Biliary atresia with splenic malformation syndrome
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 April 2024
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Biliary atresia with splenic malformation syndrome?
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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.
BARE
Our mission is to drive research and education for better outcomes in treatment and management within the Biliary Atresia community.
Global Liver Institute
To improve the lives of individuals and families impacted by liver disease through promoting innovation, encouraging collaboration, and scaling optimal approaches to help eradicate liver diseases.
Help Hope Live
Help Hope Live assists individuals living with catastrophic injuries and illnesses to fundraise toward their medical expenses and related costs.
Syndromes Without A Name (SWAN) Australia
Provide information, support and advocacy to families caring for a child with an undiagnosed or rare genetic condition.
Clinical Trials
For a list of clinical trials in this disease area, please click here.