Cystathioninuria

Orpha code: 212OMIM code: 219500

Definition

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.

Disease data
Classification

Disease

Synonyms
Cystathionase deficiency
Niedobóe gamma-cystationazy
Niedobór cystationazy
Niedobór liazy gamma - cystationu
Cystathionine gamma-lyase deficiency syndrome
Gamma-cystathionase deficiency
ORPHA code
212
OMIM code
219500
ICD10 code
E72.1
ICD11 code
5C50.B

No additional description.

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