Autosomal dominant tubulointerstitial disease

ICD-11 code : GB82;

Preferred Label : Autosomal dominant tubulointerstitial disease;

ICD-11 definition : 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.;

ICD-11 synonym : autosomal dominant tubulointerstitial kidney disease NOS; medullary cystic kidney disease; ADTKD - [autosomal dominant tubulointerstitial disease] NOS;

ICD-11 acronym : ADTKD NOS;

ICD-11 inclusion : medullary cystic disease; medullary cystic kidney;

Details

Origin ID

216863438;

Automatic exact mappings (from CISMeF team)
- Autosomal dominant tubulointerstitial kidney disease [ORDO Disease]
- Medullary cystic disease (disorder) [SNOMED CT concept]
- medullary cystic disease of the kidney [SNOMED Notion]
ICD-10 Mapping
- Cystic kidney disease [ICD-10 category]

Keyword's position in hierarchy(ies) :

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