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tubulo-interstitiële nefritis bij karyomegalie (aandoening)
tubulo-interstitiële nefritis bij karyomegalie
TIN bij karyomegalie
karyomegalische interstitiële nefritis
Karyomegalic interstitial nephritis
KIN - karyomegalic interstitial nephritis
Systemic karyomegaly
A rare, genetic renal disease characterized by slowly progressive, chronic, tubulointerstitial nephritis, leading to end-stage renal disease before the age of 50 years, manifesting with mild proteinuria, glucosuria and, occasionally, urinary sediment abnormalities (mainly hematuria). Mild extrarenal manifestations, such as recurrent upper respiratory tract infections and abnormal liver function tests, may be associated. Renal biopsy reveals severe, chronic, interstitial fibrosis and tubular changes, as well as hallmark karyomegalic tubular epithelial cells which line the proximal and distal tubules and have enlarged, hyperchromatic nuclei.
Id782738008
StatusPrimitive
Clinical coursechronisch
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetN11.8
TermOverige gespecificeerde chronische tubulo-interstitiële nefritis
SNOMED CT to Orphanet simple map401996
SNOMED CT to ICD-10 extended map
TargetN11.8
RuleTRUE
AdviceALWAYS N11.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified