orotic aciduria

ICD-10: D53.0MeSH: C537136ORPHA: 30

Overview

pyrimidine metabolic disorder that is characterized by an excessive secretion of orotic acid in urine

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 orotic aciduria, sourced from HPO and Orphanet clinical annotations.

Global developmental delayAnemiaOroticaciduriaAminoaciduriaOrotic acid crystalluriaAbnormality of the ureterHypertelorismWide nasal bridgeDownslanted palpebral fissuresHip dysplasiaPatent ductus arteriosusSplenomegalyRecurrent respiratory infectionsAbnormal toenail morphologyAbnormal T cell physiologyPosteriorly rotated ears

Classification & Codes

ICD-10 Code

D53.0

MeSH Code

C537136

Orphanet Code

ORPHA:30
orotic aciduria
ICD-10D53.0
MeSHC537136
OrphanetORPHA:30
Treatments0 drug(s)
Symptoms on record16 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO