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Genetic and Rare Diseases Information Center (GARD)

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X-linked adrenal hypoplasia congenita

Other Names for this Disease
  • Adrenal hypoplasia congenita
  • Congenital adrenal hypoplasia
  • X-linked AHC
  • X-linked congenital adrenal hypoplasia
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What are the signs and symptoms of X-linked adrenal hypoplasia congenita?

X-linked adrenal hypoplasia congenita is a disorder that mainly affects males. One of the main signs of this disorder is adrenal insufficiency, which occurs when the adrenal glands do not produce enough hormones. Adrenal insufficiency typically begins in infancy or childhood and can cause vomiting, difficulty with feeding, dehydration, extremely low blood sugar (hypoglycemia), and shock. If untreated, these complications may be life-threatening.[1]

Affected males may also have a shortage of male sex hormones, which leads to underdeveloped reproductive tissues, undescended testicles, delayed puberty, and an inability to father children. Together, these characteristics are known as hypogonadotropic hypogonadism.[1]

The onset and severity of these signs and symptoms can vary, even among affected members of the same family.[1]

Last updated: 8/15/2012

  1. X-linked adrenal hypoplasia congenita. Genetics Home Reference (GHR). 2008; Accessed 8/15/2012.