We present the first report of primary hyperaldosteronism in childhood due to unilateral macronodular hyperplasia. A 10-year-old white boy with severe hypertension (150/100 mm Hg), hypokalemia (1.4 mEq/liter), and suppressed plasma renin activity (PRA) (< 0.1 ng/ml/hr) demonstrated fixed PRA and aldosterone (aldo) levels that did not change with alteration of dietary sodium. The paradoxical dec...

Following the demonstration by Wilkins, Lewis, Klein, and Rosenberg (1) that cortisone controls the clinical manifestations and abnormal laboratory findings in virilizing adrenal hyperplasia, Bartter, Albright, Forbes, Leaf, Dempsey, and Carroll (2) postulated a deficient synthesis of "sugar hormone" (17-hydroxycorticosterone, Compound F) in this disease, reasoning from the unusual response of ...

Adrenocortical tumors (ACTs) are typically unilateral and can be classified as benign adrenocortical adenomas (ACAs) or malignant adrenocortical cancers (ACCs). In rare cases, tumors may occur in both adrenal glands as micronodular hyperplasia (primary pigmented nodular adrenal dysplasia) or as macronodular hyperplasia (primary bilateral macronodular adrenal hyperplasia, PBMAH). The study of ce...

Though the familial occurrence of virilization associated with hyperplasia of the adrenal cortex has long been established, there have been only two reports of the familial aggregation of virilizing adrenal carcinoma. It is the purpose of the present communication to describe a family in which 2 sibs, who were products of a consanguineous marriage, had virilizing tumours ofthe adrenal cortex pr...

Testicular adrenal rest tumors (TART) occur often as asymptomatic nodules in corticotropin-dependent lesions aberrant adrenal tissue in congenital adrenal hyperplasia (CAH) patients. The present manuscript is about an unusual case of a 16-year-old CAH patient due to 11β-hydroxylase deficiency. He underwent testicle biopsy because of testicle tumor suspicion and diagnosed with TART.

Adrenal histoplasmosis in Cushing's syndrome of adrenal origin is rare. A patient with Cushing's disease with bilateral nodular hyperplasia and histoplasmosis of both the adrenal glands is described. The diagnosis of histoplasmosis was only made post operatively as the constitutional manifestations, besides being partially masked by hypercortisolism also resemble those of tuberculosis.

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder characterized by enzyme defects in the steroidogenic pathways. Testicular adrenal rest tumors (TARTs) are may have serious consequences in patients with CAH. They probably develop from ectopic remnants of intratesticular adrenal tissue, which might be stimulated by the rise of adrenocorticotropic hormone (ACTH). Their histo...

The case-report is about a 47 year old woman with adrenocorticotropin-independent macronudular adrenal hyperplasia (AIMAH), with is a rare cause of endogenous Cushing’s syndrome. Urin free cortisol (UFC) and cortisol of 8AM were elevated along with the suppressed level of ACTH. Abdominal CT scan showed macronodules in both adrenals .The patient underwent left adrenalectomy and pathological dat...

OBJECTIVE ACTH-independent macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered the treatment of choice, but the patient is obliged to receive lifetime steroid replacement therapy and is susceptible to adrenal insufficiency crisis. New therapeutic alternatives are being proposed as new etiopathological features of the disease are known. U...

21-hydroxylase deficiency (21-OHD) caused congenital adrenal hyperplasia (CAH) is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (CO) synthesis in the adrenal glands. Testicular adrenal rest tumors (TARTs) are rarely the presenting symptoms of CAH. Here, we describe a case of simple virilizing CAH with TARTs, in a 15-year-old boy. The p...