Hereditary orotic aciduria

Orpha code: 30OMIM code: 258900

Definicja

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
Klasyfikacja

Disease

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

No additional description.

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