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Цистатіонінурія

ORPHA:212· ICD-10 E72.1· Cystathioninuria

Визначення(English summary)

A rare inborn error of metabolism characterized by abnormal accumulation of plasma cystathionine and subsequent increased urinary excretion due to cystathionine gamma-lyase deficiency. The condition is considered benign without pathological relevance. Mode of inheritance is autosomal recessive.

Поширеність
1-9 / 100 000
Успадкування
Autosomal recessive
Вік початку
All ages