Autosomal recessive hereditary disorder	Chronic tubulointerstitial nephritis	Connective tissue hereditary disorder	Hereditary nephropathy	Renal interstitial fibrosis
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Karyomegalic interstitial nephritis (disorder)
	Karyomegalic interstitial nephritis
	KIN - karyomegalic interstitial nephritis Systemic karyomegaly
	A rare genetic renal disease with characteristics of slowly progressive chronic tubulointerstitial nephritis leading to end-stage renal disease before the age of 50 years. The disease manifests mild proteinuria, glucosuria and occasionally urinary sediment abnormalities. 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. Caused by homozygous or compound heterozygous mutation in the FAN1 gene on chromosome 15q.
	tubulo-interstitiële nefritis bij karyomegalie
	TIN bij karyomegalie karyomegalische interstitiële nefritis

Id	782738008
Status	Primitive

Associated morphology	Chronic inflammation
Finding site	Structure of interstitial tissue of kidney

Associated morphology	Chronic inflammation
Finding site	Renal tubule structure

Associated morphology	Fibrosis
Finding site	Structure of interstitial tissue of kidney

Clinical course

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	N11.8
Rule	TRUE
Advice	ALWAYS N11.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified