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A 27-year-old woman goes to an emergency room with severe abdominal pain.

She had previously experienced similar episodes of pain that


Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
Question 1 of 5

The porphyrias are biochemical abnormalities in which of the following


pathways?
/A. GIycogen degradation
/B. Heme synthesis
/C. Lipoprotein degradation
/D. Nucleotide degradation
/E. Urea cycle
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Explanation - Q: 1.1

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The correct answer is B. The porphyrias are a group of rare, related


diseases that have in common a block in the heme synthesis pathway. The
block is usually partial rather than complete, and thus many of these patients
have only intermittent symptoms. Most cases of porphyria present with either
a neurovisceral pattern (including both psychiatric symptoms and abdominal
pain) or with photosensitive skin lesions. These two patterns are associated
with different forms of porphyria.
Associate abnormalities of glycogen degradation (choice A) with the
glycogen storage diseases, such as von Gierke disease, Pompe disease, and
Forbes disease.
Associate abnormalities of lipoprotein degradation (choice C) with some
forms of hyperlipoproteinemia (notably Type I).
Associate abnormalities of nucleotide degradation (choice D) with gout and
Lesch-Nyhan syndrome.
Associate abnormalities of the urea cycle (choice E) with congenital
hyperammonemia, citrullinemia, and argininosuccinic acidemia.
A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.

The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
Question 2 of 5

Following the surgery, the decision is made to screen for the porphyrias that
cause acute neurovisceral symptoms. Which of the following
tests would be most likely to be used?
/A. Erythrocyte porphyrins
/B. Total fecal porphyrins
/C. Total plasma porphyrins
/D. Total urinary porphyrins
/E. Urinary porphobilinogen
Explanation - Q: 1.2

Close

The correct answer is E. The acute neurovisceral porphyrias are those that
tend to present with severe abdominal pain, often accompanied by
neuropsychiatric symptoms. The best tests to use for screening of these
diseases are urinary porphobilinogen (PBG, either random or 24 hour) and
urinary delta-aminolevulinic acid (ALA, either random or 24 hour).
Erythrocyte porphyrins (choice A) are used for follow-up in the photosensitive
types of porphyria.
Total fecal porphyrins (choice B) are used for follow-up evaluation after
screening tests for either the photosensitive porphyrias or the acute
neurovisceral porphyrias are positive.
Total plasma porphyrias (choice C) are useful for first line screening of the
photosensitive porphyrias, and are used for further evaluation after screening
in the acute neurovisceral porphyrias.
Total urinary porphyrins (choice D) are used for further evaluation after
screening for acute neurovisceral porphyrias.
A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon

comments that he had once seen a similar case that was actually due to
porphyria.
uestion 3 of 5

Which of the following are the three most common forms of porphyria?
/A. Acute intermittent porphyria, erythropoietic protoporphyria, and porphyria
cutanea tarda
/B. Acute intermittent porphyria, hepatoerythropoietic porphyria, and variegate
porphyria
/C. Congenital erythropoietic porphyria, delta-aminolevulinic acid dehydratasedeficient porphyria, and hepatoerythropoietic porphyria
/D. Erythropoietic protoporphyria, hereditary coproporphyria, and porphyria
cutanea tarda
/E. Hereditary coproporphyria, variegate porphyria, and X-Iinked sideroblastic
anemia
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Explanation - Q: 1.3

Close

The correct answer is A. The porphyrias are complex diseases that can
easily appear overwhelming. A very useful point to know (both clinically and
for the USMLE) is that the three most common forms are acute intermittent
porphyria, erythropoietic protoporphyria, and porphyria cutanea tarda. Acute
intermittent porphyria tends to present with acute neurovisceral symptoms.
Erythrocytic protoporphyria tends to present acutely with painful skin lesions.
Porphyria cutanea tarda tends to present with chronic blistering skin lesions.
The other types listed in various choices are also porphyrias, but are less
common.
A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
uestion 4 of 5

This patient is found to have increased levels of both delta-aminolevulinic acid


(ALA) and porphobilinogen (PBG) in blood. Follow-up studies
demonstrate low PBG deaminase in erythrocytes. AIso, additional history is
elicited, revealing that the woman had started a very low

carbohydrate diet about one week before being admitted to the hospitaI. Which
of the following is the most likely diagnosis?
/A. Acute intermittent porphyria
/B. Congenital erythropoietic porphyria
/C. Erythropoietic protoporphyria
/D. Porphyria cutanea tarda
/E. X-Iinked sideroblastic anemia
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Explanation - Q: 1.4

Close

The correct answer is A. These laboratory findings are most consistent with
acute intermittent porphyria, which is due to PBG deaminase deficiency.
Patients usually, but not always, have a deficiency of erythrocyte PBG
deaminases. (Some cases have also been described in which the enzyme
deficiency is limited to liver.) The condition is an autosomal dominant disorder
that typically becomes symptomatic in women after puberty, and then often
only if a precipitating event (dieting, use of certain drugs, premenstrual) is
also present. Symptoms during the attacks can include abdominal symptoms
(pain, nausea, vomiting, constipation, diarrhea, abdominal distension, ileus),
which are thought to be due to the effects of this condition on visceral nerves.
Other symptoms that may be mediated neurologically include incontinence,
urinary retention, tachycardia, diaphoresis, hypertension, muscle weakness,
psychiatric symptoms, seizures, and rarely, severe paralysis, respiratory
insufficiency, and death. Both intravenous glucose (oral is often inadequate
due to poor intestinal function) and exogenous heme supplementation can
suppress the heme biosynthetic mechanism, and tend to ameliorate the acute
attack. Patients should be cautioned to diet gently, as intense dieting can
precipitate attacks.
Congenital erythropoietic porphyria (choice B) is characterized by severe
skin blistering that usually begins after birth, pink to dark-brown urine, normal
ALA and PBG, and increased porphyrins (primarily uroporphyrin I and
coproporphyrin I) in urine, plasma, and erythrocytes.
Erythropoietic protoporphyria (choice C) is characterized by cutaneous
photosensitivity that begins early in life and high protoporphyrin in
erythrocytes and bone marrow.
Porphyria cutanea tarda (choice D) is characterized by photosensitivity with
skin blistering, elevated plasma porphyrins, and elevated urine porphyrins
(mostly uroporphyrin and heptacarboxylporphyrin).
The very rare X-linked sideroblastic anemia (choice E), due to a deficiency of
delta-aminolevulinic acid synthase, can clinically resemble acute intermittent
porphyria, and is characterized by elevated levels of urinary ALA and

coproporphyrin.
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A 27-year-old woman goes to an emergency room with severe abdominal pain.


She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
Question 5 of 5

Which of the following drugs would be most likely to induce an attack of


abdominal pain in this patient?
/A. Acetaminophen
/B. Aspirin
/C. Barbiturate
/D. GIucocorticoid
/E. Insulin
Explanation - Q: 1.5

Close

The correct answer is C. Some symptomatic episodes of acute porphyria


(including acute intermittent porphyria, hereditary coproporphyria, variegate
porphyria, and aminolevulinic acid dehydratase porphyria) are triggered by
drug ingestion, and administration of drugs to undiagnosed patients can
cause an acute exacerbation of an ongoing attack of acute porphyria. Drugs
considered unsafe for use in these patients notably include alcohol,
anticonvulsants, barbiturates, many other sedatives, and sulfonamide
antibiotics. Of particular concern are the sedative agents, since it may be very
tempting to give an obviously agitated patient a sedative to allow easier
examination of the patient. Many other drugs are also on the lists of
potentially dangerous drugs in these patients. Once the diagnosis is
established, the patient should be instructed to always inform her/his
physician of her condition, and ask that the safety of drugs prescribed in
patients with porphyria be checked. Many of the drugs that can induce or
exacerbate an attack of porphyria do so by increasing the activity of the
cytochrome P450 system, which indirectly triggers an increase in heme
biosynthesis. The other medications listed in the choices are "safe" in these
patients.

A 47-year-old woman presents to the emergency department with


cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 1 of 7

Which of the following organs is the most likely source of this woman's pain?
/A. Appendix
/B. Diaphragm
/C. Esophagus
/D. Gallbladder
/E. Stomach
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Explanation - Q: 2.1

Close

The correct answer is D. This woman most likely has gallstones.


Cholelithiasis, or the formation of calculi (gallstones) within the gallbladder, is
very common in the United States, with over 500,000 cholecystectomies
being performed yearly. While many cases of gallstone disease are
symptomatic, right upper quadrant pain with referral of the pain to the lower
right scapula should specifically suggest gallbladder disease. The pattern of
episodes of several hours of pain followed by long periods of freedom from
pain is also typical of symptomatic gallstone disease.
The appendix (choice A) would most likely cause lower abdominal pain.
Pain from irritation of the diaphragm (choice B) can cause right upper
quadrant pain and referred pain in the supraclavicular area (rather than the
subscapular pain of biliary colic). The absence of right upper quadrant
tenderness to palpation, and the history of pain after a fatty meal also argue
against this diagnosis.
Esophageal pain (choice C) related to regurgitation of gastric contents
(heartburn) can occur postprandially, but tends to radiate into the neck, throat,

or even face.
Peptic ulcer pain of gastric origin (choice E) is usually described as causing
burning, gnawing, or hunger, and may be relieved by eating.
A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 2 of 7

Which of the following techniques would be most appropriate to demonstrate the


patient's most likely diagnosis?
/A. Colonoscopy
/B. CT scan of the abdomen
/C. Esophagoduodenoscopy
/D. MRI scan of the abdomen
/E. UItrasonography
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Explanation - Q: 2.2

Close

The correct answer is E. Real-time ultrasonography, with 98% sensitivity


and 95% specificity, is considered the method of choice for diagnosing
possible gallbladder stones.
Colonoscopy (choice A) and esophagoduodenoscopy (choice C) might be
helpful for excluding alternative diagnoses, but would not themselves
establish a diagnosis of gallstone disease.
CT (choice B) and MRI (choice D) scans of the abdomen are expensive
tests whose use is not warranted, since real-time ultrasonography performs
as well or better.
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A 47-year-old woman presents to the emergency department with


cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of

freedom from pain. On physical examination, she is noted to have tenderness to


deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 3 of 7

Following appropriate diagnostic studies, the patient is taken to the surgical suite.
During the surgery, the surgeon inserts his fingers from
right to left behind the hepatoduodenal ligament. As he does so, his fingers enter
which of the following?
/A. Ampulla of Vater
/B. Common bile duct
/C. Epiploic foramen
/D. Greater peritoneal sac
/E. Portal vein
Explanation - Q: 2.3

Close

The correct answer is C. The space behind the stomach, hepatoduodenal


ligament, and hepatogastric ligament is the omental bursa. This space can be
entered by passing through the epiploic foramen of Winslow, as described in
the question stem.
The common bile duct enters the duodenum through the ampulla of Vater
(choice A).
The hepatoduodenal ligament contains the common bile duct (choice B), the
portal vein (choice E), and the hepatic artery.
The greater peritoneal sac (choice D) lies anterior to the stomach and
hepatoduodenal ligament.
A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 4 of 7

During the cholecystectomy, the surgeon ligates the cystic artery. This is typically
a branch of which of the following?

/A. Gastroduodenal artery


/B. Left gastroepiploic artery
/C. Right gastroepiploic artery
/D. Right hepatic artery
/E. Superior pancreaticoduodenal

artery
Explanation - Q: 2.4

Close

The correct answer is D. The cystic artery is generally a branch of the right
hepatic artery.
The gastroduodenal artery (choice A) is a branch of the (common) hepatic
artery.
The left gastroepiploic artery (choice B) is a branch of the splenic artery.
The right gastroepiploic artery (choice C) is a branch of the gastroduodenal
artery.
The superior pancreaticoduodenal artery (choice E) is a branch of the
gastroduodenal artery.
A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 5 of 7

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Pathologic examination of the specimen removed by the surgeon demonstrates


the presence of numerous yellow stones (shown above).
These are most likely composed primarily of which of the following?
/A. Bilirubinate
/B. Calcium phosphate
/C. Cholesterol
/D. Cystine
/E. Struvite
Explanation - Q: 2.5

Close

The correct answer is C. The stones are gallstones, and their yellow color
indicates that they are composed of cholesterol. Cholesterol stones are the
most common form of gallstones. Risk factors include female sex, multiparity,
obesity, increased age (female, fat, forty, and fertile) and North American
Indian race.
Bilirubinate (choice A) gallstones, which are usually associated with
hemolytic anemias, are less common, brown, rather than yellow, and often
faceted.
Calcium phosphate (choice B), cystine (choice D), and struvite (choice E)
composition can be seen in kidney stones.
A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of

freedom from pain. On physical examination, she is noted to have tenderness to


deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 6 of 7

If this patient had a small stone lodge near the ampulla of Vater, which of the
following complications would be most likely to occur?
/A. Crohn disease
/B. Diabetes mellitus
/C. Pancreatitis
/D. Peptic ulcer
/E. Polyarteritis nodosa

Explanation - Q: 2.6

Close

The correct answer is C. A small gallstone obstructing the pancreatic


outflow is a well-known cause of acute pancreatitis. The other conditions
listed are not caused by gallstones.
A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 7 of 7

If this patient had refused surgical treatment, which of the following would be the
most appropriate pharmacotherapy to provide definitive
treatment and thereby relieve associated pain?
/A. Ampicillin
/B. CIofibrate
/C. Meperidine
/D. Oxycodone
/E. Ursodiol
Explanation - Q: 2.7

Close

The correct answer is E. The question is asking, "Which of the following will
eradicate a gallstone?" When a gallstone is eliminated the pain will
subsequently be eliminated. This question is NOT asking, "which of the
following is the most appropriate form of pain control?". Ursodiol
(ursodeoxycholic acid) is a hydrophilic bile acid that is used to dissolve small
(< 20 mm), non-calcified, radiolucent cholesterol gallstones in patients with
functioning gallbladders who cannot undergo (or refuse) cholecystectomy.
Analgesics and antibiotics, such as ampicillin (choice A), are administered
when appropriate, but do not help eradicate the stones.
Clofibrate (choice B) is an antihyperlipidemic that is associated with the
development of gallstones. High-risk patients, such as diabetics and the
elderly, should be watched closely.
As a side note, if this question were asking: "which of the following is the most
appropriate form of pain control in this patient", the most appropriate answer
would be meperidine. Meperidine (choice C) is the narcotic of choice since it
causes the least amount of spasm of the sphincter of Oddi. In other words,
meperidine is preferred over oxycodone (choice D).
A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 1 of 5

Which of the following is the most likely diagnosis?


/A. Colon carcinoma
/B. Infectious colitis
/C. Inflammatory bowel disease
/D. Ischemic colitis
/E. Necrotizing enterocolitis
Explanation - Q: 3.1

Close

The correct answer is D. A patient with severe abdominal pain and rectal

bleeding with an unremarkable physical examination is likely suffering from


ischemic colitis. "Pain out-of-proportion to examination" is a classic finding for
ischemic colitis. The previous episodes of less severe pain represent
ischemic angina. An infarction has occurred, as indicated by the rise in serum
lactate secondary to the colon's anaerobic metabolism. The history of
coronary artery disease also suggests this diagnosis, as the atherosclerotic
processes that contribute to his CAD are also likely present in his abdominal
vasculature.
Colon cancer (choice A) would produce less acute symptoms, but
occasionally, colon cancer may present acutely with obstructive symptoms.
Patients may have bleeding and abdominal pain, but the pain is typically
intermittent and accompanied by nausea, vomiting, abdominal distention, and
absence of flatus.
Infectious colitis (choice B) is incorrect. While patients may have bleeding
and abdominal pain, nothing in the history suggests a disease of infectious
origin (no sick contacts or foreign travel). The acute onset also suggests a
vascular event, rather than an infectious one.
Inflammatory bowel disease (IBD) (choice C) is incorrect because while the
patient reports previous episodes, an elderly man with IBD would likely have
a chronic history of abdominal pain and bleeding.
Necrotizing enterocolitis (choice E) affects premature infants and would not
be relevant in this setting.
A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 2 of 5

The lactate produced from the anaerobic metabolism in the infarcted gut will
likely be which of the following?
/A. Exhaled as a fruity odor
/B. Incorporated into glycogen in the liver
/C. Incorporated into myoglobin in muscle
/D. Incorporated into urea in the urine

/E.

Secreted by the kidneys unchanged


Explanation - Q: 3.2

Close

The correct answer is B. Lactate is converted into glucose, and then


glycogen in the liver by a process know as the Cori cycle.
Choice A is incorrect, as lactate would not be exhaled. A fruity odor on the
breath would be a sign of ketoacidosis.
While some of the carbon from the lactate may be incorporated into peptides
via Krebs intermediates (e.g., choice C), the vast majority would be left as
carbohydrate.
Urea (choice D) represents a means of eliminating nitrogenous waste.
Choice E is wrong, as the kidneys would retain the lactate, rather than
excreting it.
A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 3 of 5

If this patient's disease were drug-induced, which of the following agents would
most likely be responsible?
/A. Acetaminophen
/B. Amiodarone
/C. Cocaine
/D. Dexamethasone
/E. Nitroglycerin
Explanation - Q: 3.3

Close

The correct answer is C. Cocaine is a sympathomimetic drug that indirectly


acts on both the alpha and beta adrenergic receptors on the vasculature. As
such, cocaine may cause vasospasm in the abdominal vasculature leading to

infarction and ischemic colitis. Similar vasospastic events may occur in the
coronary vasculature, leading to myocardial infarction.
Acetaminophen (choice A) is an analgesic, and would not play a role in
producing ischemic colitis.
Amiodarone (choice B) is an antiarrhythmic, and would not contribute to
ischemic colitis.
Dexamethasone (choice D) is a steroidal anti-inflammatory drug. Not only
would this medication not cause ischemic colitis, it might mask the symptoms
due to its potent anti-inflammatory properties.
Nitroglycerin (choice E) is a venodilator, and would not contribute to ischemic
colitis. As a venodilator, nitroglycerin is used to treat coronary ischemia by
reducing cardiac preload.
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A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 4 of 5

While the patient is in the emergency department, the pain becomes increasingly
severe. Several hours after his initial examination, the
patient becomes febrile and is now exquisitely tender to palpation. He writhes in
pain when the physician jostles the bed. Air is seen under
the diaphragm in an upright chest x-ray film. These new findings suggest which
of the following?
/A. Abdominal aortic aneurysm
/B. Bowel obstruction
/C. Cholecystitis
/D. Hypovolemia
/E. Perforation with peritonitis
Explanation - Q: 3.4

Close

The correct answer is E. This patient has experienced a bowel perforation.


Air under the diaphragm in an upright chest film provides definitive evidence
that a hollow viscus has ruptured. Air near the liver on a left lateral decubitus

(patient lays with the left side down) is an alternative study to demonstrate
perforation. Spillage from the perforated bowel has irritated and inflamed the
peritoneum, resulting in peritonitis. Symptoms of peritonitis include extreme,
sharp pain exacerbated by jostling (patients often report that the bumpy ride
to the emergency department caused extreme pain). Patients will be
exquisitely tender to palpation and percussion and may have abdominal
rigidity. Fever typically accompanies peritonitis.
While an abdominal aortic aneurysm or AAA (choice A) presents as acute
abdominal pain, this pain is described as tearing and may radiate to the back.
A pulsatile abdominal mass may be palpated. The air on the chest film is also
inconsistent with AAA.
This patient does not have bowel obstruction (choice B). Signs and
symptoms of bowel obstruction include: nausea, vomiting, intermittent
abdominal pain, hypovolemia, abdominal distention, absence of flatus, and a
"step ladder" bowel pattern on abdominal films.
Cholecystitis (choice C) typically presents as right upper quadrant (RUQ)
pain, fever, and jaundice. Patients usually have a history of colicky RUQ pain.
While the patient is at risk for hypovolemia (choice D), none of the symptoms
listed typify hypovolemia. Signs and symptoms of mild to moderate
hypovolemia include malaise, dry mouth, thirst, decreased skin turgor,
tachycardia, hypotension, and decreased urine output.
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A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 5 of 5

Upon surgical exploration of the abdomen, the colon is dull and dusky from the
mid transverse colon to the rectum. The patient has occluded
which of the following vessels?
/A. Celiac trunk
/B. Cystic artery
/C. External iliac artery
/D. Inferior mesenteric artery
/E. Superior mesenteric artery

Explanation - Q: 3.5

Close

The correct answer is D. The inferior mesenteric artery distributes blood to


the embryologic hindgut. This includes the distal 1/3 of the transverse colon
to the rectum. The rectum is spared because it receives circulation from the
inferior rectal artery (not mesenteric).
The celiac trunk (choice A) supplies the embryologic foregut. The first three
branches include the splenic artery, the left gastric artery, and the common
hepatic artery. This patient has no findings in this distribution.
The cystic artery (choice B) supplies the gall bladder. There are no gall
bladder findings in this case.
The external iliac artery (choice C) gives rise to the vessels of the lower
extremity. Symptoms of occlusion or stenosis might include buttock and thigh
pain exacerbated by walking. Severe stenosis might give patients buttock and
thigh pain, even at rest.
The superior mesenteric artery (choice E) supplies the embryologic hindgut.
This extends from the duodenum to the proximal 2/3 of the transverse colon.
A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 1 of 5

Which of the following would be the most likely cause of this type of pain?
/A. Acute appendicitis
/B. Acute hepatitis
/C. Acute pancreatitis
/D. Chronic hepatitis
/E. Myocardial infarction
Explanation - Q: 4.1

Close

The correct answer is C. The typical pain described occurs in approximately


50% of patients with acute pancreatitis. Other patients may have milder pain
or even, uncommonly, pain first felt in the lower abdomen.
The pain of acute appendicitis (choice A) is often felt first as referred pain
near the umbilicus, with tenderness on palpation in the left lower quadrant.

Acute hepatitis (choice B) can cause pain referred to the right shoulder.
Chronic hepatitis (choice D) does not usually cause pain.
Myocardial infarction (choice E) can cause substernal pain and pain radiating
to the left shoulder.
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A 45-year-old man goes to an emergency department because he is


experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 2 of 5

In addition to alcohol use, which of the following is a common predisposing factor


for this patient's disease?
/A. Biliary tract stones
/B. Duodenal cancer
/C. Gastric carcinoma
/D. Kidney stones
/E. Peptic ulcer
Explanation - Q: 4.2

Close

The correct answer is A. The overwhelmingly most common predisposing


factors for acute pancreatitis are gallstones (more specifically tiny ones that
lodge in the extrahepatic bile duct system) and alcohol abuse.
Rarely, nearby cancers (choices B and C) can occlude the pancreatic duct
system and cause a secondary acute pancreatitis.
Kidney stones (choice D) have no relationship with pancreatitis.
Peptic ulcers (choice E) that erode into the pancreas can uncommonly
secondarily inflame the pancreas.
A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 3 of 5

Marked serum elevation of which of the following markers would most strongly
substantiate the likely diagnosis?
/A. Acid phosphatase

/B. Amylase
/C. Aspartate aminotransferase
/D. AIkaline phosphatase
/E. Creatinine kinase

Explanation - Q: 4.3

Close

The correct answer is B. The usual markers for pancreatitis are amylase
and lipase. Marked elevation of amylase usually means either pancreatic
disease or salivary gland disease; lipase will be elevated in pancreatic
disease but not salivary gland disease. If you see elevated amylase on a
USMLE question, you should think of pancreatitis or salivary gland disease
(mumps, salivary gland stone). However, you should be aware, for your
general medical knowledge, that modest elevations of amylase can be seen
in a much wider variety of settings (often reflecting either subclinical
pancreatic damage or hemoconcentration of pancreatic enzymes), including
GI obstruction, mesenteric thrombosis and infarction, macroamylasemia (a
genetic condition with abnormal amylase), renal disease, ruptured tubal
pregnancy, lung cancer, acute alcohol ingestion, and following abdominal
surgery.
Associate acid phosphatase (choice A) with diseases involving the prostate
and, to lesser degrees, bone, the heart, platelets, and the liver.
Associate aspartate aminotransferase (choice C) with diseases of the heart,
muscle, liver, pancreas (though not as important for diagnosis as amylase
and lipase), and brain.
Associate alkaline phosphatase (choice D) with diseases of bone, liver, and
to lesser degrees, lung and heart.
Associate creatinine kinase (choice E) with diseases of the heart, muscle,
brain, and the general body (trauma, surgery).
A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 4 of 5

The patient has a severe course that requires treatment in an ICU. CIinically, he
appears similar to patients with sepsis, with fever, elevated
white count, hypotension, increased pulse rate, shallow and rapid breathing,
oliguria, and a blunted sensorium, in addition to his pain and
abdominal tenderness. These clinical findings are most likely related to which of
the following?
/A. Activation of the inflammatory cascade
/B. AIcohol withdrawal symptoms

/C. AIIergic reaction to alcohol


/D. Drug toxicity effect
/E. Secondary infection with mixed

flora gut bacteria


Explanation - Q: 4.4

Close

The correct answer is A. Acute pancreatitis can either be relatively mild, or a


severe condition that may cause death. It is thought that, in severe cases,
leakage of enzyme-containing pancreatic secretions into the tissues/and or
blood stream causes cleavage of precursors, thus strongly activating the
complement and inflammatory cascades. These, in turn, produce abundant
cytokines, which worsen the symptoms. The clinical result is similar to sepsis,
with risk of multi-organ failure and death. The treatment of acute pancreatitis
is primarily supportive, and may include careful attention to fluid resuscitation,
oxygen supplementation, cardiovascular support, dialysis, management of
electrolyte abnormalities, pain control, and total parenteral nutrition.
Alcohol allergy (choice C) or withdrawal (choice B) do not play any
additional part in most of these symptoms once the pancreatitis has
developed.
Infection (choice E) and drug toxicity (choice D) are also not a necessary
part of the clinical picture, although physicians may worry that the patient's
general clinical status is masking other, potentially more treatable, problems.
A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 5 of 5

The patient's condition resolves in about two weeks, but he continues to drink
after leaving the hospitaI. When seen several years later, he
has had a number of similar episodes, and now has chronic severe abdominal
pain. CT scan demonstrates a single, smooth-walled, fluid
filled space in the tail of the pancreas, which can be reached by the radiologist
for CT-guided aspiration with an approach from the back. The
fluid aspirated is yellowish, clear, and acellular. Which of the following is the most
likely diagnosis?
/A. Pancreatic microcystic adenoma
/B. Pancreatic mucinous cystadenocarcinoma
/C. Pancreatic mucinous cystadenoma
/D. Pancreatic pseudocyst
/E. Pancreatic solid-cystic tumor
Explanation - Q: 4.5
The correct answer is D. Pancreatic pseudocyst is a fairly common

Close

complication of both acute and chronic pancreatitis, and appears to develop


when trapping of pancreatic digestive juices (containing amylase, lipase, and
proteases) causes a "digestion" of part of the pancreas, leaving a fluid filled
cystic space. The term "pseudocyst", rather than "cyst", is used by purists
because the space does not have an epithelial lining, and is hence not a "true
cyst". Pseudocysts are usually solitary and typically measure 5-10 cm in
diameter. They can be surgically excised (and the surrounding tissue will
typically show evidence of chronic pancreatitis in long-standing cases) or
sometimes, if the anatomy is favorable, drained into adjacent hollow viscera.
Some are medically managed if small.
Most true neoplasms of the pancreas contain (often large numbers of)
smaller, multiple, cysts. These tumors can be benign or malignant, and the
ones with mucus-secreting epithelium (choices B and C) are more common
than those with a serous lining (choices A and E).
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A 17-year-old boy is taken to the emergency department because he has


developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a lowgrade fever.
Question 1 of 5

Examination of the abdomen demonstrates right lower quadrant tenderness at


the junction of the middle and outer thirds of the line joining the
umbilicus to the anterior superior spine of the iliac. This location is known as
which of the following?
/A. Gubernaculum
/B. Langer's line
/C. Linea alba
/D. McBurney's point
/E. Tunica albuginea
Explanation - Q: 5.1

Close

The correct answer is D. The point described is McBurney's point, which


overlies the location of the appendix in most individuals.
The gubernaculum (choice A) is the fibrous cord that connects the primordial
testis or ovary to the anterolateral abdominal wall.
Langer's lines (choice B) are the cleavage lines of the skin.
The linea alba (choice C) is a sheet-like aponeurosis that covers the anterior

abdominal wall.
The tunica albuginea (choice E) is a tough fibrous coat that covers the testis.
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A 17-year-old boy is taken to the emergency department because he has


developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a lowgrade fever.
Question 2 of 5

Which of the following is the most likely diagnosis?


/A. Appendicitis
/B. Diverticulitis
/C. Gallstones
/D. Rectal ulcer
/E. Renal colic
Explanation - Q: 5.2

Close

The correct answer is A. This patient has a typical presentation for


appendicitis, and the diagnosis is confirmed by the presence of localized
tenderness at McBurney's point.
Diverticulitis (choice B) is usually a disease of middle-aged or older
individuals and most commonly affects the left-lower quadrant.
Symptomatic gallstone disease (choice C) causes pain and tenderness in the
right upper quadrant.
Rectal ulcer (choice D) causes pain with stool movement, but does not
usually produce tenderness identifiable on abdominal examination.
Renal colic (choice E) usually produces flank or lower back pain.
A 17-year-old boy is taken to the emergency department because he has
developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a lowgrade fever.
Question 3 of 5

The patient also exhibits an increase in pain in the right lower quadrant from the
passive extension of the right hip joint. This finding suggests
that the inflammation also involves which of the following?
/A. BIadder
/B. External oblique muscle
/C. Femur
/D. IIiopsoas muscle
/E. Transverse abdominal muscle
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Explanation - Q: 5.3

Close

The correct answer is D. This patient has a "positive psoas sign," which is
an increase in pain from passive extension of the right hip joint. This
maneuver stretches the iliopsoas muscle, which lies behind the appendix and
can become secondarily inflamed when the appendiceal inflammation
extends through the serosa. The psoas sign is clinically useful in both
confirming the appendix as the probable origin of the patient's pain, and
indicating that the inflammation is transmural and that the risk of rupture and
peritonitis is increased.
The bladder (choice A) is located more medially, and is usually not affected
by appendicitis.
The external oblique (choice B) and transverse abdominal (choice E)
muscles are in the anterior and lateral abdominal walls, and do not usually
become inflamed with appendicitis.
The femur (choice C) is moved during the extension of the right hip joint, but
is not the source of the pain.
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A 17-year-old boy is taken to the emergency department because he has


developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a lowgrade fever.
Question 4 of 5

The patient is prepared for immediate surgery. Cefotaxime is administered


before, during, and after surgery. The specimen, once removed,
is sent to the laboratory for pathology and bacteriologic culture. A malodorous
pus surrounds the serosa of the surgical specimen, and a
mixed gram-negative flora is cultured. Rapid enzyme tests for beta-Iactamase
production are positive. Which of the following drugs should be
added to the initial cefotaxime regimen?
/A. Bacitracin

/B. CIavulanic acid


/C. CIindamycin
/D. Isoniazid
/E. Vancomycin

Explanation - Q: 5.4

Close

The correct answer is B. Clavulanic acid is a beta-lactamase inhibitor, which


when administered with beta lactam agents, irreversibly binds and inactivates
bacterial beta-lactamases, thereby permitting the companion drug to disrupt
bacterial cell wall synthesis. Suspected appendicitis is usually treated with
prompt appendectomy, since delay is associated with increased risk of
potentially life-threatening peritonitis and sepsis.
Bacitracin (choice A) is not correct, since this drug inhibits bacterial cell wall
synthesis by binding to and inhibiting the dephosphorylation of a membranebound lipid pyrophosphate. Gram-negative bacteria are resistant to this
agent, and it would not have a synergistic effect if administered with a third
generation cephalosporin.
Clindamycin (choice C) is not correct, because this drug blocks protein
elongation by binding to the 50S ribosome. Although it is effective against
anaerobic gram-negative bacilli, it would not have a complementary effect
when administered with a third generation cephalosporin.
Isoniazid (choice D) is not correct because it inhibits the synthesis of mycolic
acids for the cell wall of actively dividing Mycobacteria. It would not be
effective in the flora of this patient's gut, nor would it act synergistically with
third generation cephalosporins.
Vancomycin (choice E) is not correct because it disrupts cell wall synthesis in
growing gram-positive bacteria. It would not be effective against the flora of
this patient's gut, nor would it act synergistically with third generation
cephalosporins.
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A 17-year-old boy is taken to the emergency department because he has


developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a lowgrade fever.
Question 5 of 5

The patient's postoperative recovery is uneventfuI, but 10 days after discharge,


he returns to his physician complaining of continuous lowgrade fever. An abscess is drained transrectally, and
organisms are
cultured from the pus. Which of the following is an attribute of

this organism that makes it an important abscess former?


/A. It is an anaerobe
/B. It is an intracellular pathogen
/C. Its endotoxin lacks 2,3-ketodeoxyoctonate
/D. Mycolic acid
/E. Prodigious capsule
Explanation - Q: 5.5

Close

The correct answer is E. Prevotella (Bacteroides) is a frequent cause of


abscesses in the intestinal tract because it is a normal flora organism and
produces a large capsule, which impedes phagocytosis.
Although the genus is anaerobic (choice A), it is not this attribute which
causes its formation of abscesses.
Prevotella is extracellular, not an intracellular pathogen (choice B).
Although Prevotella does indeed have this type of endotoxin (choice C), the
absence of this molecule decreases the toxicity of the toxin, and does not
contribute to its proclivity toward abscess formation.
Mycobacteria, and not other genera such as Prevotella, are known for their
long-chain fatty acids (mycolic acids; choice D).

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