oligomeganephronia

ORPHA: 2260

Overview

renal hypoplasia characterized by bilateral reduced kidney size with a marked decrease in the total number of nephrons

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 oligomeganephronia, sourced from HPO and Orphanet clinical annotations.

Renal insufficiencyProteinuriaElevated circulating creatinine concentrationDecreased numbers of nephronsDecreased glomerular filtration rateAbnormal nephron morphologyBilateral renal hypoplasiaGlomerulomegalyRenal tubular atrophySmall for gestational agePremature birthStage 5 chronic kidney diseaseAbnormal renal cortex morphologyAbnormal medullary pyramid morphologyDehydrationPolydipsiaUnilateral renal agenesisAbnormality of the faceMicrognathiaHearing impairmentOptic disc colobomaCongenital diaphragmatic herniaHypertensionSeizureSecundum atrial septal defectPulmonary hypoplasiaPulmonary venous occlusionBranchial cystNeurodevelopmental delayAbnormality of limbs

Classification & Codes

Orphanet Code

ORPHA:2260
oligomeganephronia
OrphanetORPHA:2260
Treatments0 drug(s)
Symptoms on record30 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO