<prompt> Enzyme most commonly deficient in congenital adrenal hyperplasia is21 beta hydroxylase5 alpha reductase11 beta hydroxylase17 alpha hydroxylase  <response>: Ans. a 21 beta hydroxylaseRef: Bailey and love, 27th ed Campbell 11th Ed consolidationCongenital adrenal hyperplasia or Adrenogenital syndrome: Features Salt wasters virilizers due to aldosterone deficiency Simple virilizers Aldosterone normal Non classified no virlization, no salt wasting Virilization and adrenal insufficiency in children are pathognomonic of congenital adrenal hyperplasia CAH. Autosomal recessive disorder The most frequent defect 95 per cent is the 21 bhydroxylase deficiency, which has an incidence of 1 in 5000 live births. Excessive ACTH secretion is caused by the loss of cortisol and this leads to an increase in androgenic cortisol precursors and to CAH. CAH may present in girls at birth with ambiguous genitalia or as late-onset disease at puberty. Little Hercules: Hypertension and short stature, caused by the premature epiphyseal plate closure, are common signs. In female causes Female hermaphrodite Diagnosed antenatally by 9-11 weeks by chorionic villus sampling Antenatally mother should be treated with IV Dexamethasone Affected patients are treated by replacement of Hydrocortisone - fludrocortisone. Large hypoplastic adrenals may need to be removed if symptomatic. 