Article
Unusual presentation of biliary atresia splenic malformation syndrome with autosomal dominant hypospadias.
Genetic counseling (Geneva, Switzerland) - 1 Jan 2011
Tumgor G, Cogulu O, Onay H, Ekmekci A Y, Aydogdu S, Durmaz B, Kilic M, Ozkinay F
Abstract excerpt
Biliary atresia is associated with polysplenia in 2-10% of cases and is defined as Biliary Atresia Splenic Malformation syndrome (BASM). The main features of BASM syndrome include extrahepatic biliary atresia and polysplenia besides the characteristic findings of laterality anomalies, cardiac anomalies, intraabdominal vascular anomalies, pancreatic anomalies and malrotation. Here we present a 6-month-old male...
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