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candidiasis and endocrinopathy syndrome

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This is an autosomal recessive polyglandular deficiency state characterised by childhood onset and defective cellular immunity to Candida albicans which is refractory to conventional chemotherapy; however remission is reported with ketoconazole plus transfer factor. There is no HLA linkage.

The autoimmune element comprises:

  • hypoparathyroidism is common
  • diabetes is rare
  • adrenal insufficiency may develop acutely
  • pernicious anaemia
  • chronic active hepatitis
  • membranoproliferative glomerulonephritis
  • sprue

The associated disorders must be managed conventionally even if the Candida is successfully treated.

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