Lipoid congenital adrenal hyperplasia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical Therapy
Management of salt-wasting crises and mineralocorticoid treatment are as for other forms of salt-wasting congenital adrenal hyperplasia: saline and fludrocortisone.
Glucocorticoids can be provided at minimal replacement doses because there is no need for suppression of excessive adrenal androgens or mineralocorticoids. As with other forms of adrenal insufficiency, extra glucocorticoid is needed for stress coverage.
XX females with lipoid CAH may need estrogen replacement at or after puberty. To date, ovulation and pregnancy has not been reported even with early diagnosis and careful glucocorticoid replacement to suppress ACTH-induced lipid damage to the ovaries.
Nearly all XY children have been so undervirilized that they have been raised as girls. The testes have been uniformly nonfunctional and are removed to prevent long term neoplastic risk.