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Hereditary orotic aciduria

ORPHA:30DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

UMPS
uridine monophosphate synthetase
Disease-causing germline mutation(s) (loss of function) in
OMIM: 613891

Фенотипы (16)

Очень частый (80–99%)5
HP:0001263Global developmental delay
HP:0001903Anemia
HP:0003218Oroticaciduria
HP:0003355Aminoaciduria
HP:0003526Orotic acid crystalluria
Частый (30–79%)11
HP:0000069Abnormality of the ureter
HP:0000316Hypertelorism
HP:0000431Wide nasal bridge
HP:0000494Downslanted palpebral fissures
HP:0001385Hip dysplasia
HP:0001643Patent ductus arteriosus
HP:0001744Splenomegaly
HP:0002205Recurrent respiratory infections
HP:0008388Abnormal toenail morphology
HP:0011840Abnormality of T cell physiology
HP:0000358Posteriorly rotated ears

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы