The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study.
We carried out a retrospective review of infants with biliary atresia splenic malformation (BASM). We found that 56 infants (10.2%) met the criteria for inclusion from a series of 548 infants (from January 1977 to December 2004). Syndromic infants were more likely to be female (P = .04) and to have a higher incidence of antenatal pathology (specifically maternal diabetes; 12.5% vs 1.2%; P < .0001). Situs inversus was noted in 21 (37%) and cardiac abnormalities in 25 (45%) infants. There were no differences in liver histology (eg, degree of liver fibrosis) or in the HLA genotype between BASM and nonsyndromic infants. Five-year and 10-year estimated native liver survival were 46% and 32%, respectively. There were 7 long-term survivors with their native liver and a follow-up of more than 10 years; all were anicteric. BASM is a distinct subgroup, with an implied onset during the embryological phase of organ development.
Davenport
Mark
M
Department of Paediatric Surgery, Kings College Hospital, Denmark Hill, London, UK. Mark.Davenport@kingsch.nhs.uk
Tizzard
Sarah A
SA
Underhill
James
J
Mieli-Vergani
Giorgina
G
Portmann
Bernard
B
Hadzić
Nedim
N
D000015:Abnormalities, Multiple / Q000235:genetics / Q000601:surgery; D001656:Biliary Atresia / Q000150:complications* / Q000235:genetics / Q000601:surgery; D005260:Female; D005838:Genotype; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome; D016896:Treatment Outcome
D006680:HLA Antigens