Harrisons Principles of Internal Medicine Self-Assessment and Board Review 18th Ed.

SECTION X. Endocrinology and Metabolism

QUESTIONS

DIRECTIONS: Choose the one best response to each question.

X-1. All of the following represent examples of hypothalamic-pituitary negative feedback EXCEPT:

A.  Cortisol on the CRH-ACTH axis

B.  Gonadal steroids on the GnRH-LH/FSH axis

C.  IGF-1 on the growth hormone–releasing hormone (GHRH)-GH axis

D.  Renin-angiotensin-aldosterone axis

E.  Thyroid hormones on TRH-TSH axis

X-2. Endocrine dysfunction can be separated into glandular hyperfunction or hypofunction, or hormone resistance. Which of the following diseases is due to hormone resistance?

A.  Graves’ disease

B.  Hashimoto’s thyroiditis

C.  Pheochromocytoma

D.  Sheehan’s syndrome

E.  Type 2 diabetes mellitus

X-3. Secretion of gonadotropin-releasing hormone (GnRH) normally stimulates the release of luteinizing hormone (LH) and follicle-stimulating hormone (FSH), which promote the production and release of testosterone and estrogen. Which mechanism best explains how long-acting gonadotropin-releasing hormone agonists (e.g., leuprolide) decrease testosterone levels in the management of prostate cancer?

A.  GnRH agonists also promote the production of sex hormone–binding globulin, which decreases the availability of testosterone.

B.  Negative feedback loop between GnRH and LH/FSH.

C.  Sensitivity of LH and FSH to pulse frequency of GnRH.

D.  Translocation of the cytoplasmic nuclear receptor into the nucleus with constitutive activation of GnRH.

X-4. The mineralocorticoid receptor in the renal tubule is responsible for the sodium retention and potassium wasting that is seen in mineralocorticoid excess states such as aldosterone-secreting tumors. However, states of glucocorticoid excess (e.g., Cushing’s syndrome) can also present with sodium retention and hypokalemia. What characteristic of the mineralocorticoid-glucocorticoid pathways explains this finding?

A.  Higher affinity of the mineralocorticoid receptor for glucocorticoids

B.  Oversaturation of the glucocorticoid degradation pathway in states of glucocorticoid excess

C.  Similar, but distinct, DNA-binding sites producing the same metabolic effect

D.  Upregulation of the mineralocorticoid-binding protein in states of glucocorticoid excess

X-5. All of the following hormones are produced by the anterior pituitary EXCEPT:

A.  Adrenocorticotropic hormone

B.  Growth hormone

C.  Oxytocin

D.  Prolactin

E.  Thyroid-stimulating hormone

X-6. A 22-year-old woman who is otherwise healthy undergoes an uneventful vaginal delivery of a full-term infant. One day postpartum she complains of visual changes and severe headache. Two hours after these complaints, she is found unresponsive and profoundly hypotensive. She is intubated and placed on mechanical ventilation. Her blood pressure is 68/28 mmHg, heart rate is regular at 148 beats/min, and oxygen saturation is 95% on FiO2 0.40. Physical exam is unremarkable. Her laboratories are notable for glucose of 49 mg/dL and normal hematocrit and white blood cell count. Which of the following is most likely to reverse her hypotension?

A.  Activated drotrecogin alfa

B.  Hydrocortisone

C.  Piperacillin/tazobactam

D.  T4

E.  Transfusion of packed red blood cells

X-7. A 45-year-old man reports to his primary care physician that his wife has noted coarsening of his facial features over several years. In addition, he reports low libido and decreased energy. Physical examination shows frontal bossing and enlarged hands. An MRI confirms that he has a pituitary mass. Which of the following screening tests should be ordered to diagnose the cause of the mass?

A.  24-hour urinary free cortisol

B.  ACTH assay

C.  Growth hormone level

D.  Serum IGF-1 level

E.  Serum prolactin level

X-8. All of the following are potential causes of hyperprolactinemia EXCEPT:

A.  Cirrhosis

B.  Hirsutism

C.  Nipple stimulation

D.  Opiate abuse

E.  Rathke’s cyst

X-9. A 28-year-old woman presents to her primary care physician’s office with 1 year of amenorrhea. She reports mild galactorrhea and headaches. Although she is sexually active, a urine pregnancy test is negative. Serum prolactin level is elevated and she is subsequently diagnosed with a microscopic prolactinoma. Which of the following represents the primary goal of bromocriptine therapy for her condition?

A.  Control of hyperprolactinemia

B.  Reduction in tumor size

C.  Resolution of galactorrhea

D.  Restoration of menses and fertility

E.  All of the above

X-10. A 58-year-old man undergoes severe head trauma and develops pituitary insufficiency. After recovery, he is placed on thyroid hormone, testosterone, glucocorticoids, and vasopressin. On a routine visit he questions his primary care physician regarding potential growth hormone deficiency. All of the following are potential signs or symptoms of growth hormone deficiency EXCEPT:

A.  Abnormal lipid profile

B.  Atherosclerosis

C.  Increased bone mineral density

D.  Increased waist:hip ratio

E.  Left ventricular dysfunction

X-11. A 75-year-old man presents with development of abdominal obesity, proximal myopathy, and skin hyperpigmentation. His laboratory evaluation shows a hypokalemic metabolic alkalosis. Cushing’s syndrome is suspected. Which of the following statements regarding this syndrome is true?

A.  Basal ACTH level is likely to be low.

B.  Circulating corticotropin-releasing hormone is likely to be elevated.

C.  Pituitary MRI will visualize all ACTH-secreting tumors.

D.  Referral for urgent performance of inferior petrosal venous sampling is indicated.

E.  Serum potassium level below 3.3 mmol/L is suggestive of ectopic ACTH production.

X-12. A 23-year-old college student is followed in the student health center for medical management of panhypopituitarism after resection of craniopharyngioma as a child. She reports moderate compliance with her medications but feels generally well. A TSH is checked and is below the limits of detection of the assay. Which of the following is the next most appropriate action?

A.  Decrease levothyroxine dose to half of current dose.

B.  Do nothing.

C.  Order free T4 level.

D.  Order MRI of her brain.

E.  Order thyroid uptake scan.

X-13. A 23-year-old woman presents to the clinic complaining of months of weight gain, fatigue, amenorrhea, and worsening acne. She cannot precisely identify when her symptoms began, but she reports that without a change in her diet she has noted a 12.3-kg weight gain over the past 6 months. She has been amenorrheic for several months. On examination she is noted to have truncal obesity with bilateral purplish striae across both flanks. Cushing’s syndrome is suspected. Which of the following tests should be used to make the diagnosis?

A.  24-hour urine free cortisol

B.  Basal adrenocorticotropic hormone (ACTH)

C.  Corticotropin-releasing hormone (CRH) level at 8 a.m.

D.  Inferior petrosal venous sampling

E.  Overnight 1-mg dexamethasone suppression test

X-14. A patient visited a local emergency room 1 week ago with a headache. She received a head MRI, which did not reveal a cause for her symptoms, but the final report states, “An empty sella is noted. Advise clinical correlation.” The patient was discharged from the emergency department with instructions to follow up with her primary care physician as soon as possible. Her headache has resolved, and the patient has no complaints; however, she comes to your office 1 day later very concerned about this unexpected MRI finding. What should be the next step in her management?

A.  Diagnose her with subclinical panhypopituitarism, and initiate low-dose hormone replacement.

B.  Reassure her and follow laboratory results closely.

C.  Reassure her and repeat MRI in 6 months.

D.  This may represent early endocrine malignancy—whole-body positron emission tomography/CT is indicated.

E.  This MRI finding likely represents the presence of a benign adenoma—refer to neurosurgery for resection.

X-15. A 31-year-old woman is admitted to the hospital after an appendectomy for acute appendicitis. The surgery is uncomplicated, but postoperatively she is noted to make copious urine (6 L/d) and complain of severe thirst. On the third postoperative day, her BUN and creatinine are noted to be elevated. On further questioning, she reports a long history of extreme thirst, urinary frequency, and occasional episodes of enuresis that she was too embarrassed to bring to the attention of a health care practitioner. Aside from oral contraceptives, she takes no medications and reports no past medical history. Which of the following is the most appropriate first step to confirm her diagnosis?

A.  24-hour urine volume and osmolarity measurement

B.  Fasting morning plasma osmolarity

C.  Fluid deprivation test

D.  MRI of the brain

E.  Plasma ADH level

X-16. A 63-year-old man is admitted to the hospital to begin induction chemotherapy for acute myelomonocytic leukemia (AML-M4). He is afebrile and has been feeling well other than fatigue and bruising. His physical examination is notable for normal vital signs and no focal findings other than three 1- × 2-cm subcutaneous nodules that had previously been demonstrated to be cutaneous spread of AML-M4. On the night of admission, the patient’s wife calls for assistance because her husband’s mental status is altered. He is confused and somnolent. You notice that there are four urinals filled with urine by his bed. His wife reports that for the last 6 hours he has been urinating frequently and has been drinking water constantly. However, over the last hour, despite urinating frequently, he has not been able to drink water due to somnolence. Laboratory studies are notable for an absolute neutrophil count of 400, a platelet count of 35,000, and a serum sodium of 155. Which of the following therapies should be administered immediately?

A.  All-trans retinoic acid (ATRA)

B.  Desmopressin

C.  Hydrochlorothiazide

D.  Hydrocortisone

E.  Lithium

X-17. Which of the following is the most common cause of preventable mental deficiency in the world?

A.  Beriberi disease

B.  Cretinism

C.  Folate deficiency

D.  Scurvy

E.  Vitamin A deficiency

X-18. Which of the following proteins is the primary source of bound T4 in the plasma?

A.  Albumin

B.  Gamma globulins

C.  Transthyretin

D.  Thyroid peroxidase

E.  Thyroxine-binding globulin

X-19. All of the following are associated with increased levels of total T4 in the plasma with a normal free T4 EXCEPT:

A.  Cirrhosis

B.  Pregnancy

C.  Sick-euthyroid syndrome

D.  Familial dysalbuminemic hyperthyroxinemia

E.  Familial excess thyroid-binding globulin

X-20. Which of the following is the most common cause of hypothyroidism worldwide?

A.  Graves’ disease

B.  Hashimoto’s thyroiditis

C.  Iatrogenic hypothyroidism

D.  Iodine deficiency

E.  Radiation exposure

X-21. A 75-year-old woman is diagnosed with hypothyroidism. She has long-standing coronary artery disease and is wondering about the potential consequences for her cardiovascular system. Which of the following statements is true regarding the interaction of hypothyroidism and the cardiovascular system?

A.  Myocardial contractility is increased with hypothyroidism.

B.  A reduced stroke volume is found with hypothyroidism.

C.  Pericardial effusions are a rare manifestation of hypothyroidism.

D.  Reduced peripheral resistance is found in hypothyroidism and may be accompanied by hypotension.

E.  Blood flow is diverted toward the skin in hypothyroidism.

X-22. A 38-year-old mother of three presents to her primary care office with complaints of fatigue. She feels that her energy level has been low for the past 3 months. She was previously healthy and was taking no medications. She does report that she has gained about 5 kg and has severe constipation, for which she has been taking a number of laxatives. A TSH is elevated at 25 mU/L. Free T4 is low. She is wondering why she has hypothyroidism. Which of the following tests is most likely to diagnose the etiology?

A.  Antithyroglobulin antibody

B.  Antithyroid peroxidase antibody

C.  Radioiodine uptake scan

D.  Serum thyroglobulin level

E.  Thyroid ultrasound

X-23. A 54-year-old woman with long-standing hypothyroidism is seen in her primary care physician’s office for a routine evaluation. She reports feeling fatigued and somewhat constipated. Since her last visit, her other medical conditions, which include hypercholesterolemia and systemic hypertension, are stable. She was diagnosed with uterine fibroids and started on iron recently. Her other medications include levothyroxine, atorvastatin, and hydrochlorothiazide. A TSH is checked and it is elevated to 15 mU/L. Which of the following is the most likely reason for her elevated TSH?

A.  Celiac disease

B.  Colon cancer

C.  Medication noncompliance

D.  Poor absorption of levothyroxine due to ferrous sulfate

E.  TSH-secreting pituitary adenoma

X-24. An 87-year-old woman is admitted to the intensive care unit with depressed level of consciousness, hypothermia, sinus bradycardia, hypotension, and hypoglycemia. She was previously healthy with the exception of hypothyroidism and systemic hypertension. Her family recently checked in on her and found that she was not taking any of her medications because of financial difficulties. There is no evidence of infection on exam, urine microscopy, or chest radiograph. Her serum chemistries are notable for mild hyponatremia and a glucose of 48. TSH is above 100 mU/L. All of the following statements regarding this condition are true EXCEPT:

A.  External warming is a critical feature of therapy in patients with a temperature above 34°C (93.2°F).

B.  Hypotonic intravenous solutions should be avoided.

C.  IV levothyroxine should be administered with IV glucocorticoids.

D.  Sedation should be avoided if possible.

E.  This condition occurs almost exclusively in the elderly and often is precipitated by an unrelated medical illness.

X-25. A 29-year-old woman is evaluated for anxiety, palpitations, and diarrhea and is found to have Graves’ disease. Before she begins therapy for her thyroid condition, she has an episode of acute chest pain and presents to the emergency department. Although a CT angiogram is ordered, the radiologist calls to notify the treating physician that this is potentially dangerous. Which of the following best explains the radiologist’s recommendation?

A.  Iodinated contrast exposure in patients with Graves’ disease may exacerbate hyperthyroidism.

B.  Pulmonary embolism is exceedingly rare in Graves’ disease.

C.  Radiation exposure in patients with hyper-thyroidism is associated with increased risk of subsequent malignancy.

D.  Tachycardia with Graves’ disease limits the image quality of CT angiography and will not allow accurate assessment of pulmonary embolism.

E.  The radiologist was mistaken; CT angiography is safe in Graves’ disease.

X-26. What percentage of patients with hyperthyroidism and atrial fibrillation convert to sinus rhythm after treatment of thyroid state alone?

A.  20%

B.  30%

C.  50%

D.  70%

E.  90%

X-27. Which of the following statements best describes Graves’ ophthalmopathy?

A.  Although a cosmetic problem, Graves’ ophthalmopathy is rarely associated with major ocular complications.

B.  Diplopia may occur from periorbital muscle swelling.

C.  It is never found without concomitant hyperthyroidism.

D.  The most serious complication is corneal abrasion.

E.  Unilateral disease is not found.

X-28. Which of the following is the most important mechanism of action of propylthiouracil in the treatment of Graves’ disease?

A.  Impaired production of transthyretin

B.  Inhibition of production of thyroid-stimulating immunoglobulins

C.  Inhibition of the function of thyroid peroxidase

D.  Reduced peripheral conversion of T4 to T3

E.  Reversal of iodine organification

X-29. A 44-year-old male is involved in a motor vehicle collision. He sustains multiple injuries to the face, chest, and pelvis. He is unresponsive in the field and is intubated for airway protection. An intravenous line is placed. The patient is admitted to the intensive care unit (ICU) with multiple orthopedic injuries. He is stabilized medically and on hospital day 2 undergoes successful open reduction and internal fixation of the right femur and right humerus. After his return to the ICU, you review his laboratory values. TSH is 0.3 mU/L, and the total T4 level is normal. T3 is 0.6 μg/dL. What is the most appropriate next management step?

A.  Initiation of levothyroxine

B.  Initiation of prednisone

C.  Observation

D.  Radioiodine uptake scan

E.  Thyroid ultrasound

X-30. A 29-year-old woman presents to your clinic complaining of difficulty swallowing, sore throat, and tender swelling in her neck. She has also noted fevers intermittently over the past week. Several weeks prior to her current symptoms she experienced symptoms of an upper respiratory tract infection. She has no past medical history. On physical examination, she is noted to have a small goiter that is painful to the touch. Her oropharynx is clear. Laboratory studies are sent and reveal a white blood cell count of 14,100 cells/μL with a normal differential, erythrocyte sedimentation rate (ESR) of 53 mm/h, and a thyroid-stimulating hormone (TSH) of 21 μIU/mL. Thyroid antibodies are negative. What is the most likely diagnosis?

A.  Autoimmune hypothyroidism

B.  Cat-scratch fever

C.  Graves’ disease

D.  Ludwig’s angina

E.  Subacute thyroiditis

X-31. What is the most appropriate treatment for the patient described in question X-30?

A.  Iodine ablation of the thyroid

B.  Large doses of aspirin

C.  Local radiation therapy

D.  No treatment necessary

E.  Propylthiouracil

X-32. Which of the following is consistent with a diagnosis of subacute thyroiditis?

A.  A 38-year-old female with a 2-week history of a painful thyroid, elevated T4, elevated T3, low TSH, and an elevated radioactive iodine uptake scan

B.  A 42-year-old male with a history of a painful thyroid 4 months ago, fatigue, malaise, low free T4, low T3, and elevated TSH

C.  A 31-year-old female with a painless enlarged thyroid, low TSH, elevated T4, elevated free T4, and an elevated radioiodine uptake scan

D.  A 50-year-old male with a painful thyroid, slightly elevated T4, normal TSH, and an ultrasound showing a mass

X-33. A healthy 53-year-old man comes to your office for an annual physical examination. He has no complaints and has no significant medical history. He is taking an over-the-counter multivitamin and no other medicines. On physical examination he is noted to have a nontender thyroid nodule. His thyroid-stimulating hormone (TSH) level is checked and is found to be low. What is the next step in his evaluation?

A.  Close follow-up and measure TSH in 6 months

B.  Fine-needle aspiration

C.  Low-dose thyroid replacement

D.  Positron emission tomography followed by surgery

E.  Radionuclide thyroid scan

X-34. A patient has neurosurgery for a pituitary tumor that requires resection of the gland. Which of the following functions of the adrenal gland will be preserved in this patient immediately postoperatively?

A.  Morning peak of plasma cortisol level

B.  Release of cortisol in response to stress

C.  Sodium retention in response to hypovolemia

D.  None of the above

X-35. Which of the following is the most common cause of Cushing’s syndrome?

A.  ACTH-producing pituitary adenoma

B.  Adrenocortical adenoma

C.  Adrenocortical carcinoma

D.  Ectopic ACTH secretion

E.  McCune-Albright syndrome

X-36. All of the following are features of Conn’s syndrome EXCEPT:

A.  Alkalosis

B.  Hyperkalemia

C.  Muscle cramps

D.  Normal serum sodium

E.  Severe systemic hypertension

X-37. All of the following statements regarding asymptomatic adrenal masses (incidentalomas) are true EXCEPT:

A.  All patients with incidentalomas should be screened for pheochromocytoma.

B.  Fine-needle aspiration may distinguish between benign and malignant primary adrenal tumors.

C.  In patients with a history of malignancy, the likelihood that the mass is a metastasis is approximately 50%.

D.  The majority of adrenal incidentalomas are non-secretory.

E.  The vast majority of adrenal incidentalomas are benign.

X-38. A 43-year-old man with episodic, severe hypertension is referred for evaluation of possible secondary causes of hypertension. He reports feeling well generally, except for episodes of anxiety, palpitations, and tachycardia with elevation in his blood pressure during these episodes. Exercise often brings on these events. The patient also has mild depression and is presently taking sertraline, labetalol, amlodipine, and lisinopril to control his blood pressure. Urine 24-hour total metanephrines are ordered and show an elevation of 1.5 times the upper limit of normal. Which of the following is the next most appropriate step?

A.  Hold labetalol for 1 week and repeat testing.

B.  Hold sertraline for 1 week and repeat testing.

C.  Immediately refer for surgical evaluation.

D.  Measure 24-hour urine vanillymandelic acid level.

E.  Send for MRI of the abdomen.

X-39. A 45-year-old man is diagnosed with pheochromocytoma after presentation with confusion, marked hypertension to 250/140 mmHg, tachycardia, headaches, and flushing. His fractionated plasma metanephrines show a normetanephrine level of 560 pg/mL and a metanephrine level of 198 pg/mL (normal values: normetanephrine: 18–111 pg/mL; metanephrine: 12–60 pg/mL). CT scanning of the abdomen with IV contrast demonstrates a 3-cm mass in the right adrenal gland. A brain MRI with gadolinium shows edema of the white matter near the parietooccipital junction consistent with reversible posterior leukoencephalopathy. You are asked to consult regarding management. Which of the following statements is true regarding management of pheochromocytoma is this individual?

A.  Beta-blockade is absolutely contraindicated for tachycardia even after adequate alpha-blockade has been attained.

B.  Immediate surgical removal of the mass is indicated, because the patient presented with hypertensive crisis with encephalopathy.

C.  Salt and fluid intake should be restricted to prevent further exacerbation of the patient’s hypertension.

D.  Treatment with phenoxybenzamine should be started at a high dose (20–30 mg three times daily) to rapidly control blood pressure, and surgery can be undertaken within 24–48 hours.

E.  Treatment with IV phentolamine is indicated for treatment of the hypertensive crisis. Phenoxybenzamine should be started at a low dose and titrated to the maximum tolerated dose over 2–3 weeks. Surgery should not be planned until the blood pressure is consistently below 160/100 mmHg.

X-40. Which of the following ethnic populations in the United States has the highest risk of diabetes mellitus?

A.  Asian American

B.  Hispanic

C.  Non-Hispanic black

D.  Non-Hispanic white

X-41. Which of the following defines normal glucose tolerance?

A.  Fasting plasma glucose below 100 mg/dL

B.  Fasting plasma glucose below 126 mg/dL following an oral glucose challenge

C.  Hemoglobin A1C below 5.6% and fasting plasma glucose below 140 mg/dL

D.  Hemoglobin A1C below 6.0%

E.  Fasting plasma glucose below 100 mg/dL, plasma glucose below 140 mg/dL following an oral glucose challenge, and hemoglobin A1C below 5.6%

X-42. A 37-year-old woman with obesity presents to the clinic for a routine health evaluation. She reports that over the last year she has had two yeast infections treated with over-the-counter remedies and frequently feels thirsty. She does report waking up at night to urinate. Which of the following studies is the most appropriate first test in evaluating the patient for diabetes mellitus?

A.  Hemoglobin A1C

B.  Oral glucose tolerance test

C.  Plasma C-peptide level

D.  Plasma insulin level

E.  Random plasma glucose level

X-43. All of the following are risk factors for type 2 diabetes mellitus EXCEPT:

A.  BMI above 25 kg/m2

B.  Delivery of a baby more than 3.5 kg

C.  HDL level below 35 mg/dL

D.  Hemoglobin A1C 5.7–6.4%

E.  Systemic hypertension

X-44. A 27-year-old woman with mild obesity is seen in her primary care office for increased thirst and polyuria. Diabetes mellitus is suspected, and a random plasma glucose of 211 mg/d confirms this diagnosis. Which of the following tests will strongly indicate that she has type 1 diabetes mellitus?

A.  Anti–GAD-65 antibody.

B.  Peroxisome proliferator-activated receptor γ-2 polymorphism testing.

C.  Plasma insulin level.

D.  Testing for HLA-DR3.

E.  There is no laboratory test indicating type 1 diabetes mellitus.

X-45. In patients with impaired fasting glucose, all of the following interventions have been proven to decrease progression to type 2 diabetes mellitus EXCEPT:

A.  Diet modification

B.  Exercise

C.  Glyburide

D.  Metformin

X-46. A patient is evaluated in the emergency department for complications of diabetes mellitus with an episode of life stressors. All of the following laboratory tests are consistent with the diagnosis of diabetic ketoacidosis EXCEPT:

A.  Arterial pH 7.1

B.  Glucose 550 mg/dL

C.  Markedly positive plasma ketones

D.  Normal serum potassium

E.  Plasma osmolality 380 mosm/mL

X-47. All of the following are consistent with nonproliferative diabetic retinopathy EXCEPT:

A.  Blot hemorrhages

B.  Cotton-wool spots

C.  Neovascularization

D.  Occurs in first or second decade of diabetes mellitus

E.  Retinal vascular microaneurysms

X-48. A 68-year-old man with poorly controlled type 2 diabetes mellitus is admitted to the hospital with an ulcer on the lateral surface of his right lower extremity that has been painful and appears purulent. He has had 3 days of fever. All of the following interventions are recommended to improve wound healing in a patient with a diabetic wound EXCEPT:

A.  Appropriate use of antibiotics

B.  Debridement

C.  Hyperbaric oxygen

D.  Off-loading

E.  Revascularization

X-49. Pick the correct combination of onset of action and duration of action for the following insulins:

A.  Aspart: 1 hour, 6 hours

B.  Detemir: 2 hours, 12 hours

C.  Lispro: 0.5 hour, 2 hours

D.  NPH: 2 hours, 14 hours

E.  Regular: 0.25 hour, 6 hours

X-50. A 54-year-old woman is diagnosed with type 2 diabetes mellitus after a routine follow-up for impaired fasting glucose showed that her hemoglobin A1C is now 7.6%. She has attempted to lose weight and to exercise with no improvement in her hemoglobin A1C, and drug therapy is now recommended. She has mild systemic hypertension that is well controlled and no other medical conditions. Which of the following is the most appropriate first-line therapy?

A.  Acarbose

B.  Exenatide

C.  Glyburide

D.  Metformin

E.  Sitagliptin

X-51. The Diabetes Control and Complications Trial (DCCT) provided definitive proof that reduction in chronic hyperglycemia:

A.  Improves microvascular complications in type 1 diabetes mellitus

B.  Improves macrovascular complications in type 1 diabetes mellitus

C.  Improves microvascular complications in type 2 diabetes mellitus

D.  Improves macrovascular complications in type 2 diabetes mellitus

E.  Improves both microvascular and macrovascular complications in type 2 diabetes mellitus

X-52. A patient is seen in the clinic for follow-up of type 2 diabetes mellitus. Her hemoglobin A1C has been poorly controlled at 9.4% recently. The patient can be counseled to expect all the following improvements with improved glycemic control EXCEPT:

A.  Decreased microalbuminuria

B.  Decreased risk of nephropathy

C.  Decreased risk of neuropathy

D.  Decreased risk of peripheral vascular disease

E.  Decreased risk of retinopathy

X-53. A 21-year-old female with a history of type 1 diabetes mellitus is brought to the emergency department with nausea, vomiting, lethargy, and dehydration. Her mother notes that she stopped taking insulin 1 day before presentation. She is lethargic, has dry mucous membranes, and is obtunded. Blood pressure is 80/40 mmHg, and heart rate is 112 beats/min. Heart sounds are normal. Lungs are clear. The abdomen is soft, and there is no organomegaly. She is responsive and oriented × 3 but diffusely weak. Serum sodium is 126 meq/L, potassium is 4.3 meq/L, magnesium is 1.2 meq/L, blood urea nitrogen is 76 mg/dL, creatinine is 2.2 mg/dL, bicarbonate is 10 meq/L, and chloride is 88 meq/L. Serum glucose is 720 mg/dL. All of the following are appropriate management steps EXCEPT:

A.  3% sodium solution

B.  Arterial blood gas

C.  Intravenous insulin

D.  Intravenous potassium

E.  Intravenous fluids

X-54. Which of the following studies is the most sensitive for detecting diabetic nephropathy?

A.  Creatinine clearance

B.  Glucose tolerance test

C.  Serum creatinine level

D.  Ultrasonography

E.  Urine albumin

X-55. Alteration in which of the following substance levels is the first defense against hypoglycemia?

A.  Cortisol

B.  Epinephrine

C.  Glucagon

D.  Insulin

E.  Insulin-like growth factor

X-56. A 25-year-old health care worker is seen for evaluation of recurrent hypoglycemia. She has had several episodes at work over the past year in which she feels shaky, anxious, and sweaty, and when she measures her finger stick glucose, it is 40–55 mg/dL. She drinks orange juice and feels better. These episodes have not happened outside the work environment. Aside from oral contraceptives, she takes no medications and is otherwise healthy. Which of the following tests is most likely to demonstrate the underlying cause of her hypoglycemia?

A.  Measurement of insulin-like growth factor 1

B.  Measurement of fasting insulin and glucose levels

C.  Measurement of fasting insulin, glucose, and C-peptide levels

D.  Measurement of insulin, glucose, and C-peptide levels during a symptomatic episode

E.  Measurement of plasma cortisol

X-57. All of the following statements regarding hypoglycemia in diabetes mellitus are true EXCEPT:

A.  Individuals with type 2 diabetes mellitus experience less hypoglycemia than those with type 1 diabetes mellitus.

B.  From 2–4% of deaths in type 1 diabetes mellitus are directly attributable to hypoglycemia.

C.  Recurrent episodes of hypoglycemia predispose to the development of autonomic failure with defective glucose counterregulation and hypoglycemia unawareness.

D.  The average person with type 1 diabetes mellitus has two episodes of symptomatic hypoglycemia weekly.

E.  Thiazolidinediones and metformin cause hypoglycemia more frequently than sulfonylureas.

X-58. A 58-year-old man is seen in his primary care physician’s office for evaluation of bilateral breast enlargement. This has been present for several months and is accompanied by mild pain in both breasts. He reports no other symptoms. His other medical conditions include coronary artery disease with a history of congestive heart failure, atrial fibrillation, obesity, and type 2 diabetes mellitus. His current medications include lisinopril, spironolactone, furosemide, insulin, and digoxin. He denies illicit drug use and has fathered three children. Examination confirms bilateral breast enlargement with palpable glandular tissue that measures 2 cm bilaterally. Which of the following statements regarding his gynecomastia is true?

A.  He should be referred for mammography to rule out breast cancer.

B.  His gynecomastia is most likely due to obesity with adipose tissue present in the breast.

C.  Serum testosterone, LH, and FSH should be measured to evaluate for androgen insensitivity.

D.  Spironolactone should be discontinued and exam followed for regression.

E.  Liver function testing should be performed to screen for cirrhosis.

X-59. All the following drugs may interfere with testicular function EXCEPT:

A.  Cyclophosphamide

B.  Ketoconazole

C.  Metoprolol

D.  Prednisone

E.  Spironolactone

X-60. Clinical signs and findings of the presence of ovulation include all of the following EXCEPT:

A.  Detection of urinary LH surge

B.  Estrogen peak during secretory phase of menstrual cycle

C.  Increase in basal body temperature more than 0.5°F in the second half of the menstrual cycle

D.  Presence of mittelschmerz

E.  Progesterone level above 5 ng/mL 7 days before expected menses

X-61. A couple has been married for 5 years and has attempted to conceive a child for the last 12 months. Despite regular intercourse they have not achieved pregnancy. They are both 32 years of age and have no medical problems. Neither partner is taking medications. Which of the following is the most likely cause of their infertility?

A.  Endometriosis

B.  Male causes

C.  Ovulatory dysfunction

D.  Tubal defect

E.  Unexplained

X-62. A couple seeks advice regarding infertility. The female partner is 35 years old. She has never been pregnant and took oral contraceptive pills from age 20 until age 34. It is now 16 months since she discontinued her oral contraceptives. She is having menstrual cycles approximately once every 35 days, but occasionally will go as long as 60 days between cycles. Most months, she develops breast tenderness about 2–3 weeks after the start of her menstrual cycle. When she was in college, she was treated for Neisseria gonorrhoeae that was diagnosed when she presented to the student health center with a fever and pelvic pain. She otherwise has no medical history. She works about 60 hours weekly as a corporate attorney and exercises daily. She drinks coffee daily and alcohol on social occasions only. Her body mass index (BMI) is 19.8 kg/m2. Her husband, who is 39 years old, accompanies her to the evaluation. He also has never had children. He was married previously from the ages of 24–28. He and his prior wife attempted to conceive for about 15 months, but were unsuccessful. At that time, he was smoking marijuana on a daily basis and attributed their lack of success to his drug use. He has now been completely free of drugs for 9 years. He suffers from hypertension and is treated with lisinopril 10 mg daily. He is not obese (BMI, 23.7 kg/m2). They request evaluation for their infertility and help with conception. Which of the following statements is true in regard to their infertility and likelihood of success in conception?

A.  Determination of ovulation is not necessary in the female partner as most of her cycles occur regularly, and she develops breast tenderness midcycle, which is indicative of ovulation.

B.  Lisinopril should be discontinued immediately because of the risk of birth defects associated with its use.

C.  The female partner should be assessed for tubal patency by a hysterosalpingogram. If significant scarring is found, in vitro fertilization should be strongly considered to decrease the risk of ectopic pregnancy.

D.  The prolonged use of oral contraceptives for more than 10 years has increased the risk of anovulation and infertility.

E.  The use of marijuana by the male partner is directly toxic to sperm motility, and this is the likely cause of their infertility.

X-63. Which of the following forms of contraception have theoretical efficacy of more than 90%?

A.  Condoms

B.  Intrauterine devices

C.  Oral contraceptives

D.  Spermicides

E.  All of the above

X-64. A 30-year-old male, the father of three children, has had progressive breast enlargement during the last 6 months. He does not use any drugs. Laboratory evaluation reveals that both LH and testosterone are low. Further evaluation of this patient should include which of the following?

A.  24-hour urine collection for the measurement of 17 ketosteroids

B.  Blood sampling for serum glutamic-oxaloacetic transaminase (SGOT) and serum alkaline phosphatase and bilirubin levels

C.  Breast biopsy

D.  Karyotype analysis to exclude Klinefelter’s syndrome

E.  Measurement of estradiol and human chorionic gonadotropin (hCG) levels

X-65. The Women’s Health Initiative study investigated hormonal therapy in postmenopausal women. The study was stopped early due to increased risk of which of the following diseases in the estrogen-only arm?

A.  Deep venous thrombosis

B.  Endometrial cancer

C.  Myocardial infarction

D.  Osteoporosis

E.  Stroke

X-66. A 37-year-old man is evaluated for infertility. He and his wife have been attempting to conceive a child for the past 2 years without success. He initially saw an infertility specialist, but was referred to endocrinology after sperm analysis showed no sperm. He is otherwise healthy and only takes a multivitamin. On physical examination his vital signs are normal. He is tall and has small testes, gynecomastia, and minimal facial and axillary hair. Chromosomal analysis confirms Klinefelter’s syndrome. Which of the following statements is true?

A.  Androgen supplementation is of little use in this condition.

B.  He is not at increased risk for breast tumors.

C.  Increased plasma concentrations of estrogen are present.

D.  Most cases are diagnosed prepuberty.

E.  Plasma concentrations of FSH and LH are decreased in this condition.

X-67. A 17-year-old woman is evaluated in your office for primary amenorrhea. She feels as if she has not entered puberty in that she has never had a menstrual period and has sparse axillary and pubic hair growth. On examination, she is noted to be 150 cm tall. She has a low hairline and slight webbing of her neck. Her follicle-stimulating hormone level is 75 mIU/mL, luteinizing hormone is 20 mIU/mL, and estradiol level is 2 pg/mL. You suspect Turner’s syndrome. All of the following tests are indicated in this individual EXCEPT:

A.  Buccal smear for nuclear heterochromatin (Barr body)

B.  Echocardiogram

C.  Karyotype analysis

D.  Renal ultrasound

E.  Thyroid-stimulating hormone (TSH)

X-68. A 35-year-old man is seen in the emergency department for evaluation of epigastric pain, diarrhea, and reflux. He reports frequent similar episodes and has undergone multiple endoscopies. In each case he was told that he has a duodenal ulcer. He has become quite frustrated because he was told that ulcers are usually due to a bacteria that can be treated, but he does not have Helicobacter pylori present on any of his ulcer biopsies. His current medications are high-dose omeprazole and oxycodone/acetaminophen. He is admitted to the hospital for pain control. Which of the following is the most appropriate next step in his diagnostic evaluation?

A.  CT scan of the abdomen.

B.  Discontinue omeprazole for 1 week and measure plasma gastrin level.

C.  Gastric pH measurement.

D.  Plasma gastrin level.

E.  Screen for parathyroid hyperplasia.

X-69. A 48-year-old female is undergoing evaluation for flushing and diarrhea. Physical examination is normal except for nodular hepatomegaly. A CT scan of the abdomen demonstrates multiple nodules in both lobes of the liver consistent with metastases in the liver and a 2-cm mass in the ileum. The 24-hour urinary 5-HIAA excretion is markedly elevated. All the following treatments are appropriate EXCEPT:

A.  Diphenhydramine

B.  Interferon α

C.  Octreotide

D.  Ondansetron

E.  Phenoxybenzamine

X-70. While undergoing a physical examination during medical student clinical skills, the patient in question X-69 develops severe flushing, wheezing, nausea, and lightheadedness. Vital signs are notable for a blood pressure of 70/30 mmHg and a heart rate of 135 beats/min. Which of the following is the most appropriate therapy?

A.  Albuterol

B.  Atropine

C.  Epinephrine

D.  Hydrocortisone

E.  Octreotide

X-71. A 49-year-old male is brought to the hospital by his family because of confusion and dehydration. The family reports that for the last 3 weeks he has had persistent copious, watery diarrhea that has not abated with the use of over-the-counter medications. The diarrhea has been unrelated to food intake and has persisted during fasting. The stool does not appear fatty and is not malodorous. The patient works as an attorney, is a vegetarian, and has not traveled recently. No one in the household has had similar symptoms. Before the onset of diarrhea, he had mild anorexia and a 5-lb weight loss. Since the diarrhea began, he has lost at least 5 kg. The physical examination is notable for blood pressure of 100/70 mmHg, heart rate of 110 beats/min, and temperature of 36.8°C (98.2°F). Other than poor skin turgor, confusion, and diffuse muscle weakness, the physical examination is unremarkable. Laboratory studies are notable for a normal complete blood count and the following chemistry results:

image

A 24-hour stool collection yields 3 L of tea-colored stool. Stool sodium is 50 meq/L, potassium is 25 meq/L, and stool osmolality is 170 mosmol/L. Which of the following diagnostic tests is most likely to yield the correct diagnosis?

A.  Serum cortisol

B.  Serum TSH

C.  Serum VIP

D.  Urinary 5-HIAA

E.  Urinary metanephrine

X-72. An 18-year-old girl is evaluated at her primary care physician’s office for a routine physical. She is presently healthy. Her family history is notable for a father and two aunts with MEN 1, and the patient has undergone genetic testing and carries the MEN 1 gene. Which of the following is the first and most common presentation for individuals with this genetic mutation?

A.  Peptic ulcer disease

B.  Hypercalcemia

C.  Hypoglycemia

D.  Amenorrhea

E.  Uncontrolled systemic hypertension

X-73. A 35-year-old male is referred to your clinic for evaluation of hypercalcemia noted during a health insurance medical screening. He has noted some fatigue, malaise, and a 4-lb weight loss over the last 2 months. He also has noted constipation and “heartburn.” He is occasionally nauseated after large meals and has water brash and a sour taste in his mouth. The patient denies vomiting, dysphagia, or odynophagia. He also notes decreased libido and a depressed mood. Vital signs are unremarkable. Physical examination is notable for a clear oropharynx, no evidence of a thyroid mass, and no lymphadenopathy. Jugular venous pressure is normal. Heart sounds are regular with no murmurs or gallops. The chest is clear. The abdomen is soft with some mild epigastric tenderness. There is no rebound or organomegaly. Stool is guaiac positive. Neurologic examination is nonfocal. Laboratory values are notable for a normal complete blood count. Calcium is 11.2 mg/dL, phosphate is 2.1 mg/dL, and magnesium is 1.8 meq/dL. Albumin is 3.7 g/dL, and total protein is 7.0 g/dL. TSH is 3 μIU/mL, prolactin is 250 μg/L, testosterone is 620 ng/dL, and serum insulin-like growth factor 1 (IGF-1) is normal. Serum intact parathyroid hormone level is 135 pg/dL. In light of the patient’s abdominal discomfort and heme-positive stool, you perform an abdominal computed tomography (CT) scan that shows a lesion measuring 2 × 2 cm in the head of the pancreas. What is the diagnosis?

A.  Multiple endocrine neoplasia (MEN) type 1

B.  MEN type 2a

C.  MEN type 2b

D.  Polyglandular autoimmune syndrome

E.  Von-Hippel Lindau (VHL) syndrome

X-74. A 55-year-old male is admitted to the intensive care unit with fever and cough. He was well until 1 week before admission, when he noted progressive shortness of breath, cough, and productive sputum. On the day of admission the patient’s wife noted him to be lethargic. Emergency response found the patient unresponsive. He was intubated in the field and brought to the emergency department. His medications include insulin. The past medical history is notable for alcohol abuse and diabetes mellitus. Temperature is 38.9°C (102°F), blood pressure is 76/40 mmHg, and oxygen saturation is 86% on room air. On examination, the patient is intubated on mechanical ventilation. Jugular venous pressure is normal. There are decreased breath sounds at the right lung base with egophony. Heart sounds are normal. The abdomen is soft. There is no peripheral edema. Chest radiography shows a right lower lobe infiltrate with a moderate pleural effusion. An electrocardiogram is normal. Sputum Gram stain shows gram-positive diplococci. White blood cell count is 23 × 103/μL, with 70% polymorphonuclear cells and 6% bands. Blood urea nitrogen is 80 mg/dL, and creatinine is 3.1 mg/dL. Plasma glucose is 425 mg/dL. He is started on broad-spectrum antibiotics, intravenous fluids, omeprazole, and an insulin drip. A nasogastric tube is inserted, and tube feedings are started. On hospital day 2, his creatinine improves to 1.6 mg/dL. However, plasma phosphate is 1.0 mg/dL (0.3 mmol/L) and calcium is 8.8 mg/dL. All of following are causes of hypophosphatemia in this patient EXCEPT:

A.  Alcoholism

B.  Insulin

C.  Malnutrition

D.  Renal failure

E.  Sepsis

X-75. In the patient in question X-74, what is the most appropriate approach to correcting the hypophosphatemia?

A.  Administer IV calcium gluconate 1 g followed by infusion of IV phosphate at a rate of 8 mmol/h for 6 hours.

B.  Administer IV phosphate alone at a rate of 2 mmol/h for 6 hours.

C.  Administer IV phosphate alone at a rate of 8 mmol/h for 6 hours.

D.  Continue close observation as redistribution of phosphate is expected to normalize over the course of the next 24–48 hours.

E.  Initiate oral phosphate replacement at a dose of 1500 mg/d.

X-76. A 35-year-old woman is admitted to the hospital at 37 weeks’ gestation following a seizure associated with an elevated blood pressure to 190/96 mmHg. She is treated acutely with magnesium sulfate intravenously for eclampsia and is starting on a continuous magnesium sulfate infusion at 1 g/h, which will be continued for 24 hours following her seizure. An emergency caesarian section is planned. Serum magnesium levels will be measured every 6 hours. What magnesium level would be worrisome for the development of central nervous system depression, respiratory muscle paralysis, and cardiac arrhythmias?

A.  0.5 mmol/L

B.  1.0 mmol/L

C.  2.5 mmol/L

D.  3.0 mmol/L

E.  5.0 mmol/L

X-77. You are caring for a 72-year-old man who has been living in a nursing home for the past 3 years. He has severe chronic obstructive pulmonary disease and requires continuous oxygen at 3 L/min. He also previously had a stroke, which has left him with a right hemiparesis. His current medications include aspirin, losartan, hydrochlorothiazide, fluticasone/salmeterol, tiotropium, and albuterol. His body mass index is 18.5 kg/m2. You are concerned that he may have vitamin D deficiency. Which of the following is the best test to determine if vitamin D deficiency is present?

A.  1,25-hydroxy vitamin D

B.  25-hydroxy vitamin D

C.  Alkaline phosphatase

D.  Parathyroid hormone

E.  Serum total and ionized calcium levels

X-78. A 42-year-old man presents to the emergency department with acute-onset right-sided flank pain. He describes the pain as 10 out of 10 in severity radiating to the groin. He has had one episode of hematuria. A noncontrast CT scan confirms the presence of a right-sided renal stone that is currently located in the distal ureter. He has a past medical history of pulmonary sarcoidosis that is not currently treated. This was diagnosed by bronchoscopic biopsy showing noncaseating granulomas. His chest radiograph shows bilateral hilar adenopathy. His serum calcium level is 12.6 mg/dL. What is the mechanism of hypercalcemia in this patient?

A.  Increased activation of 25-hydroxy vitamin D to 1,25-hydroxy vitamin D by macrophages within granulomas

B.  Increased activation of 25-hydroxy vitamin D to 1,25-hydroxy vitamin D by the kidney

C.  Increased activation of vitamin D to 25-hydroxy vitamin D by macrophages within granulomas

D.  Missed diagnosis of lymphoma with subsequent bone marrow invasion and resorption of bone through local destruction

E.  Production of parathyroid hormone–related peptide by macrophages within granulomas

X-79. A 52-year-old man has end-stage kidney disease from long-standing hypertension and diabetes mellitus. He has been managed with hemodialysis for the past 8 years. Throughout this time, he has been poorly compliant with his medications and hemodialysis schedule, frequently missing one session weekly. He is now complaining of bone pain and dyspnea. His oxygen saturation is noted to be 92% on room air, and his chest radiograph shows hazy bilateral infiltrates. Chest CT shows ground-glass infiltrates bilaterally. His laboratory data include calcium of 12.3 mg/dL, phosphate of 8.1 mg/dL, and parathyroid hormone of 110 pg/mL. Which of the following would be the best approach to the treatment of the patient’s current clinical condition?

A.  Calcitriol 0.5 μg intravenously with hemodialysis with sevelamer three times daily

B.  Calcitriol 0.5 μg orally daily with sevelamer 1600 mg three times daily

C.  More aggressive hemodialysis to achieve optimal fluid and electrolyte balance

D.  Parathyroidectomy

E.  Sevelamer 1600 mg three times daily

X-80. A 54-year-old woman undergoes total thyroidectomy for follicular carcinoma of the thyroid. About 6 hours after surgery, the patient complains of tingling around her mouth. She subsequently develops a pins-and-needles sensation in the fingers and toes. The nurse calls the physician to the bedside to evaluate the patient after she has severe hand cramps when her blood pressure is taken. Upon evaluation, the patient is still complaining of intermittent cramping of her hands. Since surgery, she has received morphine sulfate 2 mg for pain and Compazine 5 mg for nausea. She has had no change in her vital signs and is afebrile. Tapping on the inferior portion of the zygomatic arch 2 cm anterior to the ear produces twitching at the corner of the mouth. An electrocardiogram (ECG) shows a QT interval of 575 milliseconds. What is the next step in the evaluation and treatment of this patient?

A.  Administration of benztropine 2 mg IV

B.  Administration of calcium gluconate 2 g IV

C.  Administration of magnesium sulphate 4 g IV

D.  Measurement of calcium, magnesium, phosphate, and potassium levels

E.  Measurement of forced vital capacity

X-81. A 68-year-old woman with stage IIIB squamous cell carcinoma of the lung is admitted to the hospital because of altered mental status and dehydration. Upon admission, she is found to have a calcium level of 19.6 mg/dL and phosphate of 1.8 mg/dL. Concomitant measurement of parathyroid hormone was 0.1 pg/mL (normal 10–65 pg/mL), and a screen for parathyroid hormone–related peptide was positive. Over the first 24 hours, the patient receives 4 L of normal saline with furosemide diuresis. The next morning, the patient’s calcium is 17.6 mg/dL and phosphate is 2.2 mg/dL. She continues to have delirium. What is the best approach for ongoing treatment of this patient’s hypercalcemia?

A.  Continue therapy with large-volume fluid administration and forced diuresis with furosemide.

B.  Continue therapy with large-volume fluid administration, but stop furosemide and treat with hydrochlorothiazide.

C.  Initiate therapy with calcitonin alone.

D.  Initiate therapy with pamidronate alone.

E.  Initiate therapy with calcitonin and pamidronate.

X-82. A 60-year-old woman is referred to your office for evaluation of hypercalcemia of 12.9 mg/dL. This was found incidentally on a chemistry panel that was drawn during a hospitalization for cervical spondylosis. Despite fluid administration in the hospital, her serum calcium at discharge was 11.8 mg/dL. The patient is asymptomatic. She is otherwise in good health and has had her recommended age-appropriate cancer screening. She denies constipation or bone pain and is now 8 weeks out from her spinal surgery. Today, her serum calcium level is 12.4 mg/dL, and phosphate is 2.3 mg/dL. Her hematocrit and all other chemistries including creatinine were normal. What is the most likely diagnosis?

A.  Breast cancer

B.  Hyperparathyroidism

C.  Hyperthyroidism

D.  Multiple myeloma

E.  Vitamin D intoxication

X-83. All of the following are actions of parathyroid hormone EXCEPT:

A.  Direct stimulation of osteoblasts to increase bone formation

B.  Direct stimulation of osteoclasts to increase bone resorption

C.  Increased reabsorption of calcium from the distal tubule of the kidney

D.  Inhibition of phosphate reabsorption in the proximal tubule of the kidney

E.  Stimulation of renal 1-α-hydroxylase to produce 1,25-hydroxycholecalciferol

X-84. Which of the following statements regarding the epidemiology of osteoporosis and bone fractures is correct?

A.  For every 5-year period after age 70, the incidence of hip fractures increases by 25%.

B.  Fractures of the distal radius increase in frequency before age 50 and plateau by age 60 with only a modest age-related increase.

C.  Most women meet the diagnostic criteria for osteoporosis between the ages of 60 and 70.

D.  The risk of hip fracture is equal when white women are compared to black women.

E.  Women outnumber men with osteoporosis at a ratio of about 10 to 1.

X-85. A 50-year-old woman presents to your office to inquire about her risk of fracture related to osteoporosis. She has a positive family history of osteoporosis in her mother, but her mother never experienced any hip or vertebral fractures. The patient herself has also not experienced any fractures. She is white and has a 20 pack-year history of tobacco, quitting 10 years prior. At the age of 37, she had a total hysterectomy with bilateral salpingo-oophorectomy for endometriosis. She is lactose intolerant and does not consume dairy products. She currently takes calcium carbonate 500 mg daily. Her weight is 52 kg. All of the following are risk factors for an osteoporotic fracture in this woman EXCEPT:

A.  Early menopause

B.  Female sex

C.  History of cigarette smoking

D.  Low body weight

E.  Low calcium intake

X-86. All of the following diseases are associated with an increased risk of osteoporosis EXCEPT:

A.  Anorexia nervosa

B.  Chronic obstructive pulmonary disease

C.  Congestive heart failure

D.  Malabsorption syndromes

E.  Hyperparathyroidism

X-87. A 54-year-old woman is referred to the endocrinology clinic for evaluation of osteoporosis after a recent examination for back pain revealed a compression fracture of the T4 vertebral body. She is perimenopausal with irregular menstrual periods and frequent hot flashes. She does not smoke. She otherwise is well and healthy. Her weight is 70 kg and height is 168 cm. She has lost 5 cm from her maximum height. A bone mineral density scan shows a T-score of –3.5 SD and a Z-score of –2.5 SD. All of the following tests are indicated for the evaluation of osteoporosis in this patient EXCEPT:

A.  24-hour urine calcium

B.  Follicle-stimulating hormone and luteinizing hormone levels

C.  Serum calcium

D.  Thyroid-stimulating hormone

E.  Vitamin D levels (25-hydroxyvitamin D)

X-88. A 45-year-old white woman seeks advice from her primary care physician regarding her risk for osteoporosis and the need for bone density screening. She is a lifelong nonsmoker and drinks alcohol only socially. She has a history of moderate-persistent asthma since age 12. She is currently on fluticasone, 44 mg/puff twice daily, with good control currently. She last required oral prednisone therapy about 6 months ago when she had influenza that was complicated by an asthma flare. She took prednisone for a total of 14 days. She has had three pregnancies and two live births at ages 39 and 41. She currently has irregular periods occurring approximately every 42 days. Her follicle-stimulating hormone level is 25 mIU/L and 17β-estradiol level is 115 pg/mL on day 12 of her menstrual cycle. Her mother and maternal aunt both have been diagnosed with osteoporosis. Her mother also has rheumatoid arthritis and requires prednisone therapy, 5 mg daily. Her mother developed a compression fracture of the lumbar spine at age 68. On physical examination, the patient appears well and healthy. Her height is 168 cm. Her weight is 66.4 kg. The chest, cardiac, abdominal, muscular, and neurologic examinations are normal. What do you tell the patient about the need for bone density screening?

A.  As she is currently perimenopausal, she should have a bone density screen every other year until she completes menopause and then have bone densitometry measured yearly thereafter.

B.  Because of her family history, she should initiate bone density screening yearly beginning now.

C.  Bone densitometry screening is not recommended until after completion of menopause.

D.  Delayed childbearing until the fourth and fifth decade decreases her risk of developing osteoporosis so bone densitometry is not recommended.

E.  Her use of low-dose inhaled glucocorticoids increases her risk of osteoporosis threefold, and she should undergo yearly bone density screening.

X-89. What is the definition of osteoporosis by dual-energy x-ray absorptiometry testing (bone densitometry)?

A.  A patient with a bone density less than the mean of age-, race-, and gender-matched controls

B.  A patient with a bone density less than 1.0 standard deviation (SD) below the mean of race- and gender-matched controls

C.  A patient with a bone density less than 1.0 SD below the mean of age-, race-, and gender-matched controls

D.  A patient with a bone density less than 2.5 SD below the mean of race- and gender-matched controls

E.  A patient with a bone density less than 2.5 SD below the mean of age-, race-, and gender-matched controls

X-90. A 66-year-old Asian woman seeks treatment for osteoporosis. She fell and fractured her right hip, requiring a surgical intervention 3 months ago. She was told while hospitalized that she had osteoporosis but had not previously been evaluated for this. During the hospitalization, she developed a deep venous thrombosis (DVT) with pulmonary embolus, for which she is currently taking warfarin. She completed menopause at age 52. She is a former smoker, quitting about 6 years ago. She has always been thin, and her current body mass index (BMI) is 19.2 kg/m2. Her laboratory studies show calcium of 8.7 mg/dL, phosphate of 3 mg/dL, creatinine of 0.8 mg/dL, and 25-hydroxyvitamin D levels of 18 ng/mL (normal >30 ng/mL). A dual-energy x-ray absorptiometry scan of bone mineral density has a T-score of –3.0. What is the best initial therapy for this patient?

A.  Calcitonin 200 IU intranasally daily

B.  Calcium carbonate 1200 mg and vitamin D 400 IU daily

C.  Ethinyl estradiol 5 μg and medroxyprogesterone acetate 625 mg daily

D.  Raloxifene 60 mg daily

E.  Risedronate 35 mg once weekly, and calcium carbonate 1200 mg and vitamin D 400 IU daily

X-91. A 52-year-old man is found to have an elevated alkaline phosphatase level during routine blood chemistry testing prior to obtaining life insurance after changing jobs. He has a history of hypertension and hyperlipidemia. He previously had a cholecystectomy for gallstone disease. His current medications include losartan 25 mg daily, hydrochlorothiazide 25 mg daily, and rosuvastatin 20 mg daily. He is physically active and has a body mass index of 25.2 kg/m2. His only complaint is low back pain that has been more severe recently. He has had no further evaluation for his back pain. His physical examination is normal. His liver is 10 cm to percussion. It is palpable with deep inspiration at the right costal margin. It is noted to be smooth. Murphy’s sign is negative. There is no warmth or tenderness to palpation over the vertebral bodies of the lumbosacral spine. Laboratory evaluation reveals an alkaline phosphatase level of 468 U/L, alanine aminotransferase level of 22 U/L, aspartate aminotransferase level of 32 U/L, total bilirubin of 1.0 mg/dL, calcium of 9.4 mg/dL, phosphate 3.2 mg/dL, and γ-glutamyl transferase level of 20 U/L. What is the most likely diagnosis?

A.  Adverse reaction to rosuvastatin

B.  Paget’s disease

C.  Primary biliary cirrhosis

D.  Retained common bile duct stone

E.  Vertebral osteomyelitis

X-92. Which of the following tests is most likely to lead to the diagnosis of the patient in question X-91?

A.  Magnetic resonance cholangiopancreatography

B.  Magnetic resonance imaging of the lumbosacral spine

C.  Plain radiographs of the lumbosacral spine

D.  Right upper quadrant ultrasound

E.  Serum osteocalcin

X-93. Which of the following biochemical tests is most likely to be within the normal range in a healthy, active individual with Paget’s disease?

A.  Serum alkaline phosphatase

B.  Serum C-telopeptide

C.  Serum calcium

D.  Serum N-telopeptide

E.  Serum osteocalcin

X-94. A 67-year-old woman presents to the clinic after a fall on the ice a week ago. She visited the local emergency department immediately after the fall, where hip radiographs were performed and were negative for fracture or dislocation. They did reveal fusion of the sacroiliac joints and coarse trabeculations in the ilium, consistent with Paget’s disease. A comprehensive metabolic panel was also sent at that visit and is remarkable for an alkaline phosphatase of 257 U/L, with normal serum calcium and phosphate levels. She was discharged with analgesics and told to follow up with her primary care doctor for further management of her radiographic findings. She is recovering from her fall and denies any long-standing pain or immobility of her hip joints. She states that her father suffered from a bone disease that caused him headaches and hearing loss near the end of his life. She is very concerned about the radiographs and wants to know what they mean. Which of the following is the best treatment strategy at this point?

A.  Initiate physical therapy and non–weight bearing exercises to strengthen the hip.

B.  Initiate therapy with vitamin D and calcium.

C.  Initiate therapy with an oral bisphosphonate.

D.  Initiate therapy with prednisone 1 mg/kg, tapering over 6 months.

E.  No treatment is required as she is asymptomatic.

X-95. A 32-year-old man is evaluated at a routine clinic visit for coronary risk factors. He is healthy and reports no tobacco use, his systemic blood pressure is normal, and he does not have diabetes. His family history is notable for high cholesterol in his mother and maternal grandparents. Physical examination shows tendon xanthomas. A fasting cholesterol is notable for a low-density lipoprotein cholesterol (LDL-C) of 387 mg/dL. Which of the following is the most likely genetic disorder affecting this individual?

A.  Autosomal dominant hypercholesterolemia

B.  Familial defective apoB-100

C.  Familial hepatic lipase deficiency

D.  Familial hypercholesterolemia

E.  Lipoprotein lipase deficiency

X-96. All of the following are potential causes of elevated LDL EXCEPT:

A.  Anorexia nervosa

B.  Cirrhosis

C.  Hypothyroidism

D.  Nephrotic syndrome

E.  Thiazide diuretics

X-97. A 16-year-old male is brought to your clinic by his parents due to concern about his weight. He has not seen a physician for many years. He states that he has gained weight due to inactivity and that he is less active because of exertional chest pain. He takes no medications. He was adopted and his parents do not know the medical history of his biological parents. Physical examination is notable for stage 1 hypertension and body mass index of 30 kg/m2. He has xanthomas on his hands, heels, and buttocks. Laboratory testing shows a low-density lipo-protein (LDL) of 210 mg/dL, creatinine of 0.7 mg/dL, total bilirubin of 3.1 mg/dL, haptoglobin below 6 mg/dL, and a glycosylated hemoglobin of 6.7%. You suspect a hereditary lipoproteinemia due to the clinical and laboratory findings. Which test would be diagnostic of the primary lipoprotein disorder in this patient?

A.  Congo red staining of xanthoma biopsy

B.  CT scan of the liver

C.  Family pedigree analysis

D.  Gas chromatography

E.  LDL receptor function in skin biopsy

X-98. Your 60-year-old patient with a monoclonal gammopathy of unclear significance presents for a follow-up visit and to review recent laboratory data. His creatinine is newly elevated to 2.0 mg/dL, potassium is 3.7 mg/dL, calcium is 12.2 mg/dL, low-density lipoprotein (LDL) is 202 mg/dL, and triglycerides are 209 mg/dL. On further questioning he reports 3 months of swelling around the eyes and “foamy” urine. On examination, he has anasarca. Concerned for multiple myeloma and nephrotic syndrome, you order a urine protein/creatinine ratio, which returns at 14:1. Which treatment option would be most appropriate to treat his lipid abnormalities?

A.  Cholesterol ester transfer protein inhibitor

B.  Dietary management

C.  HMG-CoA reductase inhibitors

D.  Lipid apheresis

E.  Niacin and fibrates

X-99. A 40-year-old man is evaluated as part of an executive physical examination. He has read about different screening procedures on the Internet and is interested in being screened for hemochromatosis. He is otherwise healthy and takes only a daily multivitamin. Which of the following tests is the most appropriate first step to screen for this disorder?

A.  Genetic testing for C282Y mutation.

B.  HFE activity assay.

C.  Liver MRI.

D.  Screening for hemochromatosis is not cost-effective and not advised.

E.  Transferrin saturation, serum iron, and serum ferritin.

X-100. A 55-year-old white male with a history of diabetes presents to your office with complaints of generalized weakness, weight loss, nonspecific diffuse abdominal pain, and erectile dysfunction. The patient has a past history of hypercholesterolemia and takes atorvastatin. The examination is significant for hepatomegaly without tenderness, testicular atrophy, and gynecomastia. Skin examination shows a diffuse slate-gray hue that is slightly more pronounced on the face and neck. Joint examination shows mild swelling of the second and third metacarpophalangeal joints on the right hand. Which of the following studies is most likely to lead to the correct diagnosis?

A.  Anti–smooth muscle antibody

B.  Ceruloplasmin

C.  Hepatic ultrasound with Doppler imaging

D.  Hepatitis B surface antibody

E.  HFE gene mutation screen

X-101. A 28-year-old man is evaluated for recurrent abdominal pain. He reports that over the last 5 years he has had bouts of severe abdominal pain that is diffuse with distention and not accompanied by vomiting or diarrhea. The pain is not crampy and occurs approximately four to five times per year. One of these episodes was accompanied by hallucinations. He is otherwise healthy, reports engaging in weight lifting, and admits to occasionally using anabolic steroids. During several prior episodes he visited the emergency department and underwent extensive testing including abdominal CT scan with IV and oral contrast. No cause was identified and the symptoms spontaneously resolved after about a day. Which of the following is the next most appropriate step in his evaluation?

A.  Endoscopy and colonoscopy

B.  Measurement of plasma gastrin level during attack

C.  Measurement of urine porphyrobilinogen during attack

D.  Prescription of hyoscyamine

E.  Referral to psychiatry

X-102. A 58-year-old man is evaluated as part of a life insurance physical examination. He reports feeling well and is without complaints. His past medical history is notable for mild hyperlipidemia and an episode of appendicitis several years ago. He exercises about twice a week playing tennis and does not smoke. His only medication is atorvastatin. On physical examination he is mildly obese, but vital signs and the remainder of the examination are normal. Laboratories are drawn and are normal, with the exception of a uric acid level of 12 mg/dL. Which of the following statements regarding this finding is true?

A.  Allopurinol should be prescribed.

B.  Careful evaluation for features of insulin resistance should be undertaken.

C.  He is at an increased risk of uric acid nephrolithiasis.

D.  Most patients with hyperuricemia produce more uric acid than the general population.

E.  Over 10 years, most patients with hyperuricemia will develop gout.

X-103. All of the following are associated with hyperuricemia EXCEPT:

A.  Cardiovascular disease

B.  Gouty arthritis

C.  Nephrolithiasis

D.  Peripheral neuropathy

E.  Urate nephropathy

X-104. A 28-year-old woman seeks counseling before getting pregnant. She had a brother who died at age 9 of Lesch-Nyhan syndrome, and she is a known carrier of the genetic defect. She has no significant past medical history, and her husband has no significant family history. Which of the following statements is true?

A.  Her children have no risk of disease since she is not symptomatic.

B.  Her husband should be screened for carrying the genetic defect of Lesch-Nyhan syndrome.

C.  If she has a daughter, the child has a 50% chance of being a carrier.

D.  If she has an affected son, starting him on allopurinol from birth will prevent clinical manifestations of disease.

E.  She should start taking allopurinol to decrease her risk of gout and urate nephropathy.

X-105. A 28-year-old man is admitted to the intensive care unit with fulminant hepatic failure and hemolysis. On further questioning, his family reports that he has been diagnosed with depression for 5 years and been told that his liver is “damaged.” He is taking an antidepressant and occasional ibuprofen, but no other medications. Physical examination is notable for ascites and altered mental status with dystonia. Abdominal CT scan shows no biliary obstruction, but a cirrhotic liver. Which of the following findings would be most likely to confirm the underlying diagnosis?

A.  24-hour urine level of iron

B.  Brain MRI showing damage to the basal ganglia

C.  Genotype for HFE mutation

D.  Schistocytes on peripheral blood smear

E.  Slit-lamp ocular examination showing Kayser-Fleischer rings

X-106. Which of the following is the most appropriate initial treatment for the patient in question X-105?

A.  Cholestyramine

B.  D-penicillamine

C.  Liver transplantation

D.  Trientine

E.  Zinc

X-107. Which of the following is true of Wilson’s disease?

A.  Early diagnosis is crucial as highly effective therapy is available.

B.  It is inherited in an autosomal dominant pattern.

C.  Serum copper levels are usually two to three times above normal.

D.  The frequency of disease in the general population is approximately 1%.

E.  The liver and pancreas are the most commonly affected organs.

X-108. A 19-year-old girl is evaluated by her primary care physician for recurrent long bone fractures. She has fractured her femur twice and her humerus three times. She has not had an abnormal number of falls and reports also having easy bruising. Aside from these repeated orthopedic injuries, she is otherwise healthy. Physical examination shows mildly disfigured bones; small, amber-yellowish teeth; and bluish-colored sclera. Osteogenesis imperfecta is suspected. Which of the following statements is true regarding this condition?

A.  A mutation in type 1 procollagen likely is present in this patient.

B.  Bone biopsy is needed for definitive diagnosis.

C.  Bisphosphonates have shown long-term success in preventing long bone fractures in this condition.

D.  Fractures in females tend to increase after puberty.

E.  Increased bone mineral density may be demonstrated on x-ray absorptiometry.

X-109. More than 90% of patients with Marfan’s syndrome have mutations in which gene?

A.  BMPR2

B.  COL1A1

C.  Fibrillin

D.  TGFb

E.  Type IV collagen

X-110. A 21-year-old woman comes to the clinic on the advice of her yoga instructor. She recently began classes to increase her activity level and her instructor told her that her joints seemed incredibly flexible, particularly given her inexperience. During the Scorpion exercise, she was able to extend well beyond her classmates. The patient reports good health and a mostly sedentary lifestyle. Throughout her life, she’s been able to perform feats of joint laxity. Her physical examination is notable only for velvety skin and flexible joints. She is able to hyperextend her wrists at least 90°. Which of the following statements regarding this patient is true?

A.  She is at risk of aortic dissection or rupture in the next 20 years.

B.  She is at risk of hip dislocation in the next 20 years.

C.  She is at risk of uterine rupture during pregnancy.

D.  She likely has a mutation in the elastin gene.

E.  She likely has a mutation of the fibrillin gene.