Board Simulation Potpourri Christian Nasr, MD, FACE, FACP Endocrinology & Metabolism Institute Cleveland Clinic Intensive Review of Endocrinology & Metabolism – September 2014 Question 1 1. Which drug does not inhibit cortisol biosynthesis? A. Aminoglutethimide B. Etomidate C. Ketoconazole D. Rifampin E. Metyrapone F. Suramin Question 2 2. Among the following presentations of differentiated thyroid carcinoma, which one is associated with a relatively good prognosis? A. Rapidly expanding neck mass in an 80-year old man B. Metastatic axillary adenopathy C. Diffuse lung uptake on whole body radioiodine scan with normal chest CT in a 20-year old woman D. Metastases visible on FDG-PET but not on radioiodine whole body scan E. Tall-cell variant of thyroid carcinoma Question 3 3. Which of these cancers has the strongest link with GH excess (acromegaly)? A. B. C. D. Breast cancer Lung cancer Prostate cancer Colorectal cancer Question 4 4. Which of these cancers has the weakest link with GH excess (acromegaly)? A. B. C. D. Breast cancer Lung cancer Prostate cancer Colorectal cancer Question 5 Which statement regarding testicular dysfunction in HIV-infected patients is most accurate? A. Hypogonadism is most often primary B. Megestrol acetate used in AIDS wasting may inhibit critical enzymes in testicular steroidogenesis and lead to hypogonadism C. Levels of sex hormone–binding globulin are usually decreased in HIV-infected patients D. Physiologic testosterone replacement results in increased lean body mass, improved quality of life, and reduction in indices of depression in HIV-infected men with hypogonadism Question 6 • A 75-yo man was referred to you because of fatigue, weight loss and generalized aches for the last two years. He has a history of a “stress fracture” of his foot 1.5 years ago. Bone density ordered recently by his podiatrist showed osteoporosis in the hip and osteopenia in the lumbar spine. He has a subcutaneous nodule on his right shin. • Lab results: PTH = 78 pg/mL (Nl 10-60) Calcium = 9.8 mg/dL (Nl 8.5-10.5) Phosphorus 1.3 mg/dL (Nl 2.5-4.5) Vitamin D 25OH was 31 ng/mL (Nl 30-100) 6. What findings are most consistent with his presentation? A. Low vitamin D 1,25; high urine phosphorus; high serum FGF-23 B. High urine calcium; high vitamin D 1,25; high serum FGF23 C. Low urine calcium; low urine phosphorus; low serum FGF-23 D. Low vitamin D 1,25; low urine phosphorus; low serum FDG-23 Question 7 7. What is the correct statement regarding familial dysalbuminemic hyperthyroxinemia: A. The albumin variant has a high affinity for T4 and T3 B. Free T4 index (FTI) is normal C. TSH is suppressed D. Free T4 is normal E. It is transmitted as an autosomal recessive trait Question 8 A 28-year old man is brought in by his wife because of cognitive changes, short term memory deficit, involuntary movements, weakness and numbness of his legs with recent loss of balance leading to falls. The symptoms have been progressively worsening over a few months. He had to stop working recently. Magnetic resonance imaging of the brain revealed patchy white matter changes. His wife noted that his skin has been a little darker and that he has had a decreased libido and erections. He has lost some weight and his appetite seems to have decreased. Baseline serum cortisol was 7 ug/dL and ACTH was 150 pg/mL. After the intravenous injection of 250 mcg of cosyntropin, his serum cortisol rose to 16 ug/dL at 30 minutes and 19 ug/dL at 60 minutes. There is no family history of similar problems. You made the correct diagnosis of this condition. Which characteristic is true about this condition? A. B. C. D. E. It is autosomal recessive There is decreased synthesis of very-long chain fatty acids The defective gene is ABCD1 Plasmapheresis will stop the progression The abnormality is at the neuromuscular junction Question 9 A 26 year old man is referred to you for lack of sexual development. He has been dealing with this since teenage. It became a problem when he recently met a partner and sought medical help. He is of normal height but he has a enuchoid habitus. He has no gynecomastia, penis is small and testicles are in the scrotum but they are very small. The patient cannot smell coffee or vanilla. Lab results: Normal chemistry panel Serum PRL = 12 ng/mL Serum TSH = 0.44 mU/L Serum Free T4 = 1.2 ug/dL Serum total testosterone 20 ng/mL Serum LH = 0.4 IU/L Serum FSH = 0.4 IU/L Magnetic resonance imaging: Normal brain and pituitary 9. Which one of the following characteristics is true about the patient’s condition? A. This patient has seminiferous tubular dysgenesis B. There is KAL1 mutation C. There is FGFR1 mutation D. There is a high risk of aortic valvular disease and ruptured berry aneurysms E. There is a high risk of acute leukemia, lymphoma, and midline germ cell tumors Question 10 One of your patients with uncontrolled type-2 diabetes mellitus comes to see you because of an ulcer that she has had on the bottom of her left foot over the first metatarsal head for 4-6 weeks. Her typical glycated hemoglobin is around 9%. On exam, she has a deep ulcer that you were able to probe to the bone. She has poor sensation in her feet and there is mild redness around the ulcer. There is foul smelling discharge on the sock. Lab test results: WBC = 12,500/microL Sed rate = 50 mm/hr You wanted to confirm osteomyelitis to decide how long to treat her with antibiotics. 10. Which test has the best specificity for the diagnosis of osteomyelitis? A. Plain radiograph of the foot B. Indium-labeled WBC scan C. Triple-phase bone scan D. MRI with contrast E. Blood culture Question 11 A 62 year old woman is referred to you by her primary care physician for suspicion of a neuroendocrine tumor. She has had intermittent diarrhea for 15 years which was diagnosed as irritable bowel syndrome. For the last 3 years she has had episodes of spontaneous facial flushing with identifiable triggers lasting 10-20 minutes. She has no respiratory symptoms. Her physician ordered lab tests that showed the following: CBC = normal Hepatic panel = normal Urine 5-HIAA = 350 mg/24 hr (Normal <30 mg/24 hr) TSH = 0.40 mU/L (Normal 0.40-5.50) T4, free = 1.4 ng/dL (Normal 0.7 – 1.8) 11. What is the next best diagnostic test in this patient? A. MRI of the chest and abdomen B. Somatostatin-receptor whole body Scintigraphy C. Measurement of plasma 5-Hydroxytrytophan D. Radioactive iodine uptake and scan E. Repeat urine measurement of 5-HIAA with restriction of foods and drugs that can cause elevation Question 12 You are asked to see a 63-year old woman who has been hospitalized for the last 2 weeks with multiple painful skin ulcers occurring on the low abdomen and thighs. You tell the attending that you are not a dermatologist but he insists that he wants your help because he is suspecting that her condition could have an endocrine basis. You find out that she has had end-stage renal disease (ESRD) for which she has been receiving hemodialysis. The ESRD complicated her type-2 diabetes mellitus. The ulcers are wide and deep. The floor is covered by thin green material. Some of the areas are covered by thick black eschars. The surrounding skin appears red and thickened. Lab results: Serum creatinine = 5.3 mg/dL (normal 0.6-1.3) Serum Calcium = 8.3 mg/dL (normal 8.5-10.3) Serum PTH = 300 pg/mL (normal 15-55) Serum phosphorus = 6.2 mg/dL (nl 2.5-4.5) WBC = 14,000/uL Soft tissue radiographs of the leg: Calcifications of subcutaneous arteries 12. What is the most correct statement to tell to the attending physician? A. Parathyroidectomy will cure the cutaneous lesions and improve survival B. Changing to a non-calcium containing phosphate binder will likely improve the cutaneous lesions C. Hyperbaric oxygen therapy improves the lesions in most patients D. Oral sodium thiosulphate can improve the lesions E. The prognosis is very bad and no single intervention has proven effective. Supportive therapy, selective debridement and preventing infection are crucial Question 13 You see your 58-year old man with type-2 diabetes mellitus during an office follow-up visit. He had coronary revascularization 12 months ago. His BMI is 40 kg/m2 and he continues to struggle with following a low-calorie diet. He has not cut back on cigarette smoking. His blood pressure averages 140/95 mmHg despite triple-therapy. His last LDL-cholesterol was 120 mg/dL and he has cut back significantly on his consumption of animal fat compared to 6 months ago and he has been taking his 20 mg of pravastatin regularly. Lab test results a week ago: HbA1c = 8.2% Total cholesterol = 157 mg/dL LDL-cholesterol = 90 mg/dL HDL-cholesterol = 35 mg/dL Triglycerides = 160 mg/dL Fasting glucose = 170 mg/dL TSH = 2.230 mU/L Urine albumin/creatinine = 110 ug/mg 13. What is the best approach to lipid management in this patient? A. Add fenofibrate 145 mg daily B. Increase pravastatin to 40 mg daily C. Change from pravastatin to Lipitor 20 mg daily D. Change from pravastatin to rosuvastatin 10 mg daily E. Add ezetimibe 10 mg daily Question 14 A 50 year old former commercial sex worker was referred to you for progressive generalized weakness, poor appetite and 20-lb weight loss over 2 months. She has been nauseated and lightheaded. She has just finished a course of treatment for histoplasmosis. You recorded orthostatic hypotension and on examination you noticed increased skin and mucosal pigmentation. Lab results: ACTH = 250 pg/mL (normal 10-55) Cortisol = 2.5 ug/dL (drawn in AM) Serum potassium = 5.5 mEq/L Serum sodium = 130 mEq/L Serum calcium = 10.9 mg/dL 14. What is the best explanation for the patient’s problem? A. Bilateral adrenal hemorrhage B. Only the adrenal cortex is affected by the infectious process because of the nature of the vascular supply to the gland C. The patient may have an “acquired” form of glucocorticoid resistance due to reduced glucocorticoid receptor affinity D. The antifungal treatment precipitated the current problem E. The most common cause of adrenal gland destruction in AIDS patients is Mycobacterium Avium-intracellulare Question 15 A 44 year old man was recently diagnosed with medullary thyroid cancer (MTC) on FNA of a right thyroid nodule. He underwent total thyroidectomy with ipsilateral level VI dissection. On histology he was found to have a 2.5 cm MTC with invasion of the perithyroidal tissues and 6/6 positive lymph nodes without extranodal extension. Preoperatively he had had a normal chest radiograph and plasma metanephrines. On his 3-month postoperative visit, he is on levothyroxine 125 mcg daily and he has the following findings: Lab results: TSH = 1.5 mIU/L Calcitonin = 210 pg/mL (normal <16) Calcium = 8.2 mg/dL Neck ultrasound: No evidence of disease 15. What is the best next step in this patient? A. Reassure the patient that his prognosis is good, increase the levothyroxine to 137 mcg to suppress the TSH to 0.1-0.3 mIU/L and have him return for a 3-month follow up B. Reassure the patient that there is no need to be alarmed and repeat the calcitonin in 2-3 months C. He should undergo additional imaging to evaluate for distant metastases D. He should undergo external beam radiation to the right neck E. The patient should be considered for inclusion in a clinical trial Question 16 You inherit an 18 year old man from his pediatric endocrinologist. He has a history of whole-brain irradiation for a malignant tumor when he was 11 and developed growth hormone deficiency. He was maintained on human growth hormone treatment (hGH) and achieved satisfactory growth. He and his mother had questions regarding the benefits and risks of continuing hGH. What is the best answer to give to this patient? A. Continuing hGH will increase the risk of brain tumor recurrence B. HGH treatment may increase the risk of radiation-induced second tumor C. Extrapolating from patients with acromegaly, the overall risk of cancer will be higher D. There is no evidence that high normal IGF-1 levels are associated with higher risk of cancer E. HGH treatment is contraindicated Question 17 17. According to the American Thyroid Association guidelines for the management of thyroid nodules, which nodule in the different scenarios described below should have a diagnostic FNA? A. A 4-mm solid nodule in a patient who was exposed to ionizing radiation 10 years ago B. A 9-mm nodule in a low risk patient C. An 18-mm spongiform nodule in a low risk patient D. A purely cystic 24-mm nodule in a low risk patient E. A 13-mm solid hypoechoic nodule in a low risk patient Question 18 A 17-year old girl is brought by her parents because of concern with amenorrhea. She has been a ballet dancer since age 6 and trains for 15 hours per week. According to her mother she eats very well. She does not have any concerns that her daughter could have bulimic practices. She started menarche at age 13 and had about 4 periods a year until age 15 when she stopped having menses. She is 5’ 2” tall and weighs 75 lbs. She has normal BP and heart rate. She is not orthostatic. You do not notice any skin hyperpigmentation or pallor. She has normal Tanner stage breast and normal pubic and axillary hair. She had withdrawal menses after progesterone challenge. What do you expect to find in this young woman? A. Elevated serum leptin level B. Decreased mean GH and IGF-1 levels C. Elevated plasma cortisol D. Elevated plasma DHEAS E. Elevated FSH and LH Question 19 43 yo female had a pelvic ultrasound for pain and was found to have a left adnexal mass. She underwent left oophorectomy and was found to have a peritoneal mass which was excised. Pathology revealed struma ovarii in the ovarian lesion and benign thyroid tissue in the peritoneal lesion. She underwent laparoscopy which detected multiple peritoneal lesions which all contained “benign” thyroid tissue on biopsy. Lab work: TSH 0.675 mU/L (normal 0.4-5.5) Thyroglobulin 38.4 ng/mL (normal 0.8-49) Thyroglobulin Antibody titer 4.2 IU/mL (normal <14.4) She underwent a total thyroidectomy to allow for scanning and treatment of the peritoneal lesions. She was found to have a 0.1-cm papillary thyroid carcinoma, classical type with focal tall cell changes. Question 19 Question 19 What the correct statement about the patient’s case? A. The patient has metastatic papillary thyroid carcinoma B. The patient has peritoneal strumosis C. The patient should have the peritoneal lesions resected D. The patient should receive chemotherapy E. None of the above is correct Question 20 What is the correct statement regarding “thyroid” changes during treatment with SUNITINIB? A. Hypothyroidism is always preceded by a hyperthyroid destructive phase B. The risk of developing thyroid dysfunction decreases with time as the patient continues on treatment cycles C. The increase in thyroid hormone requirement during sunitinib treatment may be due to inhibition of MCT8 D. Sunitinib may inhibit type 3 deiodinase activity in the liver E. None of the above is correct Thank you