance that he could only a 
month or so ago, and he also noticed some swelling of his feet, usually at the end of the day. 
Physical examination revealed a medium-built well-nourished man with conjunctival pallor. The 
sclera appeared mildly icteric. His temperature was 98.6°F (37.0°C), pulse 88/min regular, 
respirations 18/min, and his blood pressure was 120/86 mm Hg. Oxygen saturation using room air 
was 90%. His lungs were clear to auscultation, bilaterally; he had a soft murmur in the precordial 
area without a thrill, and no gallops or rubs were noted. The abdomen was soft to palpation, and he 
had mild tenderness without guarding or rigidity in the right upper quadrant. No organomegaly was 
noted, and bowel sounds were present in all four quadrants. A complete blood count revealed a low 
hematocrit and a corrected reticulocyte count of greater than 3%. Which one of the following 
changes in serum levels also would most likely occur? 


1. Conjugated bilirubin, haptoglobin, and lactic acid dehydrogenase levels would all be 
elevated 

2. Conjugated bilirubin would be elevated; haptoglobin and lactic acid dehydrogenase 
levels would be decreased 

3. Unconjugated bilirubin and haptoglobin levels would decrease; the lactic acid 
dehydrogenase level would increase 

4. Unconjugated bilirubin, haptoglobin, and lactic acid dehydrogenase levels would all 
increase >/ 

5. Only the haptoglobin level would increase 


INCORRECT X 

The correct answer is 4. 

This patient has a hemolytic anemia. Hemolytic anemia occurs when more red blood cells 
are destroyed than are being produced. It could result from immune or nonimmune disorders; 
the defect could be intrinsic or extrinsic. Causes include intrinsic red cell anomalies; 
premature destruction of otherwise normal cells, as occurs in mismatched transfusion; drug 
interaction; infection due to some strains of streptococci; and hypersplenism. Intrinsic 
abnormalities of red blood cells that may cause a hemolytic anemia include hereditary 
spherocytosis, in which there usually is an abnormality in spectrin, a protein that provides 
most of the scaffolding for the red cell membrane and enables it to deform; deficiencies in 
one of several of the enzymes involved in glucose metabolism; and abnormal hemoglobins, 
such as hemoglobin S, which causes sickle cell disease, an autosomal recessive disorder 
leading to sickling of red cells under conditions of local hypo-oxygenation. A peripheral blood 
smear will help distinguish between spherocytes and sickle cells. If sickle cells are found, 
hemoglobin electrophoresis is required to establish whether the sickling is due to sickle cell 
disease. In hemolytic anemia, the levels of unconjugated bilirubin, haptoglobin, and lactic 
dehydrogenase are increased. Bilirubin is a breakdown product of hemoglobin and is usually 
transported to the liver via the circulation. For this to happen, bilirubin has to be 
noncovalently associated with albumin. This complexed form is called unconjugated or 
indirect bilirubin. Once it reaches the liver, it is metabolized and albumin is no longer required. 
Most of this bilirubin freed from albumin is covalently bound to glucuronic acid is now called 
conjugated or direct bilirubin. However, in cases of hemolysis, a large quantity of 
unconjugated bilirubin remains circulating in the blood because the liver is unable to 
conjugate such a large quantity; hence, the level of circulating unconjugated bilirubin 
increases. Haptoglobin is a circulating protein and is an acute-phase reactant, meaning its 
concentration is increased in the serum in conditions in which stress, infection, or 
inflammation occurs. In cases of hemolysis, haptoglobins bind the hemoglobin that is 
released, thus minimizing its accumulation in the plasma. The hemoglobin-haptoglobin 
complex is then eliminated by the reticuloendothelial system. Lactic dehydrogenase is 
present in the cytoplasm of red cells and is released into the circulation when cells undergo 
hemolysis, resulting in its elevation. 


(Choices 1,2,3 & 5) All are incorrect. 


40. Question 1 points 

A 77-year-old man who prides himself as having been in prime physical health all his life despite 
seldom seeing a doctor, and who claims the only medication he ever takes is aspirin, suddenly is 
plagued by severe headaches, vertigo, and a loss of balance that makes standing and walking 
difficult. Consequently, he has a fall and bumps his head. His worried daughter takes him to the 
nearest emergency department, where he is examined. He is found to be a thin man who is 
relatively muscular for his age. Although still unable to stand or walk without losing his balance, 
there is no evidence of muscle weakness. He remains fully conscious, is fully cognizant of his 
surroundings, and can answer questions quickly and lucidly. His heart and lungs are normal, as is 
an electrocardiogram (ECG). A non fasting finger stick blood glucose determination reads 152 
mg/dL of glucose. His blood pressure remains between 165 and 185 mm Hg systolic over 75-85 
mm Hg diastolic. Which of the following is the most likely underlying cause of his problem? 


1. An epidural hemorrhage 

2. Meniere syndrome 

3. Isolated systolic hypertension 

4. A cerebral infarct 

5. A pulmonary thromboembolism 


INCORRECT X 

The correct answer is 3. 

Isolated systolic hypertension is a condition that often affects the elderly; in fact, it is so 
common that it was once believed that normal systolic pressure is equal to 100 plus a 
person’s age. However, trial studies have conclusively demonstrated that elevated systolic 
blood pressure is a more significant underlying cause of cardiac disease and stroke than is 
elevated diastolic pressure; 30% of women and 20% of men older than 65 years have this 
condition. The underlying reason is a loss of arterial elasticity that occurs with aging, and the 
condition becomes more common with advancing age. The man described most likely is 
suffering from a cerebellar stroke, which typically is associated with headache and ataxia. 
(Choice 1) is a consequence of trauma and could conceivably have resulted from his fall. 
However, his fall occurred after the symptoms first appeared; typically, an epidural 
hematoma is characterized by coma after a lucid interval, and there is no mention of loss of 
consciousness. 




(Choice 2) is a cause of vertigo induced by distention of the endolymphatic compartment of 
the inner ear. Although usually idiopathic, it can be caused by head trauma or syphilis. It is 
not age associated, and there is no evidence presented in the vignette that this patient might 
have Meniere syndrome. 

(Choice 4) would not have only primary symptoms relating to ataxia but would rather have 
loss of muscular strength or cognitive function. 

(Choice 5) is usually accompanied with signs of dyspnea, chest pain, hemoptysis, and/or 
syncope. Except for syncope, the man had none of these symptoms. Moreover, there is no 
indication of a predisposition for venous thrombosis, and the one drug this man uses is 
aspirin. 


41. Question 1 points 

A 25-year-old man presents to the emergency room with a history of swelling around the left eye, 
which has become very painful. He states that initially he had noted double vision and that it hurts 
to move his eye. He was in considerable distress due to pain. The vital signs were as follows: 
temperature 101°F (38.3°C), pulse 86/min regular, respirations 18/min, blood pressure 128/78 mm 
Hg and, oxygen saturation 98% on room air. There was considerable swelling around the left eye, 
which was boggy and tender to touch. Ocular movements were present in all directions but were 
painful. The pupils were equal and reacting appropriately to light and accommodation, and 
funduscopy was unremarkable. The most likely cause for this problem is: 


1. Cavernous sinus thrombosis 

2. Corneal ulcer 

3. Sinusitis >/ 

4 . Orbital abscess 

5. Ocular trauma 


INCORRECT X 

The correct answer is 3. 

This patient has orbital cellulitis, which is most commonly due to sinusitis. Patients will often 
give a history of prior upper respiratory infection. Orbital cellulitis can lead to life-threatening 
complications, including cavernous sinus thrombosis and orbital abscess. This is an 
emergency and should be treated with intravenous broad-spectrum antibiotics. Sinusitis, if 
present, should be treated, 

(Choice 1) Although cavernous sinus thrombosis is a potential complication of orbital 
cellulitis, this patient as not yet developed this condition. In addition to fever and diplopia, it 
usually presents with a history of nausea, vomiting, and headache. Alteration in mental 


status also may be present, and the pupil is dilated. 

(Choice 2) is associated with blepharospasm (the eyelid is squeezed shut, as if squinting), 
epiphora (increased tear secretion), and ocular pain. Diplopia and fever are not features. 
(Choice 4) results from ulitis (inflammation of the gums) and is a potential complication of 
orbital cellulitis. 

(Choice 5) The patient does not give a history of trauma, and if trauma were responsible for 
the symptoms, ocular hemorrhage would probably have been associated with it and noted on 
examination. 


42. Question 1 points 

An 82-year-old retired university professor of Scott-lrish ancestry has had gastroesophageal reflux 
disease (GERD) and hypertension for a number of decades. Lately, he has been having increasing 
difficulty walking; his gait has become more and more shuffling and hesitant. More recently, he has 
also complained of excessive fatigue; for example, he might doze off in his chair while eating 
breakfast. His wife thought this might be because he did not get a good night’s sleep in fact, she 
complained that she couldn’t sleep well either because of his loud snoring. His primary care 
physician is treating his GERD with omeprazole and is using six different antihypertension drugs 
(enalapril, labetalol, clonidine, doxazosin mesylate, amlodipine besylate, and hydrochlorothiazide) to 
control his systolic hypertension, but his systolic pressure remains, at best, in the 140-160 mm Hg 
range. Frustrated, the physician arranges for his patient to be tested for possible obstructive sleep 
apnea, which theoretically could cause fatigue and muscle weakness, and contribute to the 
hypertension. Testing shows that he had an inadequate amount of rapid eye movement (REM) 
sleep, and his oxygen saturation dropped below 90% on a regular basis. He was provided with a 
continuous positive airway pressure (CPAP) device, so that he could treat himself at home. 
However, his systolic blood pressure stubbornly remained above 140 mm Hg, and he continued to 
walk poorly and doze off during the day. 

Looking for some other source for his patient’s hypertension and fatigue, the physician ordered a 
battery of blood tests including a complete blood count (CBC). Which of the following patterns could 
best explain this patient’s symptoms? 


1. Normal hemoglobin levels and a mixture of normal-looking red cells mixed in with 
some abnormally large erythrocytes, some poikilocytes, a few hypersegmented neutrophils, 
and a reduced the reticulocyte count 

2. Normal red cells mixed in with a substantial fraction of schistocytes and normal 
leucocytes and a decrease reticulocyte count 

3. Primarily spherocytic red cells, normal white cells, and a decreased reticulocyte 
count 

4. Normal red cells mixed in with some sickled cells and a decreased reticulocyte count 

5. A reduced number of normal red cells with normal white cells and a decreased 


reticulocyte count 


INCORRECT X 

The correct answer is 1. 

The pattern described in answer A describes a macrocytic/normochromic anemia (i.e., an 
anemia that produces abnormally large red cells that have normal hemoglobin content); this 
is also known as a megaloblastic anemia. This condition occurs whenever DNA synthesis is 
inhibited in an otherwise viable developing cell. The inhibition of DNA synthesis delays 
nuclear maturation and induces an imbalanced rate of development in which cell division is 
inhibited, thus permitting the erythroblasts in the bone marrow to grow to an abnormal size 
and often an aberrant shape. (The generic term for such irregularly shaped cells is 
poikilocytes.) Although cell division is inhibited cytoplasmic functions continue permitting the 
maturing red cell to take up hemoglobin. As development continues, the unusually small 
nucleus of the blast cell is lost, and the malformed red cell is transport into the circulation, 
where it is readily recognized. Folate is required for DNA synthesis, and consequently, this 
type of abnormal development always occurs under conditions of absolute or functional 
folate deficiency. Functional deficiency of folate can occur when sufficient vitamin B 12 is not 
available because vitamin B 12 is a cofactor in the homocysteine methyltransferase reaction, 
which methylates N 5 -methyl tetrahydrofolate to form tetrahydrofolate, an active form of folate 
used in DNA synthesis. (N 5 -methyl tetrahydrofolate is the end-product of all metabolically 
active forms of folate and has no other catalytic function; thus if vitamin B 12 levels are 
insufficient to permit this methylation reaction, a functional deficiency of folate will occur, 
provided dietary intake of folate is not adequate enough to overcome its loss via conversion 
of the active forms to the N 5 -methyl tetrahydrofolate derivative.) 

As ingested, dietary vitamin B 12 passes through the stomach, where it attaches to a 
glycoprotein called the intrinsic factor, which is synthesized by the gastric parietal cells; this 
binding requires a low pH. The vitamin B 12 -intrinsic factor complex then binds to specific 
receptors in the mucosal cells of the ilium and subsequently is transported into the general 
circulation. Pernicious anemia results when this complex mechanism of vitamin B 12 
absorption goes awry. Such malfunction may result from an autoimmune reaction disabling 
the production or function of the intrinsic factor, an autoimmune reaction or surgery causing 
the loss of parietal cells, or a lower than normal gastric pH. Pernicious anemia has been 
reported to be most commonly found among persons of Celtic or Scandinavian ancestry but 
in the past few decades, people from other ethnic groups have been reported to have a 
similar prevalence. This may be because in modern developed societies the hematologic 
symptoms of pernicious anemia have become more difficult to observe because they are 
commonly made imperceptible by excess folate in the diet, which makes the conversion to 
N 5 -methyl tetrahydrofolate largely inconsequential. One might think this would make the 
reduced ability to absorb vitamin B 12 unimportant; however, this vitamin has one additional 
function in normal metabolism and that is as a cofactor in the methylmalonyl CoA mutase 
reaction that converts methylmalonyl CoA to succinyl CoA. This reaction permits the 
conversion of propionyl CoA to succinate; this propionyl CoA is produced by the final (3- 
oxidation step during the oxidation of odd-carbon fatty acids, as well as from valine, leucine, 
and methionine. Thus, a deficiency of vitamin B 12 inhibits the methylmalonyl CoA mutase 


reaction and leads to an accumulation of propionyl CoA, which can get incorporated into fatty 
acids during fatty acid synthesis; this will produce odd-carbon fatty acids that then get 
incorporated into neural myelin causing neuropathy, a relatively early symptom of which is 
loss of proprioceptor function. However, over the long-term it may cause more severe 
symptoms, including psychiatric symptoms resulting in senile dementia. In addition to 
showing some hematologic symptoms, namely signs of a macrocytic-normochromic anemia, 
the patient described shows signs of neuropathy, namely loss of proprioceptor function 
marked by a shuffling hesitant gait. In addition, he might be genetically prone to develop 
pernicious anemia because of his Celtic ancestry and because his gastroesophageal reflux 
disease (GERD) is being treated with a protein pump inhibitor. 

(Choices 2,3,4 & 5) are incorrect because the root part of the answer is irrelevant and 
because each condition described should result in increased not decreased reticulocyte 
formation. 


43. Question 1 points 

A 43-year-old Caucasian man of northern European descent had been feeling a rapidly escalating 
degree of fatigue for the past several weeks and consequently consulted his primary care 
physician. Upon taking a history, his physician confirmed that, in addition to feeling tired, the patient 
has noted a shortness of breath and heart palpitations. Laboratory analyses showed low serum 
hemoglobin and haptoglobin levels and raised serum reticulocyte, lactic acid dehydrogenase, and 
bilirubin concentrations. Deducing that her patient may have a hemolytic anemia, the physician 
referred him to a hematologist, who determined that there was no family history of anemia. The 
hematologist asked the patient to void a midday urine sample into a prelabeled Erlenmeyer flask, 
and to also provide her with a second urine sample obtained immediately after rising in the morning; 
she also and told him to bring both samples into her office the following day. The patient did as 
requested; the midday urine sample had a deep orange-yellow color typical of concentrated normal 
urine, but the early morning sample was a bright reddish color. The patient most likely suffered from 
which one of the following conditions? 


1. A drug-induced non autoimmune hemolytic anemia 

2. Paroxysmal nocturnal hemolytic anemia 

3. Paroxysmal cold hemoglobinuria 

4. Warm autoimmune hemolytic anemia 

5. Acute intermittent porphyria 


INCORRECT X 


The correct answer is 2. 

Paroxysmal nocturnal hemolytic anemia (PNHA) is a rare acquired form of hemolytic 
anemia. It is characterized by hemolysis that typically occurs during the night, resulting in 
excretion of early morning urine colored dark red by hemoglobin released from lysed red 
cells. The first symptoms may present at any age from infancy into the 80s, but it most 
frequently affects adults between 17 and 75 years old, with a mean age of presentation of 42 
years. It is caused by the loss of membrane surface proteins due to an apparent mutation 
that inhibits the ability to synthesize glycosyl-phosphatidylinositol, which normally serves to 
anchor these surface proteins to the cell. The responsible gene is called phosphatidylinositol 
glycan class A (PIGA) and the mutations may be of several types, including frame shifts, 
point mutations, and deletions. This defect is believed to be a stem cell mutation because all 
cells in the hemopoietic line are affected; it is hypothesized that the mutations are caused by 
autoimmune reactions. The critical membrane proteins involved in PNHA symptomlogy 
normally react with members of the complement family, including those that normally act to 
impede the amplification of complement action; consequently, such a mutation acts to inhibit 
the normal dampening control in the complement cascade, permitting the action of 
complement to run amok and resulting in cell lysis. Because all hematopoietic cell lines are 
affected, symptoms extend beyond lysis of erythrocytes, causing a generalized 
pancytopenia that reduces the number of white cells and platelets, as well as causing an 
anemia. Thus, in addition to the anemia, thrombi often occur, particularly in large veins; these 
may cause severe disease, resulting in death. In theory, the ideal treatment is stem cell 
replacement; unfortunately, this process is not yet practical. Presently, treatment includes 
anticoagulants plus an anticomplement antibody called eculizumab. Use of this drug 
dramatically improves all almost all symptoms of PNHA. 

Whenever anemia is found in a patient, it is important to ascertain whether the anemia is due 
to a disorder in the bone marrow, to hemolysis, or to loss of blood. A corrected reticulocyte 
count will help make the differentiation. The formula for a corrected reticulocyte count is the 
patient’s reticulocyte count, multiplied by the patient’s hematocrit, divided by the expected 
hematocrit. If the corrected reticulocyte count is below 2%, the most likely cause of anemia 
is decreased red blood cell production due to a hypoproliferative bone marrow. On the other 
hand, if the corrected reticulocyte count is greater than 3%, the most likely cause is 
hemolysis. In this patient, who has hemolytic anemia, it is important to establish if the etiology 
is due to an immune or a nonimmune disorder. This can be achieved by the Coombs test. 
(Choice 1) The classic example of a drug-induced non autoimmune hemolytic anemia is a 
glucose-6 phosphate dehydrogenase deficiency. It can be ruled out in the case described 
because the onset of glucose-6 phosphate dehydrogenase deficiency is sudden and 
dependent upon ingestion of a drug, rather than a gradually worsening fatigue; moreover, the 
patient most likely would be African American or of Mediterranean descent. 

(Choice 3) is a rare condition characterized by an abrupt onset of a severe hemolytic 
anemia and hemoglobinuria when a patient is exposed to cold temperatures. (The hemolysis 
usually occurs in a limb or digit exposed to a cold temperature.) Induction of this 
phenomenon involves the attachment of a foreign antibody to the red cell, which is promoted 
by the reduced temperature; hemolysis is then induced by activation of the complement 
system when cells are warmed. The phenomenon commonly manifests itself as a sudden 






transitory development induced by infections, usually a postviral episode. Before the advent 
of antibiotics, it was more commonly observed, often among patients suffering the tertiary 
stage of syphilis. 

(Choice 4) is the most common type of hemolytic anemia and is usually found in mature 
adults. In this condition, the patient’s body makes auto-anti bodies against their own red blood 
cells, and hemolysis occurs under warm conditions (i.e., normal body temperature). 
Diagnosis is generally made by the direct Coombs test. 

(Choice 5) is a rare dominant disease caused by a deficiency of porphobilinogen deaminase. 
Insufficient activity of this enzyme leads to the accumulation of porphobilinogen in the 
cytoplasms of cells in various organs in the body. This accumulation may be influenced by 
various hormones and external stimuli, causing intermittent attacks. These attacks may 
include various digestive, muscular, or even mental aberrations and the voiding of port wine- 
colored urine, which turns to purple when exposed to ultraviolet light for a period of time. 


44. Question 1 points 

A 35-year-old Caucasian man with neurofibromatosis presents to his physician with a complaint of 
nausea and vomiting, vertigo, nystagmus, tinnitus, and nerve deafness in the right ear. These 
symptoms had gradually worsened over the past month or so. Upon examination, the physician 
noted hemianesthesia on the right side of the face. Which of the following is the most likely 
diagnosis? 


1. Mastoiditis 

2. Cerebellar tumor 

3. Vertebrobasilar arterial insufficiency 

4. Glioblastoma multiforme 

5. Acoustic Schwannoma >/ 


INCORRECT X 

The correct answer is 5. 

An acoustic schwannoma is associated with tinnitus, nausea and vomiting, vertigo, 
nystagmus, and eighth-nerve deafness. It arises in the cerebellopontine angle and may 
involve the trigeminal nerve, producing ipsilateral sensory changes in the face. It is most 
commonly a schwannoma a benign which is a benign, encapsulated tumor arising from 
Schwann cells. Patients with neurofibromatosis have an increased incidence of acoustic 
schwannomas. 

(Choice 1) Untreated mastoiditis that erodes through bone to produce acoustic nerve 
damage is rare. 




(Choices 2 & 3) (Choice 2) and vertebrobasilar artery insufficiency (Choice 3) produce 
ataxia. Furthermore, they are not usually associated with eighth-nerve damage. 

(Choice 4) is the most common primary malignancy of the brain in adults and usually 
involves the frontal lobes. It would not be expected to produce eighth-nerve damage. 


45. Question 1 points 

A 45-year-old woman sees her primary care doctor for fatigue. She states that she used to be able 
to walk a mile “with ease,” but of late has been having problems doing so. She feels tired and 
winded, and has noticed some problems eating as her tongue “feels raw.” This patient most likely 
has which one of the following conditions? 


1. Microcytic anemia 

2. Macrocytic anemia 

3. Thalassemia 

4. Sideroblastic anemia 

5. Anemia of chronic disease 


INCORRECT X 

The correct answer is 2. 

This patient has pernicious anemia due to vitamin B 12 deficiency. In younger patients such 
as this, the most common cause of pernicious anemia is an autoimmune reaction in which 
antibodies destroy the stomach’s parietal cells, which normally manufacture intrinsic factor. 
Intrinsic factor is required for B12 absorption in the terminal ileum. As in this case, patients 
may have glossitis, as well as neurological symptoms such as tingling in the fingers, loss of 
vibration and position sense, and in extreme cases, dementia. Other causes of B12 
deficiency anemia in include inadequate dietary intake of vitamin B 12 , alcoholism, partial 
gastrectomy, malabsorption syndromes, Crohn’s disease, and infestation with the fish tape 
worm (Diphyllobothrium latum), which is present in fresh water fish in the northern 
hemisphere. Antiparietal cell antibodies will be present in autoimmune cases of pernicious 
anemia. In the presence of macrocytic anemia, it is important to check both B12 and folate 
levels, as folic acid deficiency could lead to macrocytic anemia as well. Red blood cell folate 
level is more accurate than serum folate level. Whether a macrocytic anemia is due to lack of 
B12 or folic acid can be resolved by ascertaining the levels of serum methylmalonic acid and 
homocysteine. Both levels are increased in B12 deficiency, but in the case of folic acid 
deficiency, only homocysteine levels are elevated. Methylmalonic acid is an intermediate in 
fatty acid metabolism that is derived from malonate, while homocysteine, an amino acid not 
found in diet, is derived from methionine. It is involved in the metabolism of cysteine. A 








Schilling test will distinguish between nutritional or absorptive cause of macrocytic anemia. 
Conversely, it is also possible that high intake of folate will prevent signs of a macrocytic 
anemia even if a patient has a vitamin B 12 deficiency. Thus, one should pay careful attention 
to neurological symptoms that may be the only sign of a vitamin B 12 deficiency and could 
easily be overlooked; unfortunately, these may in the long run cause severe permanent 
neurological damage, including psychological aberrations. 

(Choice 1) is seen in iron deficiency that can result from inadequate intake or absorption of 
iron, or excessive blood loss, with the most common cause being inadequate intake. Clinical 
features include cheilosis, in which the lips are cracked at the angles of the mouth, 
dysphagia, brittle nails, and in extreme cases, koilonychia, also known as spooning of the 
nails. 

(Choice 3) is the most common genetic disorder worldwide. It is due to abnormal 
hemoglobin. Mature hemoglobin is a tetramer of two and two a chains. If there is an 
aberration in this combination, interaction with oxygen will result in precipitation of hemoglobin 
and destruction of the abnormal red blood cell by the spleen, with resultant hemolytic anemia. 
Patients will have microcytic hypochromic red blood cells in the peripheral smear. 
Thalassemia can affect either the a or (5 chains, hence hemoglobin electrophoresis is 
required to uncover the malady. 

(Choice 4) is usually an acquired form of anemia in which there is an inability to incorporate 
heme into protoporphyrin to create hemoglobin. Causes include myelodysplasia, which may 
progress to acute leukemia; chronic alcoholism; and lead poisoning. Patients have mild to 
moderate anemia, and usually present with fatigue. Mean corpuscular volume (MCV) is 
usually normal. In some instances, it may be decreased, causing confusion with iron 
deficiency anemia. The distinction can be made by the peripheral blood smear, which shows 
two kinds of red blood cell populations, some normal and others hypochromic. In addition, 
iron stores are increased due to the primary anomaly, which is that heme cannot be 
adequately utilized. The diagnosis is best made by examining the bone marrow, which will 
reveal ineffective erythropoiesis as noted by the presence of marked erythroid hyperplasia, 
increased iron stores, and the presence of ringed sideroblasts. The serum iron and 
transferrin saturation will be markedly elevated, and iron may be deposited in mitochondria 
as well. Sideroblastic anemia due to lead poisoning will reveal basophil stippling in the red 
blood cells and elevated lead levels in the serum. 

(Choice 5), as the name suggests, occurs in chronic systemic disorders such as infection, 
inflammation, hepatic disease, and malignancy. Chronic renal failure is also a notable cause. 
In cases other than chronic renal failure, anemia results from reduced red cell survival rate, 
and the inability of the bone marrow to step up production as iron is stashed away in the 
reticuloendothelial system where it has been delivered by haptoglobin. In chronic renal 
failure, on the other hand, anemia is due to inadequate production of erythropoietin, which is 
essential to prime the bone marrow to produce red blood cells. The diagnosis of anemia of 
chronic disease is confirmed by the presence of low serum iron, low total iron binding 
capacity, and normal or elevated levels of ferritin, and in the case of anemia of chronic 
disease, low erythropoietin levels. 


46. Question 


1 points 


A recently retired 67-year-old man makes an appointment to see his physician because his hands 
are beginning to tremble in an uncontrollable manner. During the examination, the physician notes 
that he does have an obvious resting tremor of both hands. However, he had no difficulty in picking 
up a quarter, and during that process the tremor ceased. When asked to hold his hand out, palm 
up, his fingers underwent an involuntary movement as if he was rolling a pill across his palm. He 
states that although the tremor is annoying and sometimes embarrassing, it makes little difference 
in his lifestyle; it has not even interfered with playing golf. At this time, which of the following is the 
best first step in treatment? 


1. Amantadine 

2. An anticholinergic drug 

3. Levodopa 

4. Carbidopa 

5. A dopamine agonist 

6. Selegiline 

7. Watchful waiting 


INCORRECT X 

The correct answer is 7. 

This man is suffering from early parkinsonism. Most commonly, this is due to Parkinson 
disease, a condition of unknown etiology that generally starts between the ages of 45 and 65. 
Parkinson disease is a heterogenous and progressive condition that has tremor, rigidity, 
bradykinesia, and postural instability as its cardinal features. Cases tend to fall into one of 
two major subtypes. In one group, tremor is the major symptom; in the other, postural 
instability and gait difficulty predominate. Even though symptoms do overlap, most patients 
clearly fall to the greatest extent into one or the other subcategory. Patients with 
predominantly tremor-type symptoms tend to have a slower progression of symptoms, have 
less trouble with bradykinesis, and less often develop severe mental symptoms. Other 
symptoms in more advanced cases include infrequent blinking, a blank stare, a shuffling gait 
with rapid acceleration and difficulty stopping once started, increased salivation, and severe 
depression or even dementia. No one patient needs to develop all symptoms to meet the 
diagnostic criteria, and patients who have early symptoms of gait disturbances progress 
more rapidly and are more apt to develop the more severe symptoms. The underlying 
pathology is dopamine depletion due to degeneration of the nigrostriatal system. This leads to 
an imbalance between acetylcholine and dopamine neurotransmission. The cornerstone of 
treatment is dopamine replacement coupled to blockage of the acetylcholine system. 
Unfortunately, resistance to ceratin key drugs develops with use. Therefore, an attempt is 


made to refrain from using them as long as possible. Thus, watchful waiting is typically used 
until symptoms interfere with the patient’s normal lifestyle. This patient clearly states that his 
tremor does not impinge upon his normal lifestyle. 

(Choice 1) Once it is decided that some medical intervention will be of value, amantadine is 
typically the first drug to be used while symptoms are still minimal. This antiviral drug helps to 
improve muscle control and reduce stiffness; its mode of action is not understood, but it is 
hypothesized to help release dopamine from nerve endings. Its effect is always less than 
profound, and the benefit further decreases with use. 

(Choice 2) Anticholinergics also are used to treat early parkinsonism, sometimes in 
conjunction with amantadine. Again, drug resistance develops, and these drugs are generally 
administered in increasing doses until the adverse effects outweigh the benefits. Thus, 
eventually, it becomes necessary to use the most effective treatment namely, dopamine 
replacement. However, dopamine cannot cross the blood brain barrier. 

(Choices 3 & 4) Consequently, it is usually administered as Sinemet, a preparation that 
contains levodopa and carbidopa in fixed proportions. The levodopa is converted to 
dopamine in the body, and the carbidopa inhibits the enzyme that converts levodopa to 
dopamine. But carbidopa cannot cross the blood-brain barrier; thus, by administering the two 
together there is both more levodopa available, and some of the adverse peripheral effects of 
dopamine such as nausea, vomiting, hypotension, and cardiac irregularities are avoided. 
Long-term administration of levodopa induces untoward central nervous system effects 
including dyskinesias, restlessness, confusion, and behavioral changes. Still later during 
therapy, the so-called on-off phenomena may occur, in which severity of parkinsonism may 
quickly increase at any time of the day. 

(Choice 5) Dopamine agonists that act directly on dopamine receptors were once only used 
to reduce the symptoms associated with this on-off action associated with long-term 
levodopa use. However, with the advent of newer dopamine agonists that are not derived 
from ergot, such agonists are now used to help treat early Parkinson disease and to keep 
the levodopa dose (via Sinemet) at a minimal level as long as possible. 

((Choice 6)) is an irreversible monoamine oxidase B (MAO-B) inhibitor. MAO-B selectively 
deaminates dopamine and phenethylamine (chocolate’s amphetamine). Selegiline is 
sometimes used as an adjunct treatment, with the idea of reducing symptom fluctuations 
associated with long-term levodopa therapy. During the past decade, an ever-increasing 
number of otherwise refractory patients have been treated with deep brain stimulation, which 
has proven to be a relatively safe and effective treatment for the involuntary movements 
associated with Parkinson disease. 


47. Question 1 points 

One night, a 75-year-old man was driving his wife home from a concert when he realized he was 
having great difficulty in discerning the lines designating the lanes. In fact, he almost hit another car 
when he accidently made a lane change where the road suddenly curved. His wife was greatly 
upset and insisted that he should not drive at night until he has been cleared by a doctor. 
Consequently, he made an appointment to see his primary care physician, who had been treating 


him for type 2 diabetes and hypertension for over a decade; in providing a history, he informed the 
physician that he had become aware of gradually deteriorating vision; he stated that he had been 
noticing some difficulty in reading and that he also saw halos around light fixtures and headlights 
from oncoming cars during the night. Given this history, the most likely diagnosis would be which 
one of the following? 


1. Retinal detachment 

2. Age-related macular degeneration 

3. Diabetic retinopathy 

4. Hypertensive retinopathy 

5. Cataract 


INCORRECT X 

The correct answer is 5. 

This patient has cataracts. Although cataracts can be congenital or acquired (due to 
diseases like diabetes mellitus or from medications such as long-term steroids), they most 
commonly occur with advancing age as “senile cataracts” (by the age of 80 years most 
person have had cataracts or at least precataract changes in their lens). Cataracts may be 
central or peripherally located (i.e., nuclear or cortical). Patients complain of a gradual loss of 
vision, blurred vision, and difficulty in driving a car at night, as in this case. Some of them will 
state that they have a problem with perceiving colors, while others may state that their vision 
has improved such that they require reading glasses with less power. This is often called 
“second sight,” and results from a nuclear cataract. On the other hand, patients who develop 
posterior subcapsular cataract will complain of deterioration in near vision. Although 
cataracts are often perceived as an advent normally associated with aging, they are due to 
denaturation of proteins in the lens; depending upon the major protein affected, they may be 
hard or soft, partial or more complete, unchanging or progressive. Additionally, any action or 
substance that promotes protein denaturation will accelerate cataract formation. Treatment is 
surgery, in which the old lens is removed and is replaced by a plastic one. 

(Choice 1) presents as a sudden loss of vision, which is not the presentation in this case. 
(Choice 2) is associated with progressive loss of central vision, which can appear gradually 
or suddenly. 

(Choice 3) Although diabetes can promote diabetic retinopathy that may lead to deteriorating 
vision culminating in blindness, glare halos from headlights or light fixtures are not a feature. 
(Choice 4) is associated with deteriorating vision, but once again, halos caused by glare 
from bright lights are not a feature. 


48. Question 


1 points 



A 45-year-old man who is a conforming Seventh Day Adventist who works as a technician in the 
laboratory of a Veterans Administration Hospital complains of increasing fatigue over the past few 
months. He has had to buy new trousers as his waist has been increasing in size, and he has 
difficulty in putting on his shoes. Recently, he also has noticed swelling of his breasts. These 
physical changes are most likely due to which one of the following causes? 


1. Medication 

2. Alcohol 

3. Hepatitis A 

4. Hepatitis B 

5. Hepatitis C 


INCORRECT X 

The correct answer is 5. 

This patient has cirrhosis of the liver with portal hypertension. The expanding waist line is 
due to ascites, while swollen feet cause him to have difficulty in putting on his shoes. He has 
breast enlargement (gynecomastia) because the small amount of estrogen normally present 
in a male could not be metabolized by the failing liver. Other features would include palmar 
erythema and spider nevi. Approximately 20% of patients with chronic hepatitis C will end up 
with cirrhosis around 20 years after infection. Hepatitis C is most commonly transmitted via 
IV drug use. Other modes of transmission include sex, snorting cocaine, body piercing, 
tattoos, hemodialysis, blood transfusion, and upon occasion, an accidental finger stick by a 
laboratory worker. Unfortunately, there is no vaccine against hepatitis C, and the number of 
cases that would result in mortality and development of hepatocellular carcinoma is expected 
to increase threefold within the next one or two decades. 

(Choice 1) medication is incorrect. Drugs such as isoniazid can induce hepatitis, but this is 
not very common, and nor is it common for medication-induced hepatitis to lead to cirrhosis. 
(Choice 2) Alcoholic hepatitis is a major problem, but a conforming Seventh - day Adventist 
is not likely to be an alcoholic. Moreover, not all alcoholics with hepatitis develop cirrhosis of 
the liver. The incidence of chronic alcoholics who end up with cirrhosis of the liver is in the 
range of 10%—15%. 

(Choice 3) is transmitted from the fecal-oral route and usually resolves spontaneously. 
Chronic hepatitis and cirrhosis does not occur, and immunization is available. 

(Choice 4) can lead to cirrhosis of the liver, but the incidence is not as great as it is for 
hepatis C, and immunization against it is available. 


49. Question 


1 points 



On a hot August day in North Carolina, a 35-yearold Hispanic woman is seen by her primary care 
physician for progressive fatigue and a feeling of general malaise. She informs him that, over the 
past few days, she has been noticing difficulty in walking up a brush-covered slope leading to her 
home where she lives in the country, and she suspects she may be coming down with the flu. She 
has no history of chest pain, but did complain of some difficulty in breathing. She stated that she 
bruises easily. Apart from taking ibuprofen fairly regularly, which she purchases over the counter 
for arthritis, she has not been on any other any long-term medications. She had taken one ibuprofen 
a few hours prior to seeing her physician. She has no allergies. Physical examination reveals a 
moderately built and well-nourished female who has a low-grade fever and a yellowish tinge in the 
sclera. Her vital signs are within normal limits. She has normal heart sounds and no murmurs, 
gallops, or rubs. A few basal rales are present, but the lungs are clear otherwise. Examination of 
the abdomen is unremarkable except for mild tenderness in the right upper quadrant. She does 
have mild pitting edema in the pretibial area. The most likely cause for her problem is which one of 
the following? 


1. Lyme disease 

2. West Nile virus 

3. Rocky mountain spotted fever 

4. Sarcoidosis 

5. Q fever 


INCORRECT X 

The correct answer is 3. 

Several factors provided in the case history are consistent with a diagnosis of Rocky 
Mountain spotted fever (RMSF), a disease caused by the transmission of Rickettsia rickettsii 
via a bite by a tick. These factors include the following: 

■ The possibility of a tick bite is suggested by the statement that the patient had to walk up a 
brush covered slope in order to get home and that it was August, a time when ticks are most 
prevalent. 

■ The patient lives in North Carolina (despite the name, only about 3% of all cases occur in a 
Rocky Mountain state, and most cases occur in the South Atlantic states with North Carolina 
having the greatest incidence). 

■ Difficulty in breathing is likely an early sign of pneumonitis, as part of the acute respiratory 
distress syndrome (ARDS) often found in association with RMSF. 

■ Pitting edema is present, which is a common sign of interstitial nephritis, a disease often 
induced by 

R. rickettsii. 

■ There is tenderness in the right upper quadrant, possibly an early sign of hepatomegaly, 
also a trait commonly associated with RMSF. 

■ Yellowing of the sclera is likely an early sign of jaundice caused by excess 
hyperbilirubinemia due to hemolysis induced by RMSF. 


As in the case described, initially, patients will have symptoms akin to influenza, such as 
fever, headache and myalgia. However, careful observation will discern early signs of some 
of the typical complications described above. After about the first week of the illness, a 
defining macular rash that progresses centripetally is usually seen. The initial rashes are at 
the wrists and ankles, and these may become petechial as they progress and as 
thrombocytopenia supervenes. In addition to the complications mentioned, a necrotizing 
vasculitis and uremia can also result. The diagnosis is confirmed by serology. Other 
laboratory finding suggestive of RMSF include elevated liver enzymes, thrombocytopenia, 
and hyponatremia. Treatment most typically is a tetracycline. 

(Choice 1) Although Lyme disease is also transmitted by a tick, the incidence of new cases 
is highest in the New England states and is relatively low in North Carolina; even more 
significant to the contention that this is an incorrect choice is that early signs do not include 
hepatomegaly, respiratory problems, interstitial nephritis, or jaundice. Similarly, although 
interstitial nephritis and consequently pitting edema can be caused by several entities in 
addition to RMSF including prolonged use of nonsteroidal anti-inflammatory drugs (NSAIDs); 
some antibiotics, such as penicillin and cephalosporins; and infections other than RMSF, 
such as those caused by cytomegalovirus and streptococcus none of these factors is 
included among the choices nor do they cause the other symptoms described as associated 
with RMSF. 

(Choice 2) West Nile virus is a flavivirus transmitted to humans by a mosquito bite and may 
cause encephalitis. The key features are neurological in nature, such as headache, fever, 
nuchal rigidity, stupor, and coma. Spastic paralysis and upper motor neuron signs and 
presence of lymphocytosis and elevated proteins on cerebrospinal fluid examination are 
present as well. 

(Choice 4) is a disease of unknown etiology that primarily causes granulomatous 
inflammation in the lungs. It is more common in women than in men and among African 
American and Northern European populations. Being a systemic disease, it can target 
various organs, including the skin (causing erythema nodosum), heart, liver, spleen, kidneys, 
salivary glands, and peripheral nerves. Diagnosis is by histology, in which noncaseating 
granulomas are found on biopsy. 

(Choice 5) is a rickettsial disease caused by Coxiella burnetii; it is transmitted by inhalation 
and not by vectors, as are many rickettsial diseases. It is usually seen in patients who are 
exposed to farm animals such as goats, sheep, and cattle. The patient presents with fever, 
headache, cough, and abdominal pain. Pneumonitis, hepatitis, and in some cases, 
endocarditis and encephalitis can ensue. The diagnosis is confirmed by serology. 


50. Question 1 points 

A 20-year-old man comes to his family physician with a complaint concerning fainting attacks. He 
states that he has started going to the gym where he uses the treadmill and lifts some weights. He 
feels faint and sometimes actually passes out after being on the treadmill for some time; even more 
distressing, he almost always passes out soon after he begins to lift heavy weights. His father also 
had episodes of fainting; furthermore, he died following a heart attack at the age of 54 years. The 




present patient has had a “double pulse” for a long time, and it has never bothered him. In fact, he 
likes to show off his special pulse to his friends. He denies chest pain, but does complain of some 
breathing difficulty. Upon examination, the physician observed a distinct cardiac murmur. This 
murmur would be expected to do which of the following? 


1. Increase when he sits upright or when he squats 

2. Decrease when he sits upright and remain unchanged when he squats 

3. Increase when he holds his breath or when he squats 

4. Decrease when he holds his breath or when he squats 

5. Increase when he sits upright and decrease when he squats 


INCORRECT X 

The correct answer is 5. 

This patient has hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy is a condition in 
which the left ventricular space is decreased due to the disproportionate hypertrophy of the 
interventricular septum. The restricted space reduces the amount of outflow. Performing a 
Valsalva maneuver or sitting upright increases the loudness of the murmur, whereas 
squatting reduces it. This is because a Valsalva maneuver or sitting upright decreases left 
ventricular filling and obstructs outflow. Likewise, sympathetic stimulation (as occurs during 
exercise) does the same, leading to symptoms such as syncope and even dangerous 
arrhythmias. Patients with hypertrophic cardiomyopathy have pulsus bisferiens, in which two 
peaks are felt, one following the other the “double pulse” described by the patient. Chest pain 
and dyspnea may be symptoms as well. Hypertrophic cardiomyopathy can be inherited as 
an autosomal dominant trait. It can result in dangerous arrhythmias and sudden death. His 
father must have died from sudden arrhythmias. The fact that the patient has the same 
disorder points to a genetic cause. A defibrillator should be implanted in patients who have a 
history of syncope, recurrent ventricular arrhythmias, or a family history of sudden death. 
(Choices 1,2,3 & 4) Since Choice 5 is correct therefore all other choices are incorrect. 






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