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congenitaal defect van galzuursynthese type 3 (aandoening)
congenitaal defect van galzuursynthese type 3
aangeboren galzuursynthesedefect type 3
Congenital bile acid synthesis defect type 3
Oxysterol 7-alpha hydroxylase deficiency
Congenital bile acid synthesis defect type 3 (BAS defect type 3) is a severe anomaly of bile acid synthesis characterized by severe neonatal cholestatic liver disease.
Id719454003
StatusPrimitive
Occurrencecongenitaal
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetK76.8
TermOverige gespecificeerde leverziekten
SNOMED CT to Orphanet simple map79302
SNOMED CT to ICD-10 extended map
TargetK76.8
RuleTRUE
AdviceALWAYS K76.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified