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APECED Syndrome

APECED is short for autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.

Autoimmune polyendocrine syndromes are a heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organs, although non-endocrine organs can be affected.

APECED Syndrome is a genetic autoimmune disease. It is usually characterized by at least two of the following.

  • adrenal insufficiency
  • hypoparathyroidism
  • candidiasis

APECED Syndrome is broken down into two types.

Type I affects children and adults younger than age 35. It is characterized by below normal secretion of the parathyroid gland and the failure of the adrenal cortex to secrete normal volumes of hormones. About 60% of women and about 15% of men fail to mature sexually (hypogonadism). A persistent yeast infection ( candidiasisis) is common.

Type II more frequently strikes adults with peak incidence at about 30 years. Almost invariably it involves the adrenal cortex with thyroid involvement somewhat less frequent. It may also involve the pancreatic islets producing an insulin-dependent diabetes mellitus.

 

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