Skip Header

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


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.




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


Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice.

Cookie policy

We would like to use anonymized google analytics cookies to gather statistics on how is used in aggregate. Learn more

UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health