BACKGROUND Congenital adrenal hyperplasia is a group of disorders caused by defects in the adrenal steroidogenic pathways. In its most common form, 21-hydroxylase deficiency, patients develop varying degrees of glucocorticoid and mineralocorticoid deficiency as well as androgen excess. Therapy is guided by monitoring clinical parameters as well as adrenal hormone and metabolite concentrations. ...

Congenital lipoid adrenal hyperplasia (CLAH) is the most severe form of congenital adrenal hyperplasia that caused by mutations in the steroidogenic acute regulatory protien (StAR). The mutations in StAR gene resulted in failure of the transport cholesterol into mitochondria for steroidogenesis in adrenal gland. Twin sisters (A, B) were born on 36 gestational week premature to nonrelated parent...

Pheochromocytomas are rare neoplasias of neural crest origin arising from chromaffin cells of the adrenal medulla and sympathetic ganglia (extra-adrenal pheochromocytoma). Pheochromocytoma that develop in rats homozygous for a loss-of-function mutation in p27Kip1 (MENX syndrome) show a clear progression from hyperplasia to tumor, offering the possibility to gain insight into tumor pathobiology....

OBJECTIVE To determine the frequency of nonfunctioning adrenal masses in patients with primary hyperaldosteronism. DESIGN A case series. SETTING A tertiary care hypertension clinic. PATIENTS Twenty-seven consecutive patients with primary hyperaldosteronism. MEASUREMENTS Blood pressure, serum electrolytes, supine and upright plasma renin, cortisol and aldosterone levels, selective adrena...

UNLABELLED INTRODUCTION We report the case of a patient with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency who presented with unusual anatomical and biochemical features, namely massively enlarged adrenal glands, adrenogenital rest tissue and an unexpected endocrine profile. The contribution of the adrenocortical cells in the adrenals and testicles was determined ...

Vaquez and Aubertin advance three theories in explanation of the adrenal hyperplasia; first, that it may not be the cause of hypertension but "an antitoxic hyperplasia" caused by the retained products of metabolism which may be responsible also for the hypertension; second, that it may be the cause of hypertension but secondary to the renal lesion; third, that it may be the cause of hypertensio...

In the course of an investigation into the nature of the post-spawning death of the Pacific salmon,1-3 marked hyperplasia of the adrenal cortical tissue was found to be present constantly in the sexually mature fish of both sexes.4 5 Beginning hyperplasia was observed in immature salmon taken on their spawning migration several months before they were due to spawn. This change became more prono...

OBJECTIVE To describe the etiologies, clinical characteristics, and laboratory investigations of young Thai children being evaluated for precocious pubarche. MATERIAL AND METHOD The medical records of 41 children referred for evaluation of precocious presence of pubic hair at Songklanagarind Hospital between 1995 and 2011 were retrospectively reviewed. RESULTS The etiologies of precocious p...

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) (less than 0.1 ng/ml/hr) demonstrated fixed PRA and aldosterone (aldo) levels that did not change with alteration of dietary sodium. The paradox...