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Адреноміодистрофія

ORPHA:977· ICD-10 E27.4· Adrenomyodystrophy

Визначення(English summary)

An extremely rare genetic endocrine disease characterized by primary adrenal insufficiency, dystrophic myopathy, hepatic steatosis, severe psychomotor delay, megalocornea, failure to thrive, chronic constipation, and terminal bladder ectasia which can lead to death. There have been no further descriptions in the literature since 1982.

Поширеність
<1 / 1 000 000
Успадкування
Unknown
Вік початку
Infancy, Neonatal