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atresie van galwegen met malformatiesyndroom van milt (aandoening)
atresie van galwegen met malformatiesyndroom van milt
BASM-syndroom
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.
Id717156002
StatusPrimitive
Associated morphologymorfologische afwijking
Finding sitestructuur van milt
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologyatresie
Finding sitestructuur van galweg
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
DHD Diagnosis thesaurus reference set
SNOMED CT to Orphanet simple map244283
SNOMED CT to ICD-10 extended map
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