X-linked adrenal hypoplasia congenita


X-linked congenital adrenal hypoplasia (medical condition): A genetic disorder which affects the body tissues that produce hormones . It is characterized by underdeveloped adrenal glands which results adrenal insufficiency and hypogonadotrophic hypogonadism.


Adrenal insufficiency Vomiting Feeding difficulty Shock Dehydration Low blood sugar Lack of sex hormones Underdeveloped reproductive organs Undescended testicles Delayed puberty


Adrenal hypofunction occurs when more than 90% of both adrenal glands are destroyed, an occurrence that typically results from an autoimmune process in which circulating antibodies react specifically against the adrenal tissue. Other causes include tuberculosis (once the chief cause; now responsible for less than 10% of adult cases), bilateral adrenalectomy, hemorrhage into the adrenal gland, neoplasms, and infections (acquired immunodeficiency syndrome, histoplasmosis, and cytomegalovirus).


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