Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A form of autosomal dominant tubulointerstitial kidney disease (ADTKD) due to variants in or whole gene deletions of <i>HNF1B</i>, which is characterized by chronic tubulo-interstitial nephritis, that manifests with nonsignificant urinalysis and slowly progressive renal failure. It can be associated with cystic kidney dysplasia, early onset diabetes and extrarenal manifestations. Disease data Classification Clinical subtype Synonyms ADTKD-HNF1B Dysfunkcja nerek - cukrzyca o wczesnym poczatku MODY5 Torbiele nerek - MODY Zespół RCAD Autosomalna dominująca kanalikowo-śródmiąższowa choroba nerek związana z HNF1B HNF1B-MODY HNF1B-related nephropathy MODY5 Maturity-onset diabetes of the young type 5 RCAD syndrome Renal cysts and diabetes syndrome Renal dysfunction-early-onset diabetes syndrome ORPHA code 93111 OMIM code 137920 ICD10 code N11.8 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl