severe phosphoribosylpyrophosphate synthetase superactivity

ORPHA: 411543

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with severe phosphoribosylpyrophosphate synthetase superactivity, sourced from HPO and Orphanet clinical annotations.

Uric acid nephrolithiasisGoutHyperuricemiaHyperuricosuriaIncreased phosphoribosylpyrophosphate synthetase levelNeurodevelopmental abnormalityRenal insufficiencySensorineural hearing impairmentAbnormality of the nervous systemIntellectual disabilityHypotoniaArthritisCrystalluriaAtaxiaAcute kidney injuryMotor polyneuropathyPeripheral neuropathyGlaucomaMyopiaGlobal developmental delayRecurrent respiratory infections

Classification & Codes

Orphanet Code

ORPHA:411543
severe phosphoribosylpyrophosphate synthetase superactivity
OrphanetORPHA:411543
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
severe phosphoribosylpyrophosphate synthetase superactivity | OrphanDrug