Ovarian Histological Findings in an Adult Patient with the Steroidogenic Acute Regulatory Protein (StAR) Deficiency Reveal the Impairment of Steroidogenesis by Lipoid Deposition

Abstract excerpt

CONTEXT: The steroidogenic acute regulatory protein (StAR) is essential for the production of steroid hormones. The mutations in the StAR gene typically cause congenital lipoid adrenal hyperplasia (lipoid CAH), characterized by severe adrenal insufficiency in both sexes and complete female external genitalia in genetic males. Affected 46, XX females feminize at puberty and menstruate but have progressive...

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