Internal medicine

Congenital adrenal hyperplasia

Adrenal crisis

Disease

Physiology

Obesity

Endocrinology

hirsutism

Insulin resistance

Adrenal insufficiency

Hyperandrogenism

Polycystic ovary

Medicine

The Lancet

Congenital adrenal hyperplasia is a group of autosomal recessive disorders leading to multiple complex hormonal imbalances caused by various enzyme deficiencies in the adrenal steroidogenic pathway. The most common type of congenital adrenal hyperplasia is due to steroid 21-hydroxylase (21-OHase, henceforth 21OH) deficiency. The rare, classic (severe) form caused by 21OH deficiency is characterised by life-threatening adrenal crises and is the most common cause of atypical genitalia in neonates with 46,XX karyotype.