Renal dysplasia, unilateral
ORPHA:93172· ICD-10 Q61.4
Definition
A form of renal dysplasia (RD) characterized by abnormal or incomplete development of one kidney. Unilateral RD can be segmental, and of variable severity, with renal aplasia corresponding to extreme RD. Patients may be asymptomatic if the contralateral kidney is functional. Even in cases of severe unilateral RD, i.e. renal aplasia, the risk of renal failure in childhood is minimal; however, patients may develop hypertension, proteinuria and renal failure as adults.
- Prevalence
- 1-5 / 10 000
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- All ages