Abnormal blue sclerae	Congenital hydrocephalus	Congenital malformation of the urinary system	Multiple system malformation syndrome
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Hydrocephalus, blue sclera, nephropathy syndrome (disorder)
	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.
	syndroom van hydrocefalie, blauwe sclera en nefropathie
	syndroom van waterhoofd, blauwe harde oogrok en nierziekte syndroom van hydrocephalus, blauwe sclera en nephropathie Daentl-Townsend-Siegel-syndroom syndroom van Daentl-Townsend-Siegel

Id	773280009
Status	Primitive

Associated morphology	Morphologically abnormal structure
Finding site	Scleral structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Dilatation
Finding site	Brain cerebrospinal fluid pathway
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Urinary system structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

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