cystinosis

MeSH: D003554ORPHA: 213

Overview

lysosomal storage disease characterized by the abnormal accumulation of cystine in lysosomes

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 cystinosis, sourced from HPO and Orphanet clinical annotations.

ProteinuriaNephropathyRenal tubular dysfunctionPhotophobiaHypothyroidismDelayed pubertyMuscle weaknessFailure to thriveDehydrationPolydipsiaRenal Fanconi syndromeVomitingHypophosphatemiaHypokalemiaMyopathyAminoaciduriaShort statureCorneal opacityNephrogenic diabetes insipidusFatigueType I diabetes mellitusRenal insufficiencyNephrocalcinosisHypogonadismRetinopathyBand keratopathyNephrolithiasisGrowth delaySplenomegalyMetabolic acidosisHepatomegalyRicketsOsteomalaciaHypocalcemiaGlycosuriaHyperphosphaturiaElevated circulating alkaline phosphatase concentrationEMG: myopathic abnormalitiesFeeding difficultiesAciduriaAzoospermiaVisual impairmentHypotoniaMild intellectual disabilityGait disturbancePortal hypertensionExocrine pancreatic insufficiencyFeverGlucose intoleranceConstipationMalabsorptionAphasiaHyponatremiaDecreased circulating carnitine concentrationCranial nerve paralysisAbnormality of endocrine pancreas physiology

Classification & Codes

MeSH Code

D003554

Orphanet Code

ORPHA:213
cystinosis
MeSHD003554
OrphanetORPHA:213
Treatments0 drug(s)
Symptoms on record56 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO