Hyperprolinemia type 1

Orpha code: 419OMIM code: 239500

Definition

A rare disorder of proline metabolism characterized biochemically by markedly elevated levels of proline in plasma and urine due to deficiency of proline oxidase. The reported clinical phenotype ranges from asymptomatic to variable neurologic and psychiatric manifestations (including global developmental delay, seizures, autistic features, and hyperactivity).

Disease data
Classification

Disease

Synonyms
Proline oxidase deficiency
Niedobór oksydazy prolinowej
ORPHA code
419
OMIM code
239500
ICD10 code
E72.5
ICD11 code
5C50.8

No additional description.

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