Hereditary orotic aciduria

Orpha code: 30OMIM code: 258900

Definition

A rare genetic disorder of pyrimidine metabolism characterized by early onset of megaloblastic anemia, global developmental delay, and failure to thrive, associated with massive urinary overexcretion of orotic acid (sometimes with orotic acid crystalluria). Patients without megaloblastic anemia, but with additional manifestations such as epilepsy, have also been reported.

Disease data
Classification

Disease

Synonyms
Orotidylic decarboxylase deficiency
Kwasica orotowa
Niedobór dekarboksylazy orotydyny
Niedobór syntetazy monofosforanu urydyny
Uridine monophosphate synthetase deficiency
ORPHA code
30
OMIM code
258900
ICD10 code
E79.8
ICD11 code
3A03.0

No additional description.

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