GB82

ICD-11 MMS 2026-01

Autosomal dominant tubulointerstitial disease

Description

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 uromodulin, mucin-1, renin and hepatocyte nuclear factor 1-beta. The last is associated with Maturity-Onset Diabetes of the Young (MODY) and thus is classified as MODY-5

Index terms

  • Autosomal dominant tubulointerstitial disease
  • ADTKD - [autosomal dominant tubulointerstitial disease] NOS
  • MCKD [medullary cystic kidney disease]
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  • autosomal dominant tubulointerstitial kidney disease NOS
  • medullary cystic disease
  • medullary cystic kidney
  • medullary cystic kidney disease
  • Autosomal dominant tubulointerstitial disease, Type 1
  • ADTKD-MUC1 [autosomal dominant tubulointerstitial kidney disease MUC1]
  • MCKD - [medullary cystic kidney disease] type 1
  • Mucin-1 Kidney disease
  • autosomal recessive medullary cystic disease
  • medullary cystic kidney disease, Type 1
  • nephronopthisis
  • Autosomal dominant tubulointerstitial disease Type 2
  • ATDKD-UMOD - [Autosomal dominant tubulointerstitial disease-uromodulin]
  • MCKD - [medullary cystic kidney disease] type 2
  • Uromodulin associated kidney disease
  • Uromodulin kidney disease
  • medullary cystic kidney disease, Type 2

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 22 – Injury, poisoning or certain other consequences of external causes

Foundation children elsewhere

  • LB30.8 – Medullary sponge kidney

Sibling codes

  • GB80 – Nonfamilial nongenetic cystic kidney disease
  • GB81 – Autosomal dominant polycystic kidney disease
  • GB83 – Nephronophthisis
  • GB8Y – Other specified cystic or dysplastic kidney disease
  • GB8Z – Cystic or dysplastic kidney disease, unspecified

Foundation entity

Autosomal dominant tubulointerstitial disease – http://id.who.int/icd/entity/216863438

Part of: 16: Diseases of the genitourinary system