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Biliary atresia with splenic malformation syndrome (disorder)
Biliary atresia with splenic malformation syndrome
BASM syndrome
The association of biliary atresia and splenic abnormalities. Cardiac defect, situs inversus and a preduodenal portal vein can also be present. It represents the embryonal or syndromic form of biliary atresia. It affects newborns or infants and is characterized by jaundice, pale stools, dark urine, failure to thrive, hepatomegaly, coagulopathy, anemia and often palpable spleen.
atresie van galwegen met malformatiesyndroom van milt
BASM-syndroom
Id717156002
StatusPrimitive
Associated morphologyCongenital atresia
Finding siteBile duct structure
OccurrenceCongenital
Pathological processPathological developmental process
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetQ44.2
RuleTRUE
AdviceALWAYS Q44.2
CorrelationSNOMED CT source code to target map code correlation not specified
TargetQ89.0
RuleTRUE
AdviceALWAYS Q89.0
CorrelationSNOMED CT source code to target map code correlation not specified