Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.

Definition

A disorder of adrenal gland development characterized by absence of the permanent zone of the adrenal cortex, structural disorganization of the adrenal glands, adrenal insufficiency and profound hormonal deficiencies. AHC patients manifest primary adrenal failure usually in early infancy, and hypogonadotropic hypogonadism leading to absent or incomplete sexual maturation. AHC can be inherited in an X-linked or autosomal recessive pattern.

Acronym

AHC

Synonyms

Adrenal hypoplasia, congenital, with hypogonadotropic hypogonadism
AHCH
AHC with HHG
AHC with isolated gonadotropin deficiency
AHX
Cytomegalic adrenocortical hypoplasia
X-linked Addison disease
X-linked adrenal hypoplasia congenital