blauwe sclera	congenitale afwijking van urinewegstelsel	congenitale hydrocefalie	malformatiesyndroom met betrokkenheid van meerdere systemen
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syndroom van hydrocefalie, blauwe sclera en nefropathie (aandoening)
	syndroom van hydrocefalie, blauwe sclera en nefropathie
	syndroom van Daentl-Townsend-Siegel syndroom van waterhoofd, blauwe harde oogrok en nierziekte syndroom van hydrocephalus, blauwe sclera en nephropathie Daentl-Townsend-Siegel-syndroom
	Hydrocephalus, blue sclera, nephropathy syndrome
	Daentl Townsend Siegel syndrome
	A rare genetic renal or urinary tract malformation syndrome with characteristics of nephrotic syndrome with focal segmental sclerosis associated with hydrocephalus, thin skin and blue sclerae. There have been no further descriptions in the literature since 1978.

Id	773280009
Status	Primitive

Associated morphology	morfologische afwijking
Finding site	structuur van sclera
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	dilatatie
Finding site	structuur van liquorcirculatie in hersenen
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	morfologische afwijking
Finding site	structuur van urinewegstelsel
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q87.8
Term	Overige gespecificeerde congenitale gestoorde-ontwikkelingssyndromen, niet elders geclassificeerd

SNOMED CT to Orphanet simple map

2186

SNOMED CT to ICD-10 extended map

Target	Q87.8
Rule	TRUE
Advice	ALWAYS Q87.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified