GB81

ICD-11 MMS 2026-01

Autosomal dominant polycystic kidney disease

Description

Multiple cysts in both kidneys increasing in number and size from adolescence, associated with development of hypertension and chronic renal failure. Autosomal dominant familial pattern is usual and due to mutations on chromosomes 16 and 4. Non-renal manifestations can include cysts in the liver and less commonly pancreas. Cerebral arterial aneurysms with subarachnoid haemorrhage, and other non-renal vascular abnormalities can also occur.

Index terms

  • Autosomal dominant polycystic kidney disease
  • APCKD - [autosomal polycystic kidney disease]
  • Polycystic kidney, adult type
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  • adult polycystic kidney disease
  • Autosomal dominant polycystic kidney disease type 1 without tuberous sclerosis
  • ADPCKD1 - [autosomal dominant polycystic kidney disease type 1]
  • APCKD1 - [autosomal polycystic kidney disease type 1]
  • Autosomal dominant polycystic kidney disease, Type 2
  • ADPCKD2 - [autosomal dominant polycystic kidney disease type 2]
  • APCKD2 - [autosomal polycystic kidney disease type 2]
  • PKD2 gene - [polycystic kidney disease type 2 gene]

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

Sibling codes

  • GB80 – Nonfamilial nongenetic cystic kidney disease
  • GB82 – Autosomal dominant tubulointerstitial disease
  • GB83 – Nephronophthisis
  • GB8Y – Other specified cystic or dysplastic kidney disease
  • GB8Z – Cystic or dysplastic kidney disease, unspecified

Foundation entity

Autosomal dominant polycystic kidney disease – http://id.who.int/icd/entity/91220434

Part of: 16: Diseases of the genitourinary system