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Explanation - Q: 1.1
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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
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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
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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
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
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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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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
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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
Explanation - Q: 2.2
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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
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During the cholecystectomy, the surgeon ligates the cystic artery. This is typically
a branch of which of the following?
artery
Explanation - Q: 2.4
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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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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
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
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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
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The correct answer is D. A patient with severe abdominal pain and rectal
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.
Close
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
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
(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
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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
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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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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
Close
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
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abdominal wall.
The tunica albuginea (choice E) is a tough fibrous coat that covers the testis.
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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
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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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Explanation - Q: 5.4
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