ICD-11 classes
16 Diseases of the genitourinary system
Diseases of the urinary system
Cystic or dysplastic kidney disease
GB82 — Autosomal dominant tubulointerstitial disease

ICD-11 GB82 — Autosomal dominant tubulointerstitial disease

Nonglomerular, autosomal dominant kidney diseases characterised by progressive tubulointerstitial fibrosis and progression to end-stage renal disease. Currently there are 4 known genetic defects — in oromudulin, mucin-1, renin and hepatocyte nuclear factor 1-ß. The last is associated with Maturity-Onset Diabetes of the Young (MODY) and thus is classified as MODY-5.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code GB82 contains 1 clarifying diagnosis:

1. LB30.8 — Medullary sponge kidney