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Hypoxanthine guanine phosphoribosyltransferase partial deficiency

ORPHA:79233DiseaseX-linked recessiveAll ages

Ассоциированные гены (1)

HPRT1
hypoxanthine phosphoribosyltransferase 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 308000

Фенотипы (18)

Частый (30–79%)5
HP:0000112Nephropathy
HP:0001249Intellectual disability
HP:0001332Dystonia
HP:0002149Hyperuricemia
HP:0003149Hyperuricosuria
Периодический (5–29%)12
HP:0000083Renal insufficiency
HP:0000707Abnormality of the nervous system
HP:0000791Uric acid nephrolithiasis
HP:0001250Seizure
HP:0001263Global developmental delay
HP:0001347Hyperreflexia
HP:0001919Acute kidney injury
HP:0001997Gout
HP:0002071Abnormality of extrapyramidal motor function
HP:0003259Elevated circulating creatinine concentration
HP:0012587Macroscopic hematuria
HP:0100518Dysuria
Исключён (0%)1
HP:0000742Self-mutilation

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы