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Quiz for Practitioners
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1

The use of repeated phlebotomy in the treatment of persons with symptomatic hemochromatosis may be expected to result in
A)increased skin pigmentation
B)improved cardiac function
C)return of secondary sex characteristics
D)decreased joint pain
E)an unchanged 5-year survival rate
2

A 19-year-old man has had a 5-year history of hyperglycemic episodes and glycosuria. However, he has never been hospitalized for diabetic ketoacidosis. Which of the following statements regarding the mode of inheritance of his disease is correct?
A)This disease is inherited in an autosomal recessive fashion.
B)If the patient has children, they will have approximately a 50% chance of developing diabetes.
C)The diabetic susceptibility gene in this patient resides on human chromosome 6.
D)The patient is likely to carry one of a limited number of HLA-D locus alleles.
E)The patient has an unusual susceptibility to a viral infection.
3

Which of the following studies is most sensitive for detecting diabetic nephropathy?
A)Serum creatinine level
B)Creatinine clearance
C)Urine albumin
D)Glucose tolerance test
E)Ultrasonography
4

Which of the following statements concerning intensive insulin therapy for diabetes (the use of an external insulin pump or three or more daily insulin injections guided by frequent blood glucose monitoring) is correct?
A)All patients with diabetes mellitus should receive such therapy.
B)It has been definitively shown that compared with standard therapy, such intensive therapy reduces the likelihood of retinopathy in patients with insulin-dependent diabetes mellitus.
C)Such therapy will consistently return blood glucose to normal levels, but a reduction of long-term complications has not been demonstrated.
D)With careful monitoring, an increase in the number of hypoglycemic episodes is avoided.
E)Intensive insulin therapy failed to reduce the level of glycosylated hemoglobin.
5

Evidence of continuing ovarian estrogen production in a 29-year-old woman who is being evaluated for secondary amenorrhea is provided by
A)normal plasma estrone and luteinizing hormone (LH) levels
B)a normal plasma prolactin level
C)an increase in plasma estradiol level after the administration of human chorionic gonadotropin (hCG)
D)the appearance of menses after a short course of progesterone therapy
E)a lack of hot flashes
6

Which of the following inhibits growth hormone secretion from the anterior pituitary gland?
A)Somatostatin
B)Growth hormone-releasing hormone (GHRH)
C)Hypoglycemia
D)Arginine
E)Serotonin
7

A 7-year-old girl is referred for evaluation of vaginal bleeding for 2 months. The mother says that she has not been exposed to exogenous estrogens. Physical examination reveals height at the 98th percentile, Tanner stage III breast development, and no axillary or pubic hair. No abdominal or pelvic masses are palpated. Neurologic examination is normal. Radiographic and laboratory evaluations reveal the following:
  • Brain MRI: normal pituitary and hypothalamus
  • Bone age: 10 years
  • Urinary 17-ketosteroids: 1.7 mol (0.5 mg)/g creatinine per 24 h
  • Urinary gonadotropins: undetectable

The appropriate next step in the management of this girl would be

A)exploratory laparotomy
B)treatment with medroxyprogesterone acetate
C)measurement of plasma androstenedione level
D)abdominal CT scanning and/or pelvic sonography
E)karyotype analysis
8

A 40-year-old man presents with an insidious onset of fatigue, headaches, muscle weakness, and paresthesia. Physical examination reveals hypertension, an enlarged tongue, wide spacing of the teeth, and a doughy appearance to the skin. Which of the following laboratory results would be consistent with the expected diagnosis?
A)Elevated serum thyroxine level
B)Fasting serum glucose = 3.3 mmol/L (60 mg/dL)
C)Elevated insulin-like growth factor (IGF-I)
D)Growth hormone concentration = 0.2 g/L (0.2 ng/mL) 1 h after oral administration of 100 g glucose
E)Decreased IGF binding protein 3
9

Which of the following statements concerning the diagnosis of pheochromocytoma is correct?
A)Measurement of plasma catecholamines is the preferred initial screening test.
B)Random urine samples are equivalent in diagnostic accuracy to the measurement of catecholamies or catecholamine metabolites in a 24-h urine collection.
C)After collection, the urine should be treated with dilute sodium hydroxide and refrigerated.
D)The ideal time to collect urine is during a period of clinical stability.
E)Strenuous exertion may falsely elevate the level of free urinary catecholamines.
10

An 18-year-old woman arrives in your clinic with primary amenorrhea, sexual infantilism, and clitoromegaly. She had ambigous external genitalia at birth. A laparotomy was performed at 17 months of age and revealed normal internal female genitalia. An ovarian biopsy revealed apparently normal primordial follicles. Laboratory studies revealed a normal female karyotype and high serum testosterone and androstenedione concentrations. Undetectable serum estradiol and estrone concentrations were also noted. There was a high serum follicle-stimulating hormone and LH concentration. Pelvic imaging disclosed multiple ovarian cysts. What is the most likely diagnosis?
A)Congenital adrenal hyperplasia
B)Aromatase deficiency
C)McCune-Albright syndrome
D)Kallmann's syndrome
E)Galactosemia
11

A female patient arrives in your clinic with a serum calcium of 2.7 mmol/L (10.8 mg/dL). The patient is asymptomatic, and this abnormality is found on routine laboratory analysis. A workup includes a normal CBC, normal liver function tests, and a normal serum protein electrophoresis. A serum parathyroid hormone level is 136 ng/L (136 pg/mL), a 24-h urinary calcium is 268 mg, and a serum alkaline phosphatase level is 106 U/L. The patient has no history of orthopedic fractures or nephrolithiasis. A bone densitometry study is performed that reveals a lumbar spine z-score of -0.86, a femoral neck z- score of -1.34, and a radius z-score of -1.42. A parathyroidectomy is likely to result in which of the following?
A)Normalization of the serum calcium level, improvement of the bone densitometry studies, and a decreased incidence of nephrolithiasis
B)Normalization of the serum calcium level, improvement of the bone densitometry studies, and a decreased incidence of renal failure
C)Normalization of the serum calcium level, improvement of the bone densitometry studies, and a decreased in the incidence of pelvic and hip fractures
D)Normalization of the serum calcium level, improvement of the bone densitometry studies, and a decrease in the incidence of radial fractures
E)Normalization of the serum calcium level and improvement of the bone densitometry studies only
12

A 42-year-old alcoholic man has eaten poorly for the last 10 days but has continued to drink. His family brings him to the emergency room. On neurologic examination he is confused but otherwise normal. Blood glucose concentration is 2.8 mmol/L (50 mg/dL). Intravenous infusion of a bolus of 50% glucose solution is given. His confusion worsens, and he develops horizontal nystagmus, ataxia, and a heart rate of 130 beats per minute. At this point, the man's physician should
A)order an immediate CT scan of the head
B)perform a lumbar puncture
C)administer another bolus of 50% glucose solution
D)administer intravenous folic acid, 5 mg
E)administer intramuscular thiamine, 50 mg
13

A 24-year-old woman with a several-year history of chronic, debilitating, cramping abdominal pain has been evaluated several times for this problem. In each case the possibility of psychogenic causes has been raised because of the absence of abdominal tenderness, fever, and leukocytosis during the episodes. The patient has had intermittent vomiting, constipation, arm and chest pain, and difficulty in urination. She also complains of increasing leg weakness. The attacks of abdominal pain are often associated with anxiety, insomnia, and disorientation. A prior workup has also included abdominal angiography, abdominal CT, and endoscopy. The results of all the diagnostic studies were normal. The patient's current physical examination and routine laboratory examination, including complete blood count and serum chemistries, are unremarkable. The urinary pyrrole porphobilinogen excretion is elevated. Which of the following is the most appropriate advice for this patient?
A)The patient's offspring may be at risk only if the father is also a carrier of this disease.
B)Intravenous administration of heme may ameliorate the attacks.
C)Narcotic analgesics should not be used during acute attacks.
D)The patient should avoid aspirin.
E)The patient should avoid prolonged exposure to the sun.
14

A 54-year-old obese male presented with fasting blood serum glucose of 12.2 mmol/L (220 mg/dL); this was repeated several times with similar results. He underwent a diet and exercise program, but after 6 months was able to decrease his weight only from 127 to 118 kg (280 to 260 lb). There was no significant change in his fasting serum glucose level. A test for thyroid function was normal. A fasting cholesterol level was 7.5 mmol/L (290 mg/dL), with a low-density lipoprotein (LDL) cholesterol of 4.9 mmol/L (190 mg/dL). His renal and hepatic function are within normal limits. Which of the following oral anti-diabetic agents would you recommend?
A)Metformin
B)Glipizide
C)Repaglinide
D)Acarbose
E)None, only insulin therapy should be initiated
15

A full cholesterol panel is performed on the patient described in Question XII-14. In addition to the stated cholesterol and LDL cholesterol levels, the serum triglyceride level is 3.4 mmol/L (300 mg/dL) and the high-density lipoprotein (HDL) cholesterol level is 0.9 mmol/L (35 mg/ dL). Which of the following would be the most appropriate therapeutic recommendation?
A)Diet and exercise
B)Initiation of an HMG-CoA reductase inhibitor
C)Nicotinic acid
D)Fibrate therapy
E)No pharmacologic therapy is indicated at this time; the patient should be reevaluated after tighter glycemic control is achieved
16

A 20-year-old woman has a history of multiple fractures since childhood, kyphoscoliosis, bluish-gray teeth, and conductive hearing loss. Examination of the face reveals blue sclerae. Several relatives on her mother's side have been similarly affected. She has no history of physical abuse or abnormal serum chemistries. The most likely mechanism of the patient's abnormalities is
A)excessive deposition of normal collagen fibrils in bone
B)inability to convert procollagen to collagen
C)mutation in the gene for type I procollagen
D)mutation in the gene for type II procollagen
E)mutation in the gene for type III procollagen
17

A clinical presentation that includes long thin extremities, dislocation of the ocular lens, and aortic aneurysms is most likely due to a derangement in which of the following molecules?
A)Procollagen type I
B)Procollagen type II
C)Proteoglycan
D)Elastin
E)Fibrillin
18

A 25-year-old man with a renal allograft and a history of an intracerebral abscess is evaluated for profound polyuria. He is admitted to the hospital for a water deprivation test. No fluids are given after 12 midnight. By 11 A.M. he has lost 1 kg, and urine osmolality has been 120 mosmol/kg for the last 3 h. Plasma osmolality is 320 mosmol/kg (serum sodium is 155 mmol/L). At 11 A.M. 1 g desmopressin is given by subcutaneous injection; 45 min later the urine osmolality is measured at 121 mosmol/kg. The patient is then allowed to drink. Treatment of this patient should include
A)vasopressin tannate in oil
B)hydrochlorothiazide
C)desmopressin
D)chloropropamide
E)demeclocycline
19

A person with hypercalcemia caused by sarcoidosis would have which of the following findings?
A)A normal chest x-ray
B)Increased absorption of calcium from the gastrointestinal tract
C)Normal urine calcium excretion
D)Increased serum parathyroid hormone level
E)Hypogammaglobulinemia
20

The most likely etiology for the eating disorder anorexia nervosa is
A)decreased levels of luteinizing hormone-releasing hormone (LHRH)
B)decreased levels of growth hormone
C)decreased levels of insulin-like growth factor I (somatomedin C)
D)low levels of serum thyroxine
E)psychiatric disorder
21

A 45-year-old obese man without known medical problems complains of feeling very sleepy during the day and often falling asleep while listening to friends. The most likely cause of this patient's problem is
A)narcolepsy
B)upper airway obstruction at night
C)glucocorticoid excess
D)growth hormone excess
E)estrogen excess
22

A 67-year-old man with chronic arthritis is found to have passed a uric acid stone after an episode of renal colic. On workup he is found to have multiple radiolucent stones in the left renal pelvis, uric acid excretion of 5.4 mmol/d (900 mg/d), a serum uric acid concentration of 580 mol/L (9.8 mg/dL), a serum creatinine concentration of 160 mol/L (1.8 mg/dL), and monosodium urate crystals in an effusion in the left knee. The drug of choice for long-term therapy in this patient is
A)probenecid alone
B)probenecid and sodium bicarbonate
C)allopurinol
D)colchicine
E)sulfinpyrazone
23

An obese woman has hypertriglyceridemia without hypercholesterolemia. The most appropriate first step in the treatment of this woman would be
A)weight reduction
B)nicotinic acid
C)gemfibrozil
D)clofibrate therapy
E)bile acid-binding resin therapy
24

An X-linked recessive disease characterized by nephrolithiasis, arthritis, self-mutilative behavior, and mental retardation is associated with
A)failure to excrete uric acid because of inherited defective renal tubular function
B)failure to excrete uric acid because of xanthine oxidase mutation
C)uric acid overproduction caused by inherited acceleration of purine degradation
D)increased urate production caused by an inability to convert purine bases to ribonucleotides
E)increased urate production caused by increased levels of phosphoribosylpyrophosphate
25

In designing a hormone replacement program for patients with coexistent thyroid and adrenal failure,
A)the dose of glucocorticoid must be increased slowly once thyroid replacement has been initiated
B)the dose of thyroid hormone must be increased slowly once glucocorticoid replacement has been initiated
C)mineralocorticoid replacement must also be included if combined therapy is required
D)thyroid replacement must not be initiated until treatment with glucocorticoid has been instituted
E)growth hormone replacement must also be included if combined therapy is required







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