ICD-11 classes
16 Diseases of the genitourinary system
Diseases of the urinary system
Cystic or dysplastic kidney disease
GB83 — Nephronophthisis

ICD-11 GB83 — Nephronophthisis

Autosomal recessive disease characterised by polyuria, polydipsia, enuresis and chronic kidney disease with end stage renal failure occurring between birth and late adolescence depending on the NPHP gene involved. Extra-renal manifestations occur with associated multisystem genetic disorders (e.g. Senior-Loken, Cogan, Joubert)

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.