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Congenital adrenal hyperplasia (CAH/AGS)

Adrenogenital syndrome is an inherited condition affecting the adrenal glands. The body makes too little cortisol and, in some forms, too little aldosterone.

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  • Adrenogenital syndrome, usually called congenital adrenal hyperplasia, is a group of inherited conditions in which the adrenal glands cannot make certain hormones properly.
  • In the most common form, there is too little cortisol, sometimes too little aldosterone, and relatively large amounts of androgens are produced.
  • The classic salt-wasting form can cause a life-threatening adrenal crisis in a baby within days to weeks.
  • Classic CAH is treated for life with glucocorticoids and sometimes fludrocortisone and extra salt, according to a specialist plan.
  • Fever, surgery, a serious injury or repeated vomiting often requires temporary extra hydrocortisone according to individual sick-day rules.
  • Everyone at risk of an adrenal crisis should have an emergency card, an emergency injection and an emergency plan that has been practised.
  • Non-classic CAH is milder and needs treatment only if there are significant symptoms.
  • Treatment requires balance: too little hormone causes a crisis or excess androgens; too much medication can harm growth, bones, weight and blood pressure.

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