Dent disease

ICD-10: N39.8MeSH: D057973ORPHA: 1652

Overview

congenital disorder of urinary system

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 Dent disease, sourced from HPO and Orphanet clinical annotations.

Renal insufficiencyRenal tubular atrophyProteinuriaFocal segmental glomerulosclerosisProximal tubulopathyRenal phosphate wastingNephrocalcinosisCataractNephrolithiasisHematuriaHypotoniaMild intellectual disabilityAbdominal painHypercalciuriaBone painDelayed epiphyseal ossificationRicketsRecurrent fracturesAbnormality of the lower limbOsteomalaciaSparse bone trabeculaeThin bony cortexAminoaciduriaElevated circulating creatine kinase concentrationBowing of the legsBulging epiphysesEnlarged epiphysesEnlargement of the wristsMetaphyseal irregularityEnlargement of the anklesGlycosuriaHyperphosphaturiaLow-molecular-weight proteinuriaHyperuricosuriaTubulointerstitial fibrosisNon-acidotic proximal tubulopathyChronic kidney diseaseRenal hypophosphatemiaMild global developmental delayHigh serum calcitriol

Classification & Codes

ICD-10 Code

N39.8

MeSH Code

D057973

Orphanet Code

ORPHA:1652
Dent disease
ICD-10N39.8
MeSHD057973
OrphanetORPHA:1652
Treatments0 drug(s)
Symptoms on record40 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO