Lipoid congenital adrenal hyperplasia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Synonyms and keywords: Congenital lipoid adrenal hyperplasia

Overview

Lipoid congenital adrenal hyperplasia is a rare and usually the most severe form of congenital adrenal hyperplasia.

Pathophysiology

Lipoid congenital adrenal hyperplasia is an autosomal recessive disease; characterized by deficiency of all adrenal hormones and increased corticotropin (ACTH) secretion.

• This disease is caused by gene mutations on chromosome 8. This gene mutation codes for a protein called steroid acute regulatory protein (StAR). StAR helps cholesterol transport from the outer to the inner mitochondrial membrane.

On microscopic histopathological analysis, progressive accumulation of cholesterol esters are characteristic findings of Lipoid congenital adrenal hyperplasia.

Diagnosis

Symptoms

Symptoms can be sever hypotension due to adrenal crisis. Because lack of androgen production male infants usually have female external genitalia. Female infants have normal genitalia normally developed at birth and occasional patients undergo spontaneous puberty