Xanthinuria type I

Orpha code: 93601OMIM code: 278300

Definition

Type I xanthinuria, a type of classical xanthinuria (see this term), is a rare autosomal recessive disorder of purine metabolism (see this term) characterized by the isolated deficiency of xanthine dehydrogenase, causing hyperxanthinemia with low or absent uric acid and xanthinuria, leading to urolithiasis, hematuria, renal colic and urinary tract infections, while some patients are asymptomatic and others suffer from kidney failure. Less common manifestations include arthropathy, myopathy and duodenal ulcer.

Disease data
Classification

Etiological subtype

Synonyms
XDH deficiency
Niedobór dehydrogenazy ksantynowej
Niedobór oksydazy ksantynowej
Niedobór oksydoreduktazy ksantynowej
Niedobór XDH
Niedobór XO
Niedobór XOR
XO deficiency
XOR deficiency
Xanthine dehydrogenase deficiency
Xanthine oxidase deficiency
Xanthine oxidoreductase deficiency
ORPHA code
93601
OMIM code
278300
ICD10 code
E79.8
ICD11 code
-

No additional description.

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