Combined Q

Item: 1 of 46
QID: 2853
Mark
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118639 : DR. JITENDRA
A.46,XX; maternal
B.46,XX; paternal
C.46,XX; maternal and paternal
D.69,XXX; maternal and paternal
E.69,XXY; maternal and paternal
The correct answer is B. 75% chose this.
A hydatidiform mole is a noninvasive tumor caused by aberrant fertilization, leading to cystic swelling of chorionic villi and proliferation of the trophoblast. It results in a mass that can look like a "cluster of
grapes," as seen in the image. Hydatidiform moles can be complete or partial. The genotype of a complete mole is usually 46,XX, completely consisting of paternal DNA. It results when two sperm fertilize
A 23-year-old woman presents to the emergency department because of vaginal bleeding. She says that she is in her ninth week of pregnancy according to her last menstrual period. Laboratory studies
show a -human chorionic gonadotropin level of 103,000 mIU/L. The sample shown in the image is retrieved from the patient's uterus. There are no recognizable fetal parts.
Which of the following describes the most likely genotype and parental source of DNA in this mass?
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Item: 2 of 46
QID: 2350
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A.Administration of erythropoietin
B.Administration of folic acid
C.Administration of folinic acid
D.Administration of increased doses of the chemotherapy agent
E.Administration of vitamin B
12
The correct answer is C. 52% chose this.
This patient has mucositis, glossitis, and myelosuppression secondary to methotrexate treatment. Methotrexate is a folate analog that acts by inhibiting the enzyme dihydrofolate reductase, which is
responsible for converting folic acid to tetrahydrofolic acid. Tetrahydrofolic acid is responsible for the synthesis of several amino acids, purines, and thymidine, a required molecule for DNA synthesis. By
inhibiting dihydrofolate reductase, methotrexate induces a deficiency of tetrahydrofolic acid in the setting of normal folate levels. Methotrexate toxicity must therefore be treated by providing reduced folate
(folinic acid, also known as leucovorin) rather than folic acid, known as a "leucovorin rescue."
A is not correct. 11% chose this.
Erythropoietin is a hormone that stimulates RBC production in the bone marrow. Erythropoietin is used to treat anemia secondary to chronic kidney disease and myelodysplasia from chemoradiation
treatment. It cannot, however, correct anemia secondary to methotrexate because methotrexate causes deficiencies in DNA precursors so the body is unable to make new cells.
B is not correct. 25% chose this.
A 30-year-old woman who recently emigrated from Southeast Asia comes to her physician's office with uterine enlargement, a vaginal mass, and vaginal bleeding. Biopsy of the vaginal mass yields a
diagnosis of choriocarcinoma, and the patient is started on the usual therapeutic agent for this condition. After several weeks of therapy, the patient develops mild icterus; an enlarged, red, painful
tongue; and sores in the corners of the mouth. Her complete blood cell count returns:
WBCs: 2000/mm
Hemoglobin: 9.0 g/dL
Hematocrit: 26.4%
Platelets: 90,000/mm
Which of the following would most likely treat her abnormal cell count?
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Item: 3 of 46
QID: 4780
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A.-1,4-Glucosidase
B.-Galactosidase
C.-Glucocerebrosidase
D.Glucose-6-phosphatase
E.Glycogen phosphorylase
The correct answer is A. 51% chose this.
Pompe's disease is a type II glycogen storage disease that primarily affects the heart ("Pompe's trashes the pump"). Lysosomal -1,4-glucosidase is absent, which is necessary for the hydrolysis of the
outer branches of glycogen. As a result, glycogen is deposited in the myocardium, and by the sixth month of life children experience developmental delay, feeding problems, and eventual heart failure.
Skeletal muscle and the liver are also affected. ECG shows short PR intervals with large QRS complexes evidencing biventricular hypertrophy; cardiomegaly is also evident on x-ray of the chest.
Fabry's disease is lysosomal storage disease associated with -galactosidase A deficiency. Ceramide trihexoside accumulates, resulting in peripheral neuropathies of the hands and feet, angiokeratomas,
and cardiovascular and renal disease.
C is not correct. 13% chose this.
Gaucher's disease is a lysosomal storage disease associated with -glucocerebrosidase deficiency. Patients will have hepatosplenomegaly, aseptic necrosis of the femur, bone crises, and Gaucher's cells.
D is not correct. 11% chose this.
Von Gierke's disease is a type I glycogen storage disease associated with glucose-6-phosphate deficiency. Patients have severe fasting hypoglycemia with large stores of glycogen in the liver,
hepatomegaly, and increased blood lactate levels.
E is not correct. 13% chose this.
McArdle's disease is a type V glycogen storage disease associated with skeletal muscle glycogen phosphorylase. Glycogen in muscle cannot be broken down, leading to cramping and myoglobinuria with
A 5-month-old child has cardiomyopathy, an enlarged tongue, severe generalized muscular hypotonia, and hepatomegaly. Short PR intervals with giant QRS complexes are seen on ECG, and blood work
reveals elevated serum creatine kinase and decreased leukocyte acid maltase levels.
Which enzyme is deficient in this infant?
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Item: 4 of 46
QID: 2861
Mark
A.Aortic insufficiency
B.Large-joint arthritis
C.Opportunistic infections
D.Transient monocular blindness
E.Upper right quadrant abdominal tenderness
This patient's symptoms are explained best by secondary, or disseminated, stage syphilis. Syphilis is a sexually transmitted disease caused by the spirochete Treponema pallidum. It is characterized by
fever, sore throat, malaise, a generalized maculopapular rash involving the palms and soles, condylomata lata (wart-like papules on the genitals), and generalized nontender lymphadenopathy. A preceding
primary stage is characterized by a painless, indurated ulcer or chancre at the site of inoculation. If left untreated, syphilis can progress to a late stage that is characterized by end-organ damage to the
central nervous system, aorta, or musculoskeletal system. Long-term Treponema infection can destroy the vasa vasorum of the ascending aorta, leading to destruction and dilation of the vessel. The
distorted aorta consequently affects the patency of the aortic valve. Treatment with penicillin is essential.
Gonorrhea is a sexually transmitted disease caused by the gram-negative diplococcus Neisseria gonorrhoeae, which lacks both a capsule and the ability to ferment maltose (in contrast to N. meningitides).
N. gonorrhoeae infection predisposes to pelvic inflammatory disease, urethritis, pharyngitis, proctitis, ophthalmia neonatorum, and septic arthritis.
Opportunistic infection, especially by Pneumocystis jiroveci, Mycobacterium avium-intracellulare, John Cunningham (or JC) virus, toxoplasmosis, or Cryptococcus species, is indicative of immunosuppression
and/or AIDS. AIDS is the progression of HIV infection, caused by a single-stranded RNA retrovirus enveloped with reverse transcriptase. AIDS can lead to a variety of symptoms, but this patient's
presentation is best explained by syphilis infection.
A 22-year-old woman presents to the physician because of an erythematous rash on her hands and the soles of her feet. She is slightly febrile with swollen, nontender submandibular, axillary, and
inguinal lymph nodes. On questioning she reports having unprotected sex with five partners within the past year. Several months ago she noticed a single, painless, and firm lesion on her labia that
resolved without treatment.
If treatment is not begun, which of the following is this patient at increased risk of developing?
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Item: 5 of 46
QID: 1621
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A.Double-stranded RNA virus
B.Enveloped double-stranded DNA virus
C.Naked double-stranded DNA virus
D.Single-stranded DNA virus
E.Single-stranded RNA virus
The correct answer is E. 63% chose this.
The common cold is most often due to rhinoviruses (approximately 50% of cases), respiratory syncytial virus (10%), and coronaviruses (10%), which are spread by direct contact and respiratory droplets.
These viruses are single-stranded RNA viruses. Rhinoviruses are naked, whereas coronaviruses are enveloped.
Double-stranded RNA viruses include reoviruses, which can cause Colorado tick fever. Rotavirus, a type of reovirus, is the most common cause of fatal diarrhea in children.
Enveloped double-stranded DNA viruses include hepadnavirus, herpesviruses, and poxvirus.
Naked double-stranded DNA viruses include adenoviruses, which can cause febrile pharyngitis, pneumonia, and conjunctivitis.
Single-stranded DNA viruses include parvoviruses like the B19 virus, which can cause aplastic crises in sickle cell disease, erythema infectiosum, and hydrops fetalis.
A 30-year-old man presents to his primary care physician because of rhinorrhea, congestion, sore throat, and headache. Results of routine laboratory tests are within normal limits, and throat swab
staining and culture are negative for bacteria. The physician suspects a common cold.
Which of the following describes the most likely causative agent?
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Item: 6 of 46
QID: 3093
Mark
A.Krause end bulbs
B.Meissner's corpuscle
C.Merkel nerve endings
D.Pacinian corpuscle
E.Ruffini corpuscle
The correct answer is D. 56% chose this.
The most common initial sign of peripheral neuropathy in patients with diabetes is a loss of vibrational sensation. This finding is most clearly demonstrated on physical examination by placing a vibrating
tuning fork on the big toe. The sensory receptors responsible for transducing the sensation of vibration, pressure, and tension are the large, encapsulated pacinian corpuscles, which are located in the
deeper layers of the skin, ligaments, and joint capsules. They can be distinguished histologically by their onion-like appearance on cross-section.
It is important to tailor the physical examination in a diabetic patient to include testing for the major complications of diabetes: retinopathy, peripheral neuropathy, and vascular disease. Routine
funduscopic examination and ophthalmologic evaluation are necessary to monitor for the development of diabetic retinopathy. A thorough neurologic examination of the distal extremities including
monofilament testing and vibration sense testing are key to evaluate for peripheral neuropathy. Moreover, routine examination of the feet is necessary for both patient and physician as neuropathy puts
patients at risk for developing ulcers and infections. Finally, listening for carotid bruits can play a critical role in assessing the status of the vasculature as patients are at increased risk for coronary artery
disease and stroke.
Krause end bulbs are sensory receptors found in the oropharynx and conjunctiva of the eye.
Meissner's corpuscles, which are responsible for conveying the sensation of light touch, are small encapsulated sensory receptors found just beneath the dermis of hairless skin, most prominently in the
fingertips, soles of the feet, and lips. Meissner's corpuscles are involved in the reception of light discriminatory touch, not vibratory sensation, as is being tested in this case.
A 45-year-old man with type 2 diabetes mellitus presents to his physician for regular follow-up. The patient denies any changes in vision, chest pain, difficulty with gait, or injuries to the feet. There are
no carotid bruits on examination, and funduscopic examination is unremarkable. On neurologic examination, the patient has an abnormal tuning fork examination, with absence of vibratory sensation in
the feet bilaterally.
Which of the following receptors is most likely affected in this patient?
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Item: 7 of 46
QID: 10072
Mark
A
B
C
D
E
The following vignette applies to the next 2 items. The items in the set must be answered in sequential order. Once you click Proceed to Next Item, you will not be able to add or change an answer.
A 36-year-old woman with no significant past medical history is evaluated for irregular menstrual cycles starting 1 year ago and amenorrhea starting 4 months ago. She denies using any medications,
and there has been no new stress at work or at home. Initial evaluation shows a negative -hCG test and normal prolactin and thyroid-stimulating hormone levels. She reports symptoms of hot flashes,
irritability, dyspareunia, and decreased libido. Physical examination reveals a normal body mass index.
Which of the following is most consistent with this patient's condition?
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Item: 8 of 46
QID: 10073
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A.Aldosterone
B.Atrial natriuretic peptide
C.Glucagon
D.Insulin
E.Oxytocin
FSH binds to a cell membrane receptor with cAMP as the secondary messenger. Other hormones that use cAMP as a secondary messenger include glucagon, LH, parathyroid hormone,
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2
-adrenergic
receptors, -hCG, antidiuretic hormone type 2, ACTH, TSH, melanocyte-stimulating hormone, and calcitonin.
Aldosterone exerts its effect through an intracellular receptor. Other steroidal hormones include glucocorticoids, estrogen, testosterone, progesterone, vitamin D, and thyroid hormone.
Atrial natriuretic peptide binds a cell membrane receptor with cGMP as the secondary messenger. Nitric oxide is another substance that also works through intracellular cGMP.
Insulin binds and activates a dimeric tyrosine kinase receptor. Other hormones that also bind to tyrosine kinase receptors are insulin-like growth factor-1 and prolactin.
Oxytocin increases the intracellular level of inositol triphosphate (IP
3
). Other hormones that mediate their physiologic actions through IP
3
are ADH, angiotensin II, growth hormone-releasing hormone,
thyrotropin-releasing hormone, and GnRH.
The patient is subsequently found to have an elevated FSH level.
The receptor for FSH has a similar mechanism of action as the receptor to which of the following hormones?
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Item: 9 of 46
QID: 10074
Mark
A.Adipocytes
B.Adrenal gland
C.Granulosa cells
D.Hepatocytes
E.Theca cells
There are three types of estrogen: estradiol, estriol, and estrone. Estrone is a relatively weak estrogen that is synthesized in peripheral adipocytes from the aromatization of the precursor androstenedione.
Androstenedione comes from the adrenal cortex. This estrogen is important during menopause, as estradiol is no longer synthesized in the ovaries.
Adipocytes aromatize androstenedione to estrone; however, they do not synthesize the precursor molecules.
Granulosa cells are sites of estradiol synthesis from the aromatization of testosterone.
The liver is the site of estrogen metabolism in adults. During pregnancy, the maternal liver also plays an important role in the synthesis of estriol.
Theca cells synthesize testosterone, which is the precursor for estradiol synthesis. Another source of testosterone is the adrenal cortex.
Upon further evaluation, this patient with premature ovarian failure is found to have a relatively high level of a particular type of estrogen.
Where is the source of the precursor of this type of estrogen?
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Item: 10 of 46
QID: 3631
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A
B
C
D
The surgical procedure referred to (a nerve transection to decrease gastric acid secretion) is a vagotomy, or cutting of the vagus nerve. Within the vagus nerve are parasympathetic nerve fibers.
Preganglionic parasympathetic fibers are long, synapsing on target organs, while preganglionic sympathetic fibers are shorter, synapsing on the sympathetic chain. Point C is a correct representation of a
vagotomy, or transection of a preganglionic parasympathetic nerve. While postganglionic fibers (point D) could in theory be cut, this is highly unlikely to happen in practice.
A 54-year-old man with a history of peptic ulcer disease refractory to medical management with proton pump inhibitors undergoes a surgical procedure involving autonomic nerve transection designed to
decrease gastric acid secretion. In this drawing of the autonomic nervous system, each solid bar labeled A-D represents a possible point of nerve transection.
Along which point would the surgeon perform the nerve transection?
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Item: 11 of 46
QID: 2002
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A.Abnormal bleeding
B.Ataxia
C.Megaloblastic anemia
D.Rickets
E.Seizures
Methotrexate is an anti-metabolite that acts as an analog of folic acid. It inhibits dihydrofolate reductase, thus preventing the regeneration of tetrahydrofolate, the metabolically active form of folic acid. This
creates a functional deficiency of folic acid, leading to decreased DNA, RNA, and protein synthesis, ultimately preventing tumor cell proliferation. This defect in DNA synthesis, however, can also result in a
megaloblastic anemia. Leucovorin (folinic acid) replaces folic acid metabolites and is often given simultaneously to reduce the after effects of methotrexate.
Vitamin K is important in the synthesis of coagulation factors II, VII, IX, and X. Vitamin K deficiency is seen in newborns and with the use of antibiotics that inhibit intestinal bacterial production.
Methotrexate acts on the folic acid pathway and does not cause a deficiency in vitamin K.
Cobalamin (vitamin B
12
) deficiency results in a megaloblastic anemia that is accompanied by subacute combined degeneration (degeneration of the dorsal and lateral spinal columns causing paresthesias
and ataxia). Methotrexate acts on the folic acid pathway only and does not cause subacute combined degeneration.
1,25-Dihydroxycholecalciferol is the active form of vitamin D. Vitamin D deficiency is caused by reduced dietary intake or insufficient exposure to sunlight, and results in osteomalacia in adults and rickets in
children. Methotrexate acts on the folic acid pathway and does not cause a deficiency in vitamin D or rickets.
A 17-year-old African-American boy has swelling and intense pain in his left leg that worsens with activity. Initial imaging studies are suspicious for neoplasm, and a subsequent biopsy is conclusive for
osteosarcoma of the left femur. He begins an adjuvant chemotherapy regimen that includes methotrexate.
Which of the following after effects is most closely associated with administration of this drug?
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Item: 12 of 46
QID: 3643
Mark
A.Aplastic anemia
B.Hemolytic anemia
C.Megaloblastic anemia
D.Microcytic anemia
E.Sideroblastic anemia
The patient has a history of dark colored stools, suggestive of gastrointestinal blood loss from a documented carcinoma of the colon. Chronic blood loss is a major cause of iron deficiency anemia, most
often caused by menorrhagia, or as in this case bleeding gastrointestinal lesions. Iron deficiency anemia is a hypochromic microcytic anemia.
Aplastic anemia is pancytopenia with bone marrow hypocellularity. This patient's normal WBC and platelet counts are not consistent with aplastic anemia. A patient with documented colon cancer and a
history of gastrointestinal blood loss most likely has iron deficiency anemia.
Hemolytic anemias can be classified as intracorpuscular (or abnormalities of the RBC itself) and extracorpuscular (or extrinsic factors producing RBC hemolysis). Intracorpuscular hemolytic anemias include
A 76-year-old man presents to the emergency department with a 3-day history of constipation. He reports fatigue and changes in bowel habits over the period of a year, including dark stools. On
physical examination the patient's abdomen is remarkably distended. Barium enema reveals an apple-core lesion of the rectosigmoid junction, and biopsy demonstrates adenocarcinoma. Laboratory
studies show:
WBC count: 10,200/mm
Hematocrit: 26.5%
Platelet count: 231,000/mm
The patient's low hematocrit likely reveals what type of anemia?
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Item: 13 of 46
QID: 3380
Mark
A.Ataxia
B.Blindness
C.Expressive aphasia
D.Medial rectus palsy
E.Unilateral leg paralysis
The middle cerebral artery arises from the internal carotid artery and the circle of Willis and supplies the lateral aspect of the brain. If the right hemisphere is compromised, you would expect to see
left-sided weakness and sensory loss, gaze deviation to the right, and neglect of the left visual space. If the left hemisphere is compromised, the findings are similar but on the opposite side. Furthermore,
on the left the language centers are at risk. Because the middle cerebral artery supplies both Broca's and Wernicke's speech areas, an injury to the middle cerebral artery can produce either expressive
aphasia (if Broca's area is injured) or sensory aphasia (if Wernicke's area is injured). Additional impairments may include difficulty with reading, writing, and calculation.
Ataxia would result from a cerebellar injury, which could be produced by blockage of the cerebellar arteries or the posterior arteries in the circle of Willis, but not typically by injury to the middle cerebral
artery.
Blindness could be the result of injury to the ophthalmic artery or anterior communicating artery, both of which would damage the optic nerve. Alternatively, blindness could result from damage to parts of
the visual cortex supplied by the posterior cerebral artery.
Medial rectus palsy would most likely be produced by an injury to the posterior communicating artery, leading to damage in the oculomotor nucleus and/or medial longitudinal fasciculus.
A 67-year-old man has a history of high cholesterol and hypertension and has smoked cigarettes for the past 50 years. While undergoing a stressful divorce, the man suffers a massive stroke and
collapses.
An injury to the middle cerebral artery could be expected to produce which of the following symptoms?
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Item: 14 of 46
QID: 4696
Mark
A.Ammonium magnesium phosphate stone(s)
B.Calcium oxalate stone(s)
C.Calcium phosphate stone(s)
D.Cystine stone(s)
E.Uric acid stone(s)
This patient has symptoms and signs consistent with nephrolithiasis. Her urinalysis results are consistent with a concomitant urinary tract infection with an organism that is urease positive (eg, Proteus or
Klebsiella); the resultant increase in urinary pH provides a suitable environment for the formation of ammonium magnesium phosphate (also known as struvite) stones. Struvite stones are radiopaque and
often located in the renal calyces; they may grow into large stones, sometimes extending throughout the entire collecting system (known as a staghorn calculi). Treatment includes hydration, pain control,
and appropriate antibacterial therapy and prophylaxis, but larger stones frequently require either surgical removal or lithotripsy (using shock waves to break up the stones). Chemical dissolution with a
urease-inhibiting drug is frequently ineffective.
Calcium oxalate stones are the most common type of kidney stone and result from hypercalciuria. These stones are radiopaque. Therapy consists of hydration, pain control, and treatment of the underlying
cause of hypercalciuria.
Calcium phosphate stones result from hypercalcuria. These stones are radiopaque. Therapy consists of hydration, pain control, and treatment of the underlying cause of hypercalciuria.
Cystine stones result from inappropriate secretion of cystine into the urine due to an autosomal recessive defect in amino acid transport. Cystine stones are radiopaque. Treatment consists of hydration,
pain control, alkalinization of the urine, and therapy with a cystine-binding medication.
A 22-year-old woman presents to the clinic with symptoms of left-sided back pain and blood visible at the end of the urine stream. Urinalysis shows a specific gravity of 1.010 and a pH of 7.9; results are
positive for nitrite and WBC esterase, but negative for ketones and glucose. Microscopic examination of urine shows many RBCs and many bacteria.
Which of the following is the most likely underlying cause of the patient's symptoms?
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Item: 15 of 46
QID: 5043
Mark
A.Late-look bias
B.Participation bias
C.Recall bias
D.Sampling bias
E.Selection bias
Sampling bias occurs if factors unrelated to the aim of the study distinguish the subjects from the rest of the population so that the results of the study are not generalizable. In this study, those college
students who volunteer for a study on alcohol use on campus may be a different population from the rest of the student body. Mechanisms used in research study design to limit bias include blind studies,
placebo responses, cross-over studies, and randomization.
Late-look bias occurs when information is gathered at an inappropriate time in a way that distorts the conclusions. Late-look bias is present in situations in which investigators characterize a population who
has had a disease for many years, and in doing so, only sample those patients with the milder, indolent form of the illness because those with the severe form have already died from it.
Participation bias occurs when certain factors affect the final enrollment of the intended sample population. For example, not all subjects may agree to participate, or certain imaging studies or medical
records may not be available for review.
Recall bias occurs when knowledge of the presence of a disorder alters recall by subjects.
A researcher designs a study that assesses the extent of alcohol abuse among college students. Advertisements of the study are placed on campus, and 58 students (38 men, 20 women) volunteer for
the anonymous study. The students fill out an anonymous questionnaire about drinking habits that asks about such information as amount and frequency of alcohol abuse, grades, and majors. These
results are combined to yield some general statistics about alcohol abuse on a college campus.
Which type of bias is present in this study design?
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Item: 16 of 46
QID: 4434
Mark
A.Acral lentiginous
B.Actinic keratosis
C.Basal cell carcinoma
D.Fibroadenoma
E.Squamous cell carcinoma
Squamous cell carcinoma commonly appears on the face, especially around the lips. On biopsy keratin pearls are often evident. This type of cancer is locally invasive but rarely metastatic. Industrial
carcinogens such as tars and oils, old burn scars, arsenic, and exposure to ionizing radiation, in addition to sunlight, are risk factors for squamous cell carcinoma.
Acral lentiginous cancer is a type of melanoma that often appears on the palms and soles of dark-skinned individuals.
Actinic keratoses are hyperkeratotic areas with surrounding erythema that develop in sun-exposed areas. These are premalignant changes that often precede the development of squamous cell carcinoma.
Basal cell carcinoma, although also often appearing on the face and other sun-exposed areas, is often described as "pearly papules with overlying telangiectasias" and displays palisading nuclei on biopsy.
Fibroadenomas are benign, well-defined, mobile tumors of the breast that contain both glandular and fibrous tissue. These tumors, often found in young women, are believed to be related to hormonal
variations and do not increase the risk for subsequent breast cancer.
A fair-skinned 56-year-old farmer presents to the clinic with a scaling, indurated, ulcerated nodule just below his lower lip. Biopsy reveals sheets of neoplastic epidermal cells and keratin pearls.
Which of the following is the most likely diagnosis?
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Item: 17 of 46
QID: 2169
Mark
A.Acetylcholine receptors
B.Neutrophils
C.Oligodendrocytes
D.RBCs
E.Thyroid-stimulating hormone receptors
This patient has Churg-Strauss syndrome (also known as allergic granulomatosis and angiitis), which is one of a trio of diseases (Wegener's granulomatosis and microscopic polyangiitis being the others)
that are commonly referred to as the ANCA (antineutrophil cytoplasmic antibody)-associated vasculitides (ie, diseases causing inflammation of blood or lymphatic vessels). Fifty to seventy percent of
patients with Churg-Strauss syndrome have elevated levels of ANCA, usually the perinuclear pattern of staining type (p-ANCA) anti-myeloperoxide (MPO). Pulmonary vasculature involvement is common
and patients often have preexisting asthma and allergic rhinitis. They also present with markedly elevated eosinophil counts and mononeuritis multiplex (simultaneous deficits of two or several peripheral
nerves in different areas of the body). Other symptoms include uveitis, conductive hearing loss, and muscle/joint pain. An eosinophilic gastroenteritis may precede the onset of the other symptoms.
Autoantibodies to acetylcholine receptors are not particularly associated with Churg-Strauss syndrome. Myasthenia gravis is characterized by an autoimmune attack on the acetylcholine receptors of the
neuromuscular junction between motor neurons and skeletal muscle fibers.
Autoantibodies to oligodendrocytes are not particularly associated with Churg-Strauss syndrome. There is evidence suggesting that multiple sclerosis may be partially caused by autoimmune antibody
attack on central nervous system myelin-secreting oligodendrocytes.
Autoantibodies to RBCs, which may be found in certain cases of immune hemolytic anemia, are not particularly associated with Churg-Strauss syndrome.
A 53-year-old man with a long-standing history of allergic rhinitis and asthma presents with uveitis, mild hearing loss, numbness and tingling in his right hand, and diffuse joint pain for the past 10 days.
Physical examination shows weak to absent left knee patellar reflexes (right knee reflex strong and intact). Laboratory studies show a markedly elevated eosinophil count. A diagnosis is made, and the
patient is treated with cyclophosphamide. Further laboratory studies show elevated serum levels of the most common autoantibody associated with this condition.
What structure is primarily targeted by the autoantibodies that are most likely elevated in this patient's serum?
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QID: 1246
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A.Glucose
B.Glycogen
C.Oxaloacetate
D.Pyruvate
E.Urea
This patient has Pompe's disease, a glycogen storage disorder. Pompe's disease is an autosomal recessive disease that is characterized by a deficiency or defect in lysosomal -1,4-glucosidase. This enzyme
is necessary for the dissolution of the polymer linkages in glycogen. In its absence, glycogen accumulates to toxic levels in both the cytoplasm and lysosomes. This disease is commonly associated with
myopathies, particularly heart muscle thickening and subsequent failure from glycogen build-up in the cardiac muscle.
Glucose is stored as glycogen in the cells and is also present in blood. However, hyperglycemia is not responsible for the symptoms observed in this patient.
Oxaloacetate is the first intermediate in the Krebs cycle. It is regenerated with each turn of the cycle but is not present in excessive amounts in the cell.
Pyruvate is a component of the cellular respiration pathway and an intermediate in gluconeogenesis. It is not stored in cells in any significant quantity.
Disorders of the urea cycle lead to nitrogen accumulation in the body and result in progressive lethargy and coma. They do not cause the myopathy seen in this patient.
An 8-month-old boy is brought to the pediatrician by his parents because he has recently lost the ability to crawl or hold his toys. On examination the patient is tachypneic and breathing with
considerable effort; the liver is palpable five finger widths below the right costal margin. X-ray of the chest reveals cardiomegaly. He has a difficult time sitting upright and cannot squeeze the physician's
fingers or the ring of his pacifier with any noticeable force. Despite a number of interventions, the child's symptoms continue to worsen until his death 2 weeks later.
On autopsy, it is likely that this patient's cells will contain an accumulation of which of the following substances?
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QID: 3017
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A.Adhesions along the hepatic flexure of the large colon
B.Engorgement of the splenic vessels
C.Ischemic necrosis of the pericentral vein of the hepatocytes
D.Mass at the head of the pancreas
E.Obstruction of the biliary duct
F. Papillary sloughing of the right kidney
This presentation is most consistent with acute cholecystitis, which usually presents in patients with the "4 Fs": Fat, Female, Fertile, and Forty years old. These patients usually have right upper quadrant
pain, low-grade fevers, and elevations in bilirubin and transaminase levels. This disease occurs when the gallbladder becomes inflamed, most commonly following obstruction of the cystic duct by a
gallstone.
This would not be seen on ultrasound and does not correlate with the patient's clinical picture. Adhesions do not present with fever, hyperbilirubinemia, or elevations in liver function tests.
The spleen is located in the left upper quadrant of the abdomen, not the right, so it would not be seen on that patient's right upper quadrant ultrasound.
Right upper quadrant ultrasound is not likely to show ischemic necrosis of the pericentral vein of the hepatocytes, since this is a histopathologic diagnosis.
Although a mass at the head of the pancreas could be seen on right upper quadrant ultrasound, it does not fit the clinical picture of this patient, because an obstructive pancreatic head mass is more likely
An overweight 40-year-old woman presents to her physician because of severe right upper quadrant abdominal pain. She has a low-grade fever. Laboratory tests show a serum bilirubin level of 5 mg/dL,
a serum aspartate aminotransferase level of 80 U/L, and a serum alanine aminotransferase level of 90 U/L. Ultrasonography of the right upper quadrant is performed.
What is the ultrasound image likely to show?
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Item: 20 of 46
QID: 3014
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A.-Aminobutyric acid
B.Acetylcholine
C.Glycine
D.Norepinephrine
E.Serotonin
The "rest and digest" response is stimulated by the parasympathetic nervous system (PSNS), a subsystem of the autonomic nervous system. The PSNS stimulates salivary gland secretion (salivation),
accelerated peristalsis (stomach grumbling), and dilation of blood vessels leading to the gastrointestinal tract. The major neurotransmitter of the PSNS is acetylcholine.
-Aminobutyric acid is a major inhibitory neurotransmitter commonly found in the brain. It does not cause hypersecretion from these glands.
Glycine is an inhibitory neurotransmitter released by spinal interneurons. It does not cause hypersecretion from these glands.
Norepinephrine is the primary neurotransmitter for postganglionic sympathetic neurons. Although the salivary gland is stimulated by both branches of the nervous system, the sympathetic branch does not
cause secretion from the salivary gland.
Serotonin is a neurotransmitter in the nuclei in the brainstem. It does not cause hypersecretion from these glands.
A 15-year-old boy smells what his mother is cooking in the kitchen and asks when dinner will be ready. Impatient, he complains that his stomach is grumbling and that he is salivating in anticipation of
the meal.
Which neurotransmitter is responsible for the boy's response to seeing and smelling dinner?
Bottom Line:
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Item: 21 of 46
QID: 4411
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A.Atrial septal defect
B.Mitral regurgitation
C.Patent ductus arteriosus
D.Patent foramen ovale
E.Tricuspid regurgitation
F. Ventricular septal defect
The correct answer is F. 44% chose this.
Ventricular septal defect (VSD) is the most common congenital cardiac anomaly. On physical examination it is often heard as a II-III/VI holosystolic murmur, best heard at the left sternal border. Its
harshness can be a rough indicator of its size . VSD is caused by defects in the septum of the atrioventricular canal, in the muscular septum, or in the distal conal septum in the developing fetal heart. The
anomaly can be seen alone or can be part of a syndrome, such as tetralogy of Fallot.
Atrial septal defect is characterized by a loud S
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and a wide, fixed split S
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Mitral regurgitation produces a holosystolic murmur that is best heard at the apex of the heart. It is commonly caused by rheumatic heart disease, papillary muscle dysfunction, or chordae rupture
secondary to a myocardial infarction.
Patent ductus arteriosus is not the most common congenital cardiac anomaly. It is characterized by a continuous "machine-like" murmur at the left upper sternal border.
A 4-year-old boy comes to his new pediatrician's office for a check-up. The boy looks generally well and is active. On cardiac auscultation, however, the pediatrician notes a low-pitched II/VI holosystolic
murmur, best heard at the left sternal border. Although the boy has no symptoms of a cardiac condition, the pediatrician orders an echocardiogram.
What is the most likely diagnosis?
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Item: 22 of 46
QID: 1193
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A.ATCACTA
B.GTCACCA
C.GTCACTT
D.GTCGCTA
E.GTTACTA
Transversion is a mutation in which a purine (ie, adenine or guanine) is substituted for a pyrimidine (ie, cytosine, thymine, or uracil) or a pyrimidine is substituted for a purine. In this case, adenosine, a
purine, was changed to thymine, a pyrimidine.
In this mutation guanine was substituted for adenosine. Both are purines; therefore, this is an example of transition.
In this mutation thymine was substituted for cytosine. Both are pyrimidines; therefore, this is an example of transition.
In this mutation adenosine was substituted for guanine. Both are purines; therefore, this is an example of transition.
In this mutation cytosine was substituted for thymine. Both are pyrimidines; therefore, this is an example of transition.
Which of the following mutations in the sequence GTCACTA represents a transversion mutation?
Bottom Line:
Transversion mutations occur when a purine (adenine or guanine) base is substituted for a pyrimidine base (cytosine, thymine, or uracil). Transition mutations occur when a purine is switched for a purine
or a pyrimidine is switched for a pyrimidine.
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Item: 23 of 46
QID: 3124
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A.Erectile dysfunction
B.Penile cancer
C.Priapism
D.Urinary incontinence
E.Urinary retention
A leak into the dorsal and saphenous veins, which demonstrates a venous outflow abnormality, is typically due to insufficient relaxation of the smooth muscle resulting from excessive adrenergic tone or
damaged parasympathetic innervation. Venous outflow abnormalities are common causes of erectile dysfunction in older men. In a normal erection, blood flow accelerates, increasing the pressure within
the intracavernosal spaces and blocking penile venous outflow. Abnormalities in venous outflow, such as the leak from the dorsal vein to the saphenous vein found in this patient, can result in failure to
acquire or maintain a firm erection.
Penile cancer is rare in developed countries. It is generally a squamous cell carcinoma and is diagnosed by biopsy. Abnormalities in venous blood flow are not normally found in cancer.
Priapism is a condition in which a penile erection is maintained for >4 hours. It is a medical emergency with many causes, including sickle cell disease, spinal cord lesions, and pharmacological adverse
effects. A leak from the dorsal vein to the saphenous vein would result in failure to acquire or maintain an erection, not failure to return to the flaccid state.
Urinary incontinence is a common problem, especially in older persons. Causes include detrusor overactivity and neurologic impairment. Abnormal penile blood flow is not normally implicated in urinary
incontinence.
A 57-year-old man undergoes cavernosography, a radiographic examination that shows the corpora cavernosa and draining veins after injection of contrast into the corpora. The test demonstrates a leak
from the dorsal vein to the saphenous vein.
Which of the following was the most likely indication for this patient's cavernosography?
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Item: 24 of 46
QID: 2365
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A.Angiotensin-converting enzyme inhibitor
B.Carbonic anhydrase inhibitor at the proximal convoluted tubule
C.Inhibition of the Na-K-2Cl symporter at the thick ascending limb
D.Sodium channel inhibitor at distal convoluted tubule
E.Spironolactone
Spironolactone is a potassium-sparing diuretic often used in combination with thiazide diuretics to prevent hypokalemia. Common adverse effects, including gynecomastia, testicular atrophy, and decreased
libido, occur because spironolactone is a weak androgen receptor antagonist in addition to being an aldosterone receptor antagonist. Note that the other potassium-sparing diuretics, amiloride and
triamterene, do not cause these adverse effects.
Angiotensin-converting enzyme inhibitors, commonly called ACE inhibitors, act to block the conversion of angiotensin I to angiotensin II. ACE inhibitors are used in patients with hypertension and congestive
heart failure primarily for their ability to vasodilate arterioles and reduce afterload. The most common adverse effects include dry cough and rash.
Acetazolamide is a carbonic anhydrase inhibitor that acts at the proximal convoluted tubule. It leads to diuresis of bicarbonate. Common adverse effects include metabolic acidosis, blurred vision, and
calcium oxalate or calcium phosphate kidney stones.
Furosemide is a loop diuretic that can cause hyponatremia. It acts on the thick ascending limb to inhibit the Na-K-2Cl symporter. It is commonly used in heart failure patients to maintain an appropriate
volume status. Other adverse effects of furosemide include hypokalemia, hypomagnesemia, and contraction metabolic alkalosis.
A 55-year-old man with a 10-year history of well-controlled hypertension and mild congestive heart failure presents to the physician's office complaining of decreased sex drive and enlargement of his
breasts.
Which of the following is the most likely cause of this patient's symptoms?
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Item: 25 of 46
QID: 10110
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A.Anti-AchR
B.Anti-double-stranded DNA antibodies
C.Anti-Scl70
D.Anti-SS-B
E.Antigliadin
This patient's constellation of symptoms points to systemic lupus erythematosus (SLE), an autoimmune condition resulting in tissue damage secondary to autoantibody production. Clinical symptoms
include hemolytic anemia, leukopenia, arthralgias without joint deformity, skin rashes (especially in a malar distribution or on sun-exposed areas), renal disease, Libman-Sacks endocarditis, and neurologic
symptoms. SLE typically affects women of between 20 and 45 years of age. Antinuclear antibodies, which are seen in about 95% of patients, are the most common diagnostic test for SLE. Although highly
sensitive for the condition, they are not specific and can be seen in multiple autoimmune conditions. To confirm the condition, physicians typically test patients for anti-double stranded DNA (anti-dsDNA)
autoantibodies. Anti-dsDNA is the most specific diagnostic test, and is positive in 40%-60% of SLE patients, but rarely if ever is present in other autoimmune conditions.
Anti-AChR antibodies are seen in myasthenia gravis, an autoimmune condition in which patients experience fluctuating muscle weakness, which is typically worst near the end of the day or after exercise.
Most patients present with ptosis or diploplia, and about 15% of patients experience bulbar symptoms including dysarthria, dysphagia, and difficulty chewing. Only a small minority of patients present with
only distal limb weakness.
A 24-year-old woman presents to her primary care physician complaining of pain in her neck and hands. She works as a music teacher, and notes that playing the piano has become increasingly
uncomfortable for her. She also has difficulty turning her head to see her class as a result of "swelling along her jaw line." Review of systems is notable for weakness and malaise accompanied by a
5.4-kg (12-lb) weight loss in the past several months. The patient initially attributed her symptoms to an unusually cold winter that year, but she states that they did not improve when she went on
vacation in Mexico. In fact, she states that on the first day, she developed a red rash on her face and hands despite the use of a large amount of sunscreen. There is no evidence of joint deformity, but pain is
elicited by both active and passive movements of the digits. Blood tests reveal moderate anemia and mild leukopenia.
Which of the following, if present in high titer, would be the most specific test to confirm the most likely diagnosis?
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QID: 10111
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A.Chronic renal failure
B.Diffuse membranous glomerulopathy
C.Diffuse proliferative glomerulonephritis
D.Minimal change disease
E.Rapidly progressive glomerulonephritis
Kidney disease is seen in almost 90% of lupus patients. This patient's clinical presentation of hypertension, periorbital puffiness, and hematuria is consistent with nephritic syndrome. Proteinuria is also
present in patients with nephritic syndrome, but it does not exceed 3.5 g/d. This patient is most likely suffering from diffuse proliferative glomerulonephritis (DPGN), the most common subtype of kidney
disease in SLE. SLE-induced kidney disease results from a type III hypersensitivity reaction to high levels of circulating immune complexes. Circulating immune complexes can either deposit in the
glomeruli, or develop in situ. Immune complexes accumulate in the subendothelium and activate complement, which serves as a chemoattractant for neutrophils. Recruited neutrophils cause glomerular
tissue damage, creating the characteristic wire looping appearance seen on electron microscopy.
Kidney failure can result from DPGN; however, diabetes mellitus, hypertension, and RPGN are far more common causes. Clinical symptoms of renal failure include, but are not limited to, progressive
azotemia, osteomalacia and osteoporosis secondary to hypocalcemia, hypertension, congestive heart failure, hemorrhagic gastritis, hyperkalemia, hyperphosphatemia, and waxy casts in the urine.
Laboratory values for patients with kidney failure include elevated glomerular filtration rate.
Diffuse membranous glomerulopathy (DMG) is the second most common subtype of nephrotic syndrome seen in adults. The hallmark of nephrotic syndrome is massive proteinuria of >3.5 g/d, pitting
edema, and ascites secondary to hypoalbuminemia, hypertension, hypogammaglobulinemia, and fatty casts with maltese crosses in the urine. DMG can be primary, or can be secondary to drugs such as
The patient begins taking nonsteroidal anti-inflammatory drugs to control her rash and muscle complaints, which initially kept her symptoms well controlled. Several years later, she returns to her
physician complaining of progressive periorbital puffiness, swelling around the ankles, and gross hematuria. Her blood pressure is 140/90 mm Hg. Laboratory tests reveal a glomerular filtration rate
within the normal range. Urinalysis is significant for RBC casts; proteinuria is present at <2.5 g/d.
What is the patient's most likely diagnosis given her history and present symptoms?
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Item: 27 of 46
QID: 10112
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A.Cyclophosphamide
B.Hydroxychloroquine
C.Intravenous immune globulin
D.Kidney transplant
E.Nonsteroidal anti-inflammatory drugs
Cyclophosphamide is an immunosuppressive agent typically reserved for SLE patients with significant organ involvement, such as severe lupus-induced nephritis. This agent is typically given in combination
with glucocorticoids to reduce the titer of autoantibodies in SLE. Glucocorticoids as single therapy is used primarily to treat mild symptoms such as rash, arthralgias, and serositis when nonsteroidal
anti-inflammatory drugs (NSAIDs) have failed. They are often used in combination with stronger immunosuppressive agents such as cyclophosphamide when significant organ involvement (especially of the
kidneys) is seen.
Hydroxychloroquine and other antimalarials are most effective to manage the skin manifestations and musculoskeletal symptoms of SLE. Although antimalarials have been shown to be effective in
preventing major kidney and central nervous system damage caused by SLE, once kidney disease has begun, they are not the drugs of choice to treat the disease or prevent its further progression.
Intravenous immune globulin is pooled immunoglobulins from thousands of donors. It is most commonly used in the treatment of immunodeficiency disorders or acute inflammatory or autoimmune
conditions such as idiopathic thrombocytopenic purpura. While one of its off-label uses is in SLE, it is not the most appropriate therapy given that the clinical data are insufficient to support its widespread
use in SLE.
Kidney transplantation is not indicated as a first-line treatment. The patient has DPGN, but it has not progressed toward end-stage or near-end-stage renal disease given that her glomerular filtration rate is
Over the course of the next several months, the patient's kidney function declines significantly. She discusses her options with her physician.
Which of the following is the most appropriate first-line intervention to slow the progression of her disease?
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Item: 28 of 46
QID: 10101
Mark
A.Chlorpromazine
B.Haloperidol
C.Olanzapine
D.Thioridazine
E.Ziprasidone
Given the patient's complicated history of psychiatric illness and multiple admissions in the past, the patient described above likely has schizophrenia. With her presentation of delusions and extreme
agitation, haloperidol was likely given to treat the agitation and the psychotic symptoms. Haloperidol is a highly potent typical antipsychotic that works primarily by blocking dopamine D
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receptors. One of
the acute adverse effects of highly potent antipsychotics is acute dystonia, which can present as torticollis, involuntary contractions of the neck leading to abnormal posturing, as described in this patient.
Other manifestations of acute dystonia include jaw spasms and buccolingual spasms. Men and younger patients demonstrate a greater susceptibility to this complication. In general, typical antipsychotics
have a higher risk for developing extrapyramidal symptoms (EPS) than atypical antipsychotics. Typical antipsychotics include haloperidol, thioridazine, chlorpromazine, and fluphenazine. Of the typical
antipsychotics, the risk of EPS is greatest when using potent dopamine antagonists with few anticholinergic effects, thus greatly disrupting the dopaminergic and cholinergic balance in the striatum. Among
the choices given above, haloperidol is the most potent typical antipsychotic with the least anticholinergic effect, thus making it the most likely agent to cause the acute dystonia seen in this patient.
Chlorpromazine is a typical antipsychotic. However, it is not as potent as haloperidol and only carries an intermediate risk for developing extrapyramidal symptoms with its use.
Olanzapine is an atypical antipsychotic, and it carries a low risk of causing EPS.
A 28-year-old woman with a long history of psychiatric illness resulting in multiple admissions in the past is brought in by her husband. On examination the patient is extremely combative and agitated.
She refused blood draws because she believes her alien blood is toxic to medical personnel. A drug is given and within several hours the patient develops jaw spasms and an abnormal posturing of her
neck due to increased tension in her neck muscles.
What drug was she most likely given?
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Item: 29 of 46
QID: 10102
Mark
A.Blocks acetylcholine receptors
B.Blocks dopamine receptors
C.Blocks histamine receptors
D.Blocks voltage-sensitive calcium channels
E.Inhibits acetylcholinesterase
Anticholinergic agents/acetylcholine receptor blockers are used to treat neuroleptic-induced acute dystonia. Benztropine is typically given to relieve the dystonic symptoms. While the exact pathophysiology
of acute dystonia is still unclear, anticholinergics have been used to treat EPS of patients on neuroleptics. EPS result from the pharmacologic blockade of dopamine receptors in the striatum, thus mimicking
the degeneration of neurons in the substantia nigra seen in parkinsonism. By inhibiting dopamine signaling, the balance between the dopaminergic and cholinergic transmission in the striatum is disrupted
and shifts towards the cholinergic side. By administering anticholinergics, the balance in the striatum is restored and the patient's EPS are relieved.
Antipsychotics are dopamine receptor blockers. Giving the patient described above another dopamine receptor blocker would worsen her symptoms. While switching to another antipsychotic that is less
likely to cause EPS in this patient should be the next course of action, administering a dopamine receptor blocker in the acute setting of this patient's presentation would not relieve or reverse her
symptoms.
While diphenhydramine, a first-generation histamine (H
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patient's symptoms. First-generation H
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blockers such as diphenhydramine and chlorpheniramine also have considerable anticholinergic effects, and it is this mechanism of action that relieves the EPS.
The patient then receives a medication to relieve the tension in her neck.
What is the mechanism of action of this medication?
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Item: 30 of 46
QID: 10103
Mark
A
B
C
D
E
Given that the patient described above likely has schizophrenia but suffered adverse effects from haloperidol, it is likely that she was switched to another antipsychotic, perhaps an atypical antipsychotic,
for long-term use. While typical neuroleptics inhibit dopamine D
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receptors, atypical antipsychotics inhibit both serotonin and dopamine receptors. Prolactin is under tonic inhibition by dopamine from the
hypothalamus. With the inhibition of dopamine by an antipsychotic medication, the tonic inhibition is relieved, and in the process, prolactin levels increase. Prolactin inhibits gonadotropin-releasing hormone
The patient is given a suitable alternative to treat her psychiatric illness. She responds favorably, is placed on this medication long-term, and now presents to her psychiatrist for follow-up.
What laboratory test abnormalities might this patient have due to her long-term use of her current medication?
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Item: 31 of 46
QID: 3995
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A.Fructokinase deficiency
B.Galactokinase deficiency
C.Galactose-1-phosphate uridyltransferase deficiency
D.Glucose-6-phosphatase deficiency
E.Lactase deficiency
Gluconeogenesis is the process by which glucose is produced in the liver and kidney from small noncarbohydrate precursors such as alanine and lactate. Glucose-6-phosphatase is a key enzyme involved in
the final step of this pathway, catalyzing the conversion of glucose-6-phosphate to glucose. A deficiency of this enzyme causes von Gierke's disease, a type 1 glycogen storage disease characterized by an
inability to produce glucose from glycogen. Affected patients, like the one described here, usually present in infancy with hypoglycemia, seizures, hepatomegaly, and lactic acidosis.
Fructokinase deficiency involves a defect in fructose metabolism, causing a benign asymptomatic condition of fructosemia and fructosuria.
Galactokinase is an enzyme involved in the conversion of galactose to glucose-1-phosphate. A deficiency in this enzyme causes a less severe form of galactosemia characterized only by galactosuria and
infantile cataracts.
Galactose-1-phosphate uridyltransferase is an enzyme involved in the conversion of galactose to glucose-1-phosphate, which can then enter a variety of biochemical pathways such as glycolysis or
gluconeogenesis. A deficiency in this enzyme causes classic galactosemia, a condition characterized by the accumulation of toxic substances (including galactitol) that cause infantile cataracts,
hepatosplenomegaly, failure to thrive, and mental retardation. Treatment involves the exclusion of galactose and lactose from the diet.
A 6-month-old infant is brought into the emergency department with seizures. She is the product of a normal, full-term pregnancy and delivery and has had no health problems in the past. Physical
examination reveals hepatomegaly. Her heart rate is 180/min and respiratory rate is 75/min. A heel stick shows that the infant is hypoglycemic.
What is the most likely etiology of the patient's presentation?
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Item: 32 of 46
QID: 1781
Mark
A.Prerenal failure due to hypotension
B.The paraneoplastic effects of the cancer
C.The patient's history of chemotherapy
D.The patient's hypokalemia
E.The patient's hypotension leading to a lactic acidosis
Acidic urine in the presence of metabolic alkalosis, known as paradoxical aciduria, is primarily a result of hypokalemia in the presence of volume contraction . When a patient is vomiting, the alkalemic and
dehydrated patients try to conserve volume by activating the renin-angiotensin-aldosterone system. As the body reabsorbs sodium, potassium is exchanged and excreted in the distal collecting ducts. To
A 56-year-old woman with a history of advanced cervical cancer who completed treatment with radiotherapy and chemotherapy several months ago is admitted to the hospital for worsening symptoms
of abdominal pain and severe continuous vomiting over a 3-day period. The patient's blood pressure is 108/75 mm Hg supine and 95/60 mm Hg standing. Laboratory studies show:
Serum pH: 7.55
Blood urea nitrogen: 31 mg/dL
Na
+
: 141 mEq/L
K
+
: 3.3 mEq/L
Cl
-
: 90 mEq/L
Total CO
2
: 35 mEq/L (normal range: 20-29 mEq/L)
Urine pH: 5.1
Urine Na
+
: 10 mEq/L
Creatinine: 1.2 mg/dL
Which of the following is the most likely cause of the observed urine pH?
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QID: 3531
Mark
A.Avoidant personality disorder
B.Paranoid personality disorder
C.Schizoid personality disorder
D.Schizophrenia
E.Schizophreniform disorder
F. Schizotypal personality disorder
This man has schizoid personality disorder, marked by a lifelong pattern of social withdrawal. People with this disorder experience discomfort with human interaction and therefore isolate themselves, avoid
close relationships, and engage in solitary activities. These patients often are viewed as eccentric, isolated, lonely, and emotionally cold.
Avoidant patients are like schizoid patients in their pervasive pattern of social inhibition. However, they do desire companionship; an intense fear of rejection leads to avoiding any situation where there is a
perceived risk of rejection. Think avoidant personality disorder in patients inhibited by feelings of inadequacy and social ineptness to the extent they will participate socially only when they are certain to be
liked.
Patients with paranoid personality disorder tend to be more socially engaged than those with schizoid personality disorder, even though they have a lifelong history of suspiciousness and mistrust of other
people. Examples include recurrent suspicion of a sexual partner's fidelity or blaming others for their problems.
Patients with schizophrenia exhibit a formal thought disorder with hallucinations, or delusional thinking. In contrast, patients with schizoid personality disorder have intact reality testing.
A 60-year-old African-American man has been reclusive, rarely leaving his home for the past 40 years. His family describes him as an emotionally cold person with few friends. Growing up, he preferred
solitary activities like reading to engaging in activities with others. Members of his church have delivered groceries to his front door once a week for the past 20 years, but he never opens the door to
greet them.
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Item: 34 of 46
QID: 1426
Mark
A.Alcohol history
B.Family history
C.Sexual history
D.Smoking history
E.Travel history
This patient exhibits signs of Buerger's disease. Buerger's, also known as thromboangiitis obliterans, is a small- to medium-vessel vasculitis. It most often affects young men from South Asia and the Middle
East. Because the pathophysiology of Buerger's is essentially small-vessel ischemia, its clinical presentation mimics that of peripheral arterial disease (including distal pulses being decreased as compared
with proximal pulses; thus the brachial and popliteal pulses can still be palpated, whereas the radial, ulnar, and dorsalis pedis pulses cannot). Buerger's disease can also include Raynaud's phenomenon
because of peripheral nerve ischemia. Tobacco use is the strongest predisposing risk factor, and a positive smoking history sharply points clinical suspicion toward Buerger's. Smoking cessation is first-line
treatment.
Alcohol use is not typically related to Buerger's disease or the other vasculitides.
Family history is not useful in diagnosing Buerger's disease. A family history of diabetes or hypertension may predispose an individual to peripheral arterial disease (PAD), which carries a similar
presentation, but the two diseases arise from separate etiologies. In differentiating between the two, PAD is an atherosclerotic disease that typically shows up in older individuals.
Buerger's disease is not known to be associated with any sexually transmitted diseases. The question stem paints the picture of a vascular disease. The most important infectious disease related to the
vascular system is tertiary syphilis, which can cause an ascending aortic aneurysm.
A 33-year-old man presents with increasing pain in his hands. On physical examination, his hands are cold and tender to palpation, with some regions of ulceration and dry gangrene over the digits.
There is also marked pallor, which the patient claims is exacerbated by cold weather. He has decreased radial, ulnar, posterior tibial, and dorsalis pedis pulses, but preserved brachial and popliteal
pulses. The rest of the examination is unremarkable.
Which of the following would be most useful in determining the cause of the patient's condition?
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Item: 35 of 46
QID: 3779
Mark
A.Despite his paralysis, the patient's consciousness remains fully intact
B.The patient cannot hear or comprehend anything around him
C.The patient has become blind and will not regain his sight
D.The patient most likely will regain movement in his extremities
E.The patient will no longer have normal sleep-wake cycles
This patient presents with locked-in syndrome, a rare neurologic disorder characterized by complete paralysis of voluntary muscles except for those that control vertical eye movement. Individuals with
locked-in syndrome are conscious and can think and reason, but can communicate only by blinking eye movements. It is caused by infarct or other injury to the ventral pons, affecting bilateral corticospinal
and corticobulbar tracts, and typically is seen in basilar artery thrombosis . Sensory pathways and reticular activating systems are spared, and therefore patients are fully aware and able to feel, hear, and
understand everything in their environment.
Patients with locked-in syndrome have total preservation of sensory function, including hearing and higher cortical function. The defects in this condition are purely motor in nature, and this patient should
have normal comprehension and consciousness of everything around him.
The visual pathways involve the eye, optic nerve, optic chiasm, optic tract and radiations, and primary visual as well as visual association cortex. All of these structures are located above the pons and
receive their blood supply from the anterior cerebral circulation, which derives from the internal carotid arteries. These structures, and thus this patient's sight, are preserved in locked-in syndrome.
This patient's lesion resulted from the complete disruption of the descending corticospinal and corticobulbar tracts. It is extraordinarily unlikely that this patient with locked-in syndrome will ever regain any
movement in his extremities.
A 56-year-old man is hospitalized in the neurology intensive care unit after suffering a stroke. The physicians explain to his wife that he has experienced a thrombosis in the basilar artery. She asks for
information regarding his prognosis.
Which of the following statements best describes this patient's current condition?
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Item: 36 of 46
QID: 3857
Mark
A.Diffuse petechiae and purpura
B.Excessive bleeding after extraction of an impacted molar
C.Hepatosplenomegaly
D.Knee pain and swelling after light trauma against a table
E.Schistocytes on peripheral blood smear
The laboratory results indicate a likely diagnosis of von Willebrand's disease (vWD), an autosomal dominant dysfunction of von Willebrand's factor (vWF) that results in impaired adhesion of platelets to
collagen. Mucocutaneous bleeding, which may occur after dental extraction, is the most common initial presentation. In patients with moderate disease, hemarthroses are rare and generally occur only with
major trauma. Instead, hemarthrosis is associated more commonly with hemophilia A or B. Impaired platelet function produces a prolonged bleeding time. vWF is a carrier molecule for factor VIII,
extending its half-life. Thus vWD results in decreased factor VIII levels and a prolonged partial thromboplastin time. Reduced ristocetin-induced platelet aggregation, a test in which ristocetin, formerly used
as an antibiotic, stimulates platelets to aggregate, is a specific and sensitive indication of vWD.
Petechiae and purpura are common signs of thrombocytopenia, which may be a result of aplastic anemia, splenic sequestration, disseminated intravascular coagulation, thrombotic thrombocytopenic
purpura, idiopathic thrombocytopenic purpura, or medications, among others. Laboratory findings would include thrombocytopenia, which is not seen in this case.
An 18-year-old woman presents with a "bleeding problem." Coagulation studies are notable for a prolonged bleeding time, prolonged partial thromboplastin time, decreased factor VIII levels, and
reduced platelet aggregation induced by an antibiotic. Results of complete blood count are as follows:
Hematocrit: 33%
Leukocyte count: 5400/mm
Platelet count: 200,000/mm
Which of the following is most likely to have been the initial presentation of the "bleeding problem"?
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Item: 37 of 46
QID: 4403
Mark
A.Attention deficit/hyperactivity disorder
B.Conduct disorder
C.Oppositional defiant disorder
D.Separation anxiety disorder
E.Tourette's syndrome
As an adult, this woman would be diagnosed with antisocial personality disorder (ASPD), which is marked by a disregard for the rights of others and the rules of society. Patients with this disorder often
have a history of behavior problems, such as criminality, conning others, impulsive behavior, violence, and lack of remorse, that typically started when they were minors. Under the age of 18, behavior that
violates social norms, such as hurting animals, setting fires, and stealing can be seen in children with conduct disorder. Evidence of conduct disorder occurring before age 15 is required for a diagnosis of
ASPD.
Attention deficit/hyperactivity disorder is characterized by emotional lability and impulsivity.
Children with oppositional defiant disorder may not comply with an authority figure's requests, but are still able to feel guilty for their own actions and empathy towards others.
Separation anxiety disorder often arises around age 7 and is characterized by clinginess and fear of loss of attachment figure.
Tourette's syndrome is characterized by involuntary, repetitive motor and vocal tics.
A 24-year-old woman is referred to a psychiatry clinic as part of her parole agreement. She has a long history of legal trouble and has been incarcerated for most of her life since age 15 years. Her most
recent imprisonment was the result of a robbery in which she assaulted one of her victims. When asked why she did this, she replies "That's the way of the world. You can't trust anybody. If you want
something you've got to take it, no matter who you hurt."
Which psychiatric disorder did this woman most likely have when she was a teenager?
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Item: 38 of 46
QID: 3865
Mark
A.t(8;14)
B.t(9;22)
C.t(11;14)
D.t(14;18)
E.t(15;17)
A 9-year-old boy with massive swelling of his right mandible is seen during a medical relief mission. He has lost a significant amount of weight over the past 2 months. In addition he has night sweats
requiring a change in his bedclothes. He has no cough, murmur, or change in bowel habits. A biopsy of the spleen is taken, and results are shown in the image.
What translocation is likely present in the malignant cells shown in the image?
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Item: 39 of 46
QID: 10097
Mark
A 42-year-old man presents to the emergency department with 8/10 pain in his right knee. The joint space is swollen, red, and exquisitely tender to palpation. Aspirated synovial fluid is examined using
light microscopy with polarized light. A representative field is shown in the image.
What initial pharmacologic therapy is most important to give this patient?
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Item: 40 of 46
QID: 10098
Mark
A.Coronary artery stenosis
B.Interstitial pulmonary fibrosis
C.Leukemia
D.Nephropathy
E.Pericarditis
Gouty nephropathy is the most common extra-articular manifestation of chronic gout. Urate crystals are deposited in the renal medullary interstitium where they can form intratubular precipitates,
inflammatory tophi, or uric acid renal stones. Tubular obstruction leads to cortical atrophy and scarring. Approximately 20% of those with chronic gout eventually die of renal failure.
Coronary artery stenosis occurs by a complex pathologic mechanism involving the deposition of lipids in the lining of the arteries. While the resultant inflammatory response is thought to play a role in the
pathogenesis of coronary artery disease, chronically elevated uric acid levels are not known to precipitate arterial stenosis.
Interstitial pulmonary fibrosis is associated with rheumatologic disease processes such as sarcoidosis. It is not, however, a known consequence of chronic gout.
Leukemia is associated with gout as a secondary cause of increased serum uric acid levels rather than as a consequence. The increased cell turnover from uncontrolled proliferative processes results in
greater nucleic acid release and breakdown, thus raising serum uric acid levels.
Pericarditis, along with inflammation of other serosal surfaces such as the pleura of the lung, may often occur in rheumatologic disorders such as lupus. It is not, however, a known consequence of chronic
After his acute flare-up resolves, the patient returns to the outpatient clinic 2 weeks later. A serum uric acid level is taken and found to be 11 mg/dL. It is determined that he will begin probenecid
therapy in an attempt to maintain lower serum levels of uric acid.
If the patient's serum uric acid is not well managed, what condition is he at the greatest risk of developing?
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Item: 41 of 46
QID: 5136
Mark
A.Abciximab
B.Aspirin
C.Clopidogrel
D.Ticlopidine
E.Tissue plasminogen activator
Platelet aggregation is a complex interaction mediated by multiple molecular interactions. Glycoprotein (GP) IIb/IIIa is expressed on the surface of platelets, and interacts with fibrinogen to allow platelet
aggregation. The monoclonal antibody abciximab works by binding to GP IIb/IIIa, thereby preventing platelet aggregation. Its indications include acute coronary syndrome, angioplasty, and percutaneous
coronary stent placement.
Aspirin or acetylsalicylic acid irreversibly inhibits cyclooxygenase, thereby inhibiting the production of prostaglandins from arachidonic acid. It does not bind directly to GP IIb/IIIa, which normally interacts
with fibrinogen.
Clopidogrel (Plavix) is an anti-platelet drug that irreversibly blocks the ADP receptor on platelets, decreasing both aggregation and the expression of GP IIb/IIIa. It does not bind directly to GP IIb/IIIa,
which normally interacts with fibrinogen.
Ticlopidine is an anti-platelet drug that irreversibly blocks the ADP receptor on platelets, decreasing both aggregation and the expression of GP IIb/IIIa. It does not bind directly to GP IIb/IIIa, which
normally interacts with fibrinogen.
A 54-year-old man with a history of hypertension and hyperlipidemia presents to the emergency department with 45 minutes of chest pressure radiating to the left arm and jaw. An ECG shows
ST-segment elevations consistent with an MI. To decrease further clot propagation, he is started on an anti-platelet agent that binds to the same receptor normally bound by fibrinogen.
Which of the following drugs is most likely being administered?
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Item: 42 of 46
QID: 2267
Mark
A.Decreased homocysteine level
B.Decreased level of lactate dehydrogenase
C.Elevated WBC count
D.Increased methylmalonic acid
E.Negative anti-intrinsic factor antibody
F. Negative result of Schilling's test
In a minority of patients with cobalamin (vitamin B
12
) deficiency, the hematocrit and mean corpuscular volume are normal. In these cases, laboratory testing for increased methylmalonic acid (MMA) can be
used to make the diagnosis. MMA is increased in vitamin B
12
deficicency because B
12
is a necessary cofactor in the conversion of MMA to succinyl CoA.
In patients with cobalamin deficiency, the homocysteine level is typically increased. The homocysteine level can be used to diagnose cobalamin deficiency in symptomatic patients in whom the hematocrit
and mean corpuscular volume are normal.
Lactate dehydrogenase is increased in cobalamin deficiency because of failed hematopoiesis.
Because cobalamin deficiency impairs DNA synthesis in all cell lines, neutropenia with hypersegmented neutrophils typically is seen in cobalamin-deficient patients.
A positive anti-intrinsic factor antibody test suggests inhibition of the binding between intrinsic factor and cobalamin. Because this decreases absorption of the vitamin, antibodies against intrinsic factor can
Physical examination of a 60-year-old woman reveals gait instability and decreased proprioception in her lower extremities. Blood tests show a normal hematocrit level and near-normal mean cell
volume. Her physician orders additional tests.
Which of the following laboratory results supports a diagnosis of cobalamin deficiency?
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QID: 1830
Mark
A.A CD4+ cell count <200/mm only
B.Serologic evidence of HIV infection and a CD4+ cell count <300/mm
C.Serologic evidence of HIV infection only
D.Serologic evidence of HIV infection, a CD4+ cell count <200/mm, and/or one or more defining opportunistic infections
E.Serologic evidence of HIV infection, a CD4+ cell count <400/mm, and/or two or more defining opportunistic infections
In 1993, the CDC refined its definition of AIDS to include all HIV-infected persons with a CD4+ cell count <200/mm, all those with a CD4 percentage <14%, and/or all those with opportunistic infections
such as pulmonary tuberculosis, recurrent pneumonia, or invasive cervical cancer.
A CD4+ cell count of <200/mm only is not a complete definition of AIDS.
Serologic evidence of HIV infection and a CD4+ cell count of <300/mm do not constitute a complete definition of AIDS.
Serologic evidence of HIV infection only is not a complete definition of AIDS.
The CD4+ cell count must be <200/mm for a diagnosis of AIDS.
A homeless man arrives at the clinic with diarrhea, multiple mouth sores, and a low-grade fever. On physical examination, white patches on his tongue that do not wipe off with a tongue blade are
noted.
Based on recommendations from the Centers for Disease Control and Prevention, which of the following is required to diagnose this illness?
Bottom Line:
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Item: 44 of 46
QID: 2607
Mark
A.Dorsal mesentery
B.Lung bud
C.Pancreatic islet cells
D.Ureteric bud
E.Ventral pancreatic bud
This patient is presenting with signs and symptoms of a pancreatic adenocarcinoma. These tumors arise most commonly in the pancreatic head, and they may not present until an advanced stage when
they have obstructed the common bile duct, resulting in jaundice. Patients with pancreatic adenocarcinoma may also experience weight loss and abdominal pain radiating to the back. This patient manifests
two other signs sometimes seen in pancreatic adenocarcinoma: migratory thrombophlebitis and a palpable gallbladder. Given both the epidemiology of this cancer and the patient's jaundice, it is likely that
his tumor arose in the head of the pancreas, which is derived from the ventral pancreatic bud. Other derivatives of the ventral bud include the uncinate process and main pancreatic duct.
The dorsal mesentery gives rise to, among other things, the adult spleen. This patient has an adenocarcinoma of the head of the pancreas. The pancreatic head is derived from the ventral pancreatic bud,
not the dorsal mesentery.
This patient has adenocarcinoma of the pancreatic head. The lung bud plays a role in development of the adult lungs, but it does not contribute to any part of the pancreas.
Pancreatic islet cells, like pancreatic acinar cells, are derived from endoderm.
The adult kidneys (except for the nephrons) are derived from the ureteric bud. This patient, however, presents with an adenocarcinoma of his pancreas.
A 62-year-old man with a history of tobacco use presents to his physician complaining that his skin has been "turning yellow." Physical examination reveals a nontender mass in his right upper quadrant
and both erythema and tenderness of his left leg. CT of the abdomen reveals an underlying malignancy.
Which of the following is the embryologic origin of the most likely site of this patient's tumor?
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Item: 45 of 46
QID: 5050
Mark
A.3 months
B.6 months
C.9 months
D.12 months
E.24 months
At 6 months, a child who has reached his appropriate developmental milestones should be able to roll over, hold a sitting position unassisted, use a raking grasp, recognize familiar people, and babble.
Given the developmental milestones achieved in this child, he is most likely to be around the age of 6 months.
At 3 months, a child who has reached his appropriate developmental milestones should be able to lift his head and shoulders when lying prone, smile socially, and coo in response to human attention.
At 9 months, a child who has reached his appropriate developmental milestones should be able to sit alone, crawl, pull himself to a standing position, use a pincer grasp, recognize familiar people, respond
to simple instructions, and use gestures.
At 12 months, a child who has reached his appropriate developmental milestones should be able to walk unassisted and say a few words. This period of development is also when children experience
separation anxiety when they are away from their parents.
At 24 months, a child who has reached his appropriate developmental milestones should be able to stack six blocks, kick a ball, undress himself, use a spoon or fork, play alongside other children in parallel
play, use approximately 250 words and two-word sentences, name body parts, and use pronouns.
A child is able to roll over, sit unassisted, and use a raking grasp to pick up objects. In addition, he recognizes his parents and grandparents and has recently begun to babble.
Based on the developmental milestones achieved, what is this child's most likely age?
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Item: 46 of 46
QID: 3544
Mark
A.Acute disseminated encephalomyelitis
B.Cryptococcal meningitis
C.Guillain?Barre syndrome
D.Herpes simplex meningitis
E.Progressive multifocal leukoencephalopathy
This patient most likely now has AIDS and progressive multifocal leukoencephalopathy (PML), an opportunistic infection that affects the central nervous system (CNS). PML is the result of the reactivation of
latent JC papovavirus, usually after the patient's CD4 count falls to less than 200/mm. Approximately 75% of all humans have been exposed to the JC virus. PML is a fatal CNS disease that causes
demyelination of the white matter. Disease progression is subacute, and it is initially marked by visual field deficits, mental status changes, and weakness. The disease progresses to blindness, dementia,
coma, and death, typically within 6 months. Cerebrospinal fluid analysis is usually unremarkable, although the fact that PML does not enhance on MRI with contrast is a key feature.
Acute disseminated (postinfectious) encephalomyelitis (ADEM) is a multifocal periventricular inflammation and demyelination after infection (eg, chickenpox, measles) or certain vaccinations (eg, rabies,
smallpox). ADEM produces multiple inflammatory lesions in the brain and spinal cord, particularly in the white matter. Hence, it presents similarly to multiple sclerosis because of its demyelinating disease
process. Unlike PML, it does not have an increased incidence in patients who have an HIV infection or AIDS.
Cryptococcal meningitis is classically associated with the acute onset of a severe headache, fever, nuchal rigidity, a change in mental status, focal neurologic signs, and high intracranial pressures with
papilledema. The clinical presentation described is more consistent with progressive multifocal leukoencephalopathy than cryptococcal meningitis.
Guillain-Barr syndrome (GBS) generally causes symmetric weakness that begins in the distal extremities and that ascends to affect the proximal extremities and the trunk. It is associated with recent
A 33-year-old HIV-positive man is taken to the emergency department by his roommate. Initially the patient complained of generalized weakness and vision difficulties, which started several weeks ago.
Later, the patient lost his peripheral vision and became unable to talk or walk. Lumbar puncture reveals unremarkable cerebrospinal fluid. MRI of the brain reveals multiple, non-contrast-enhancing
lesions in the white matter, primarily in the parietal and occipital lobes. His CD4 count is 35/mm.
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