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ABIM Review - Endocrinology

ABIM Review - Endocrinology. “There are a large number of substances, acting powerfully in minute amount, which are of great importance in physiological processes.” - Sir William Maddox Bayliss. Historical Perspective. Question 1 (The GMO special).

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ABIM Review - Endocrinology

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  1. ABIM Review - Endocrinology

  2. “There are a large number of substances, acting powerfully in minute amount, which are of great importance in physiological processes.” - Sir William Maddox Bayliss Historical Perspective

  3. Question 1 (The GMO special) • A 28 y/o male is evaluated for fatigue, declining libido, and erectile dysfunction over the past 6 months. He has no significant PMHx has a stable female sexual partner, and they are NOT interested in having children. PE shows full visual fields to confrontation, 6 mL soft testes, and a trace of gynecomastia. A random testosterone is 56 ng/dL (1.94 nmol/L), LH 0.6 mU/mL (o.6 U/L), FSH 0.5 mU/mL (0.5 U/L), and prolactin 42ng/mL (42 mg/L). He requests testosterone replacement therapy. • Before testosterone replacement is started, which of the following would be indicated next? • Trial of dopamine antagonist therapy • MRI of sella turcica • Measurement of nocturnal penile tumescence • Formal visual field testing • Trial of sildenafil therapy

  4. Question 1 (The GMO Special) • MRI of sella turcica • Serum testosterone of <100 ng/dL (3.47 nmol/L) in a young male – at any time of the day – is nearly always pathologic, particularly if accompanied by atrophic testes. Most acquired hypogonadism is due to a primary disorder, which is NOT excluded by the normal visual fields with slightly elevated prolactin. Although testosterone is best assessed in the early morning and measurement of PSA and DRE are recommended before starting testosterone replacement in an older man, this young person needs a laboratory and radiologic evaluation of the pituitary gland. • The severely depressed testosterone and mildly elevated prolactin suggest hyperprolactinemia secondary to stalk compression rather than prolactinoma. Dopamine agonist therapy will lower the prolactin but not restore testosterone production in this patient. Formal tests of visual fields, penile tumescence, and sildenafil trial are not as important as imaging, and MRI is superior to CT in evaluation the sella.

  5. Question 1 (The GMO Special)

  6. Question 1 (The GMO Special)

  7. Question 2 (The GMO Special) • A 19 y/o woman presents with CC of never experiencing breast development. No significant PMHx and takes no medications. No family hx of infertility or intersex disorders. Patient is 61 in. tall and weighs 127 lbs. Is normotensive, no dysmorphic features, and full visual fields. Has sparse axillary and pubic hair, and Tanner stage 1 breasts. Pelvic exam shows a small vagina with pink mucosa and a small uterus. LH level is 24 mU/mL, FSH 67 mU/mL, and estradiol <10 pg/mL. Which of the following tests will establish the diagnosis? • Serum total testosterone • Serum 17-hydroxyprogesterone before and after cosyntropin • Pelvic ultrasound • Karyotype • MRI of the head with fine cuts of the sella turcica

  8. Question 2 (The GMO Special) • Karyotype • Absence of secondary sexual characteristics and menopausal FSH indicates that this pt. has ovarian failure. For a short young woman with primary amenorrhea even in the absence of associated stigmata and comorbidities, Turner’s syndrome (or mosaic) is the most likely diagnosis. Other possibilities include Swyer’s syndrome (ovarian dysgenesis with 46,XY karyotype) and idiopathic ovarian failure, and the karyotype will distinguish these etiologies. Serum total testosterone is obtained if androgen insensitivity is a consideration, but lack of breast development excludes that diagnosis. Serum 17-hydroxyprogesterone would evaluate androgen excess, and the lack of pubic and axillary hair render this test unnecessary. Imaging of the ovaries and sella is not informative when gonadal failure is diagnosed by the high FSH. The 45,X_ karyotype is surprisingly common, occurring in about 1 in 100 pregnancies. However, most of these cases spontaneously abort before birth. Turner’s syndrome of mosaicism should also be considered in a woman in premature ovarian failure.

  9. Question 2 (The GMO Special)

  10. Question 2 (The GMO Special)

  11. Drugs that cause gynecomastia by uncertain mechanisms: Calcium channel blockers (verapamil, nifedipine, diltiazem) ACE Inhibitors (captopril, enalapril) Beta-blockers Amiodarone Methyldopa Reserpine Nitrates Neuroleptics Diazepam Phenytoin Tricyclic antidepressants Haloperidol Drugs that cause gynecomastia by uncertain mechanisms: Indinavir Isoniazid Ethionamide Griseofulvin Amphetamines Theophylline Omeprazole Auranofin Diethylpropion Domperidone Penicillamine Sulindac Heparin Question 2 (The GMO Special)

  12. Question 3 (IM Clinic Special) • 45 y/o man requests advice about prevention of diabetes mellitus. He has HTN, which takes a B-blocker for. His father developed type 2 DM at age 49, had HTN and HLP, and subsequently died of a MI. On PE, BP=150/95, HR=80, BMI of 31.8 • Fasting glucose 94, 2 hr. postprandial 136, Cholesterol 185, Trig 203, HDL 44, LDL 100 • Which of the following would be the most effective in preventing the development of type 2 DM in this patient? • Diet, exercise, and weight loss • Metformin • Rosiglitazone • Pioglitazone • Low dose insulin therapy

  13. Question 3 (IM Clinic Special) • Diet, exercise, and weight loss • The Diabetes Prevention Program (DPP) showed that high risk patients with obesity and metabolic syndrome, such as this patient, can reduce their risk of developing type 2 diabetes by 58% with a conscientious lifestyle modification program consisting of diet, exercise, and weight loss. In that same study, metformin decreased the risk by 31%. In Hispanic patients with previous GDM, troglitazone was shown to reduce the risk of developing type 2 DM by 55%, but neither rosiglitazone nor pioglitazone has yet been shown in published data to reduce the risk of diabetes in patients such as the one presented here. Similarly, there are no published data regarding early insulin therapy in this group.

  14. Question 3 (IM Clinic Special) • Metabolic Syndrome (based on ATP III), 3 or more components • Central obesity (male waist > 40in, female waist > 35 in) • Atherogenic dyslipidemia (Trig >150 mg/dL, HDL <40 [male], <50 [female] mg/dL) • Insulin resistance or glucose intolerance (FG > 100 mg/dL) • Raised blood pressure (130/85 mmHg or higher) • Prothrombotic state (e.g., high fibrinogen or plasminogen activator inhibitor [–1] in the blood) • Proinflammatory state (e.g., elevated high-sensitivity C-reactive protein in the blood)

  15. Question 4 (IM Clinic Special) • A healthy 54 y/o woman with a strong family hx of type 2 DM requests an oral glucose tolerance tests to evaluate her risk of developing diabetes. The results of a 75g 2-hr oral glucose tolerance test are as follows: • Fasting 104, 2 hour post 168 mg/dL • Which of the following characterizes the patient’s glycemic status? • Normal glucose tolerance • Type II DM • Impaired fasting glucose • Impaired glucose tolerance • Impaired fasting and glucose tolerance

  16. Question 4 (IM Clinic Special) • Impaired fasting & glucose tolerance • Prediabetes glycemic states consist of impaired glucose tolerance, defined as a 2-hour glucose level of 140-199 mg/dL during an oral glucose test, and impaired fasting glucose, defined as a fasting glucose level of 100-125 mg/dL • Diabetes is defined as “casual” glucose > 200 mg/dL accompanied by classic symptomsOR fasting glucose > 126 mg/dL OR a 2 hour oral glucose test > 200 mg/dL

  17. Question 5 (The Ward Special) • 65 y/o man admitted for treatment of severe hypothyroidism. He was doing well initially on thyroid replacement therapy but experienced a syncopal episode when walking to the bathroom. The pt denies palpitations or chest pain. On exam, BP 80/60, HR 72,. +JVD, diminished heart sounds, and a palpable liver edge below the right costal margin. CBC and P2 are normal. Free T4 is 0.7 ng/dL, and TSH is 22 microU/mL. EKG shows low voltage in all leads. CXR shows an enlarged cardiac silhouette. • Which of the following is the most likely cause of this patient’s change in clinical status? • Adrenal insufficiency • Ischemia • Myocarditis • Cardiomyopathy • Cardiac tamponade

  18. Question 5 (The Ward Special) • Cardiac Tamponade • Pericardial effusion is a consequence of moderate to severe hypothyroidism and is indicated by the diminished heart sounds, low voltage on EKG, and an enlarged cardiac silhouette. The “hypothyroid heart” refers to decreased contractility and pulse rate – both contributing to decreased cardiac output at a time when peripheral vascular resistance is increased.

  19. Question 6 (The Ward Special) • 48 y/o man is diagnosed with a single, surgically accessible focus of non-small cell carcinoma of the lung. He is undergoing evaluation before possible resection of his primary tumor. During CT of the chest & abdomen, a 1.5 cm mass is detected in the adrenal gland contralateral to his lung tumor. The mass is homogenous, has smooth borders, and has attenuation of 0 Hounsfield units. The patient is normotensive with normal P2 and no signs of glucocorticoid excess. • Which of the following would be the most appropriate next step in the management of this patient? • Cancel surgical resection due to presence of metastatic dx • Order fractionated plasma metanephrines, overnight dexamethasone suppression test, and plasma aldosterone/plasma renin activity ratio • Order selective adrenal venous sampling • Order MRI of the adrenal glands

  20. Question 6 (The Ward Special) • Order fractionated plasma metanephrines, overnight dexamethasone suppression test, and plasma aldosterone/plasma renin activity ratio • In patients with known extra-adrenal malignancy (lung, breast, renal), adrenal mass raises suspicion of metastases; ~20% of lung cancer pt have adrenal metastases upon initial presentation. However, unrelated adrenal incidentalomas may also be present and confuse the picture. • Radiographic imaging may prove helpful in determining risk of metastases from a nonadrenal malignancy. Benign adrenal adenomas are homogenous and have smooth borders and attenuation values of less than 10 Hounsfield units on unenhanced CT. Metastatic foci generally have higher attenuation but there is some overlap between the 2 groups • Assessment of hormonal should be performed to r/o pheochromocytoma. FNA of the mass may then be safely be conducted. Cytology will differentiate between adrenal and nonadrenal neoplasm. • Selective adrenal venous sample is helpful only in localizing sources of aldosterone production. MRI of adrenal glands offers little information that was already disclosed from CT.

  21. Question 6 (The Ward Special)

  22. Question 7 (The Chief’s Special) • 42 y/o male is evaluated for poor libido, fatigue, and skin darkening that has progressively worsened over the past 3 years. Underwent a normal puberty and has 2 children. Denies any vision loss, gynecomastia, or cold intolerance. PMHx significant for arthritis of the hands. Family hx negative for calcium or pituitary tumors but has a maternal uncle with cryptogenic cirrhosis. PE shows full visual fields, soft 12 mL testes, and b/l swollen metacarpophalangeal joints. The skin is dark and without striae or bruises. • HCT 50%, LH 0.2 mU/mL, FSH 0.2 mU/mL, Prolactin 4 ng/mL, Testosterone 128 ng/dL, PSA 0.2 ng/mL • Which of the following is the most appropriate next step in this patient’s evaluation? • Gonadotropin releasing hormone stimulation test • Karyotype • MRI of th head • Measurement of serum transferrin saturation and ferritin • Therapeutic trial of testosterone

  23. Question 7 (The Chief’s Special) • Measurement of serum transferrin saturation and ferritin • Patient has acquired hypogonadotropic hypogonadism in the setting of skin bronzing, large joint arthritis, and a family hx of liver disease in a male relative – all suggestive of hemochromatosis. The normal prolactin and visual fields argue against (but do not exclude) a pituitary tumor, and the high hematocrit is unusual in hypogonadism and a contraindication to testosterone therapy as well. • Iron deposition in the hypothalamus causes gradual irreversible loss of gonadotropin secretion. Other endocrinopathies of hemochromatosis include diabetes and adrenal insufficiency. Screening with ferritin and/or iron saturation studies and aminotransferases is the most efficient way to make the diagnosis, and genetic testing for HFE mutations is confirmatory.

  24. Question 7 (The Chief’s Special)

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