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What Is Congenital Adrenal Hyperplasia?

Congenital adrenal hyperplasia, also known as adrenogenital syndrome, is an inherited condition caused by genetic defects in hormone-producing enzymes. The disease causes the body to produce inadequate amounts of the adrenal steroid hormones cortisol and aldosterone. This causes an overproduction of the hormone androgen, a male steroid hormone.

  1. Types

    • The two main types of congenital adrenal hyperplasia are classic and nonclassic. The classic form if more severe and affects newborns and young children. The nonclassic form is milder and affects older children and young adults.

    Mild Symptoms

    • Symptoms of a mild form of congenital adrenal hyperplasia include early puberty, acne, excess facial hair in women, irregular facial hair and being shorter than your parents.

    Severe Symptoms

    • Symptoms of a severe form of congenital adrenal hyperplasia include low blood pressure and blood sugar level, benign testicular tumors, male infertility, and in girls, altered development of genitalia.

    Complications

    • A complication of classic congenital adrenal hyperplasia is a life-threatening condition called adrenal crisis. The condition causes diarrhea, vomiting, shock, low blood sugar levels, dehydration and low levels of sodium in the blood.

    Treatment

    • Treatment of congenital adrenal hyperplasia includes medication to replace the hormone the patient's body is not making. For example, hydrocortisone or dexamethasone may be prescribed to replace cortisol.

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