Phosphoribosylpyrophosphate synthetase superactivity

Orpha code: 3222OMIM code: 300661

Definition

A rare X-linked disorder of purine metabolism associated with hyperuricemia and hyperuricosuria, and comprised of two forms: an early-onset severe form characterized by gout, urolithiasis, and neurodevelopmental anomalies and a mild late-onset form with no neurologic involvement.

Disease data
Classification

Disease

Synonyms
PRPP synthetase superactivity
Nadczynność syntetazy PRPP
PRPS1 superactivity
PRPS1 superactivity
ORPHA code
3222
OMIM code
300661
ICD10 code
E79.8
ICD11 code
5C55.0Y

No additional description.

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