Cushing Syndrome MCQs

Most common cause of Conn's syndrome is aldosterone- producing adrenal adenomas Reference: Ganong, 25th edition page no.369

MEN 2 is associated with pheochromocytoma, not MEN 1 Syndromes associated with pheochromocytoma: MEN 2 (Both 2A and 2B) Von Hippel-Lindau disease Neurofibromatosis 1 Carney's triad Reference : Harrison's Principles of Internal Medicine 21st edition,page number2966

One should always think of the possibility of Sheehan syndrome when a postpartum female presents with fatigue and hyponatremia. This is attributed to secondary adrenal insufficiency. Hyperkalemia is not seen in Sheehan syndrome as low ACTH doesn't impact aldosterone production from adrenal gland. However, absence of lactation is the most specific feature associated with Sheehan syndrome. The presence of normal lactation along with presence of hyperkalemia in the patient makes the diagnosis of Sheehan syndrome unlikely. Reference : Harrison's Principles of Internal Medicine 21 st edition Page 2971

The typical triad in a patient with primary aldosteronism is: 1. Systemic hypertension 2. Hypokalemia 3. Metabolic alkalosis Most common cause for primary aldosteronism is bilateral adrenal hyperplasia followed by aldosterone producing adenoma (Conn syndrome) Reference : Harrison's Principles of Internal Medicine 21 st edition Page 2965

Beta blockers will falsely increase the ARR (beta receptors mediate the renin secretion from JG apparatus and its blockade would lower renin production) ACE inhibitors, ARBs and diuretics reduce the ARR (they inhibit the aldosterone production thereby causing compensatory increase in renin levels) CCBs, α1 blockers and direct vasodilators like hydralazine do not affect the ARR. Reference : Harrison's Principles of internal medicine 21st edition, page number. 2966

Patients receiving glucocorticoid replacement therapy should be advised to double the dose in the event of intercurrent febrile illness, accident, or mental stress such as an important examination. If the patient is vomiting and cannot take by mouth, parenteral hydrocortisone must be given urgently, as indicated above. There is no need to increase fludrocortisone dose during stress / inter current illness For minor surgery, 50-100 mg of hydrocortisone IV is given with the premedication. For major procedures this is then followed by the same regimen as for acute adrenal insufficiency. Reference : Harrison's Principles of Internal Medicine 21 st edition Page 2972

This man has spotty skin pigmentation, ACTH independent Cushing syndrome and a probable left atrial myxoma. Carney complex is diagnosable with two features out of: spotty skin pigmentation myxoma of skin / heart ( MC location for cardiac myxoma : left atrium) endocrine tumors (commonest being primary pigmented nodular adrenocortical disease ), but it is also associated with Sertoli-cell tumors, growth hormone- or prolactin-producing pituitary adenomas, thyroid adenomas and ovarian cysts) and psammomatous melanotic schwannoma (PMS). It is also diagnosable in the presence of one feature and an affected first-degree relative. It is an autosomal-dominant condition caused by an inactivating mutation of protein kinase A on chromosome 17. Reference : Harrison's Principles of Internal Medicine 21 st edition Page 2908

In Cushing's disease, steroidogenic inhibitors are used in combination with pituitary irradiation to block the adrenal effects of persistently high ACTH levels. Ketoconazole is an antimycotic drug that inhibits several P450 enzymes involved in adrenal steroidogenesis. Metyrapone inhibits 11 beta-hydroxylase activities & Mitotane suppresses cortisol hypersecretion by inhibiting 11 beta-hydroxylase and cholesterol side-chain cleavage enzymes & by destroying adrenocortical cells. Drugs acting at the level of pituitary to reduce ACTH include Pasireotide and Cabergoline Drugs that block cortisol action on receptor include Mifepristone. Reference : Harrison's Principles of Internal Medicine 21 st edition Page 2963