Autosomal recessive polycystic kidney disease

Preferred Label : Autosomal recessive polycystic kidney disease;

ICD-11 definition : Autosomal recessive polycystic kidney disease (ARPKD) is an inherited condition characterised by multiple cysts affecting the collecting ducts giving rise to nephromegaly, arterial hypertension, urinary tract infection and chronic kidney disease (failure). Liver involvement (biliary abnormalities, cholangitis, portal hypertension) are frequently associated.;

ICD-11 synonym : infantile polycystic kidney disease; IPKD - [infantile polycystic kidney disease]; autosomal recessive polycystic kidney; ARPKD - [autosomal recessive polycystic kidney disease]; Polycystic kidney, infantile type; polycystic kidney disease, infantile type; multiple infantile type cyst of kidney; infantile type polycystic degeneration of kidney; autosomal recessive infantile polycystic kidney disease;

ICD-11 acronym : IPKD; ARPKD;

ICD-11 inclusion : Polycystic kidney, infantile type;

Details

Origin ID

1424110943;

Automatic exact mappings (from CISMeF team)
- Autosomal Recessive Polycystic Kidney Disease [NCIt concept]
- Autosomal recessive polycystic kidney disease [ORDO Disease]
- PKHD1 ciliary IPT domain containing fibrocystin/polyductin [ORDO Gene]
- PKHD1 wt Allele [NCIt concept]
- Polycystic kidney disease 4 with or without polycystic liver disease [OMIM Phenotype]
- Polycystic kidney disease, infantile type (disorder) [SNOMED CT concept]
- Polycystic kidney, autosomal recessive [MedDRA LLT]
- Polycystic kidney, autosomal recessive [ICD-9 code]
- Polycystic kidney, infantile type [ICD-10 Sub-category]
- autosomal recessive polycystic kidney disease [DO Concept]
- polycystic kidney disease, infantile type [SNOMED Notion]
- polycystic kidney, autosomal recessive [MeSH Descriptor]
- polycystic kidney, autosomal recessive [MeSH concept]

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