Autosomal recessive hereditary disorder	Congenital anomaly of central nervous system	Congenital anomaly of the kidney	Congenital hepatic fibrosis	Developmental hereditary disorder	Digestive system hereditary disorder	Hereditary disorder of nervous system	Hereditary nephropathy	Multiple congenital cysts of kidney	Multiple system malformation syndrome
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Nephrocystin 3-related Meckel-like syndrome (disorder)
	NPHP3-related Meckel-like syndrome
	NPHP3 (nephrocystin 3) related Meckel-like syndrome Meckel syndrome type 7 Renal, hepatic, pancreatic dysplasia, Dandy-Walker cysts syndrome Meckel-like syndrome type 1 Goldston syndrome Nephrocystin 3-related Meckel-like syndrome
	NPHP3-related Meckel-like syndrome is a rare, genetic, syndromic renal malformation characterized by cystic renal dysplasia with or without prenatal oligohydramnios, central nervous system abnormalities (commonly Dandy-Walker malformation), congenital hepatic fibrosis, and absence of polydactyly.

Id	773737004
Status	Primitive

Associated morphology	Morphologically abnormal structure
Finding site	Structure of central nervous system
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Fibrosis
Finding site	Liver structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Polycystic change
Finding site	Kidney structure
Occurrence	Congenital
Pathological process	Pathological developmental process

SNOMED CT to Orphanet simple map

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q61.9
Term	Cystische nierziekte, niet gespecificeerd

SNOMED CT to ICD-10 extended map

Target	Q61.9
Rule	TRUE
Advice	ALWAYS Q61.9 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified