Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder affecting adrenal steroid synthesis. More than 95% of CAH cases are caused by reduced 21hydroxylase function leading to variable extent of cortisol and aldosterone deficiency in addition to androgen excess. The foundation of CAH treatment is the use of glucocorticoids. However, overtreatment leads to Cushing’s syndrome and ...

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...

Congenital Adrenal Hyperplasia (CAH) is caused by congenital insufficiency of the enzyme 21 - hydroxylase (21-OHD) in the cortisol synthesis pathway. Because of the virilizing effects of androgens over-production, affected girls develop clitoral hypertrophy. Three patients with CAH are discussed below along with their surgical management and follow-up.

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. ...

background: precocious puberty, as early physical development and low final height might lead to psychosocial problems. objective: to evaluate etiology and clinical feature of precocious puberty in a cohort of iranian children. materials and methods: in this case-series study, 44 girls and 8 boys with precocious puberty referred to endocrine reserch centre (firouzgar), institute of endocrinolog...

Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confir...

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