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...

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Bibliographic Details
Published in:The Journal of pediatrics 2006-09, Vol.149 (3), p.393-400
Main Authors: Davenport, Mark, Tizzard, Sarah A., Underhill, James, Mieli-Vergani, Giorgina, Portmann, Bernard, Hadžić, Nedim
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - genetics
Abnormalities, Multiple - surgery
Biliary Atresia - complications
Biliary Atresia - genetics
Biliary Atresia - surgery
Biological and medical sciences
Female
General aspects
Genotype
HLA Antigens - genetics
Humans
Infant
Male
Medical sciences
Miscellaneous
Public health. Hygiene
Public health. Hygiene-occupational medicine
Retrospective Studies
Spleen - abnormalities
Syndrome
Treatment Outcome
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Summary: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.
ISSN:0022-3476
1097-6833
DOI:10.1016/j.jpeds.2006.05.030