Biliary atresia with splenic malformation syndrome

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Synonyms: BASM syndrome

Biliary atresia with splenic malformation syndrome (BASM) designates the association of biliary atresia (see this term) and splenic abnormalities (mainly polysplenia and less frequently asplenia double spleen). 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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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Advocacy Organizations

BA HERO Inc.

Our mission at BA HERO is to fund research programs to help find a cause, cure or alternative treatments for Biliary Atresia while also connecting and empowering the global BA community.

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Clinical Trials

For a list of clinical trials in this disease area, please click here.