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Congenital lipoid adrenal hyperplasia due to STAR deficency

ORPHA:90790· ICD-10 E25.0

Definition

A severe form of congenital adrenal hyperplasia (CAH) characterized by severe adrenal insufficiency and sex reversal in males.

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Childhood, Infancy, Neonatal