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Infantile nephronophthisis

ORPHA:93591· ICD-10 Q61.5

Definition

A rare clinical variant of hereditary nephronophthisis characterized by reduced renal concentrating ability, chronic tubulointerstitial nephritis, cystic renal disease, and progression to end-stage renal disease (ESRD) before 3 years of age.

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Antenatal, Childhood, Infancy, Neonatal