Mifepristone Treatment in Four Cases of Primary Bilateral Macronodular Adrenal Hyperplasia (BMAH).

CONTEXT: Primary bilateral macronodular adrenal hyperplasia (BMAH) is a rare form of adrenal Cushing syndrome conventionally treated with adrenalectomy. Medical treatment is often reserved for patients not eligible for surgery. However, to date there have been few studies about the efficacy of mifepristone for the treatment of BMAH associated with hypercortisolism. OBJECTIVE: To describe a series of patients with hypercortisolism due to BMAH treated with mifepristone from multiple medical practices. DESIGN: We retrospectively assessed four patients treated with mifepristone for hypercortisolism due to BMAH who had either failed unilateral adrenalectomy, declined surgery, or were poor surgical candidates. RESULTS: Mifepristone induced clinical improvement and remission of the signs and symptoms of hypercortisolism in all described patients with BMAH. The median treatment duration at the time of efficacy response assessment was 5 months (range: 3 to 18 months). Improvement in cardiometabolic parameters was observed as early as 2 weeks after treatment was started. All patients achieved improvements in glycemic control and hypertension and had significant weight loss. The most common adverse event observed with mifepristone therapy was fatigue. Increases in TSH level occurred in two patients. CONCLUSION: Mifepristone can be an effective medical alternative to surgery in patients with hypercortisolism due to BMAH.

PREVELENCE OF PRIMARY ALDOSTERONISM IN AN URBAN HYPERTENSIVE POPULATION.

OBJECTIVE: To determine the prevalence of primary aldosteronism (PA) in hypertensive patients presenting to the primary care clinic at The Mount Sinai Hospital, regardless of the degree of hypertension and to identify clinical criteria that should prompt screening for PA. METHODS: An aldosterone:renin ratio (ARR, cutoff ≥20, with plasma aldosterone concentration [PAC] ≥10 and suppressed renin) was used to prospectively screen 296 hypertensive patients (blood pressure [BP] ≥140/90) over the age of 18 from August 2012 through May 2013. Subjects who screened positive then underwent confirmatory oral salt load testing (OSLT). RESULTS: Of the 296 patients, 14 screened positive for PA, an overall prevalence of 4.7%. Six of the 14 cases underwent confirmatory OSLT, upon which 2 were confirmed positive, for a prevalence of 0.7%. Overall, patients with confirmed PA were more likely to have resistant hypertension (42.9% vs. 18.1% (P = .0334)) and require more antihypertensive agents (2.8 ± 1.2 agents vs. 2.1 ± 1.1 agents, P = .0213). There was a trend toward lower potassium values in the cases. CONCLUSION: The prevalence of PA in our clinic is much lower than in reports from certain "at-risk" populations. PA screening is indicated in patients with resistant hypertension, regardless of serum potassium levels. ABBREVIATIONS: ARR = aldosterone:renin ratio ACTH = adrenocorticotropic hormone AVS = adrenal venous sampling BP = blood pressure MRA = mineralocorticoid receptor antagonist OSLT = oral salt load confirmatory test PA = primary aldosteronism PAC = plasma aldosterone concentration PCP = primary care provider PRA = plasma renin activity.

Primary aldosteronism: challenges in diagnosis and management.

Primary aldosteronism (PA) is the main cause of endocrine hypertension, present in approximately 10% of hypertensive patients; about one-third is secondary to aldosterone-producing adenomas. Cardiovascular and renal morbidity are out of proportion to the degree of hypertension. Physicians have compelling rationale to correctly identify and treat PA. Physicians are challenged with patient selection for screening with the aldosterone/renin ratio (ARR), interpretation of ARR, and selecting a confirmatory test. Adrenal vein sampling is performed for subtype differentiation. The treatment depends on the disease subtype and results in control of hypertension and reversal of associated excess morbidity.

The Mount Sinai clinical pathway for the management of pheochromocytoma.

OBJECTIVE: Pheochromocytomas are complex tumors that require a comprehensive and systematic management plan orchestrated by a multidisciplinary team. METHODS: To achieve these ends, The Mount Sinai Adrenal Center hosted an interdisciplinary retreat where experts in adrenal disorders assembled with the aim of developing a clinical pathway for the management of pheochromocytomas. RESULTS: The result was a consensus for the diagnosis, perioperative management, and postoperative management of pheochromocytomas, with specific recommendations from our team of adrenal experts, as well as a review of the current literature. CONCLUSION: Our clinical pathway can be applied by other institutions directly or may serve as a guide for institution-specific management.

Approach to the patient with postprandial hypoglycemia.

OBJECTIVES: Postprandial hypoglycemia in a nondiabetic patient is a frequent chief complaint across all medical specialties and represents a significant diagnostic challenge. METHODS: We conducted an electronic and print literature search using PubMed for articles published in the last 40 years using the key words "postprandial hypoglycemia," "reactive hypoglycemia," and "hyperinsulinemic hypoglycemia." All available sources were reviewed, and publications were included based on clinical relevance. RESULTS: Over the last century, the classification, etiologies, diagnosis, and management of hypoglycemia have evolved considerably. In the contemporary literature, the evaluation of hypoglycemia is divided into well-appearing and ill-appearing patients. Symptoms of hypoglycemia in the well-appearing patient may result from insulinoma, noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS), postbariatric surgery hypoglycemia, dumping syndrome, insulin autoimmunity, or postprandial syndrome. For each of these, the literature is lacking in randomized clinical trials investigating optimal diagnostic and treatment modalities, and this is due to the relatively recent description of some diseases and the rarity of cases. CONCLUSION: This review provides an overview of postprandial hypoglycemia and summarizes the proposed pathophysiologic mechanisms of postprandial hypoglycemia, with special attention paid to some of the more recently described syndromes, such as NIPHS and postbariatric surgery hypoglycemia. Diagnostic and management strategies are also discussed.

Primary aldosteronism: emerging trends.

Primary aldosteronism (PA) is the most common etiology of endocrine hypertension (HTN), and recent prevalence studies suggest that it may be under-diagnosed. Indications for screening have been expanded with recognition that many patients with PA do not have hypokalemia and that the disease may be familial. The aldosterone:renin ratio (ARR) is the preferred screening test for PA. The ARR can be interpreted in patients on most anti-hypertensive agents, and can be used to guide medical therapy of HTN even in patients without PA. Once PA is confirmed, adrenal venous sampling (AVS) should be performed to determine if PA is due to bilateral disease or a unilateral adenoma, if surgery is being considered. Targeted medical or surgical therapy improves patient outcomes.