The biliary atresia splenic malformation syndrome: A 28-year single-center retrospective study
The biliary atresia splenic malformation syndrome: A 28-year single-center retrospective study
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DOI：
10.1016/j.jpeds.2006.05.030
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发表时间：
2006-09-01
影响因子：
5.1
通讯作者：
Hadzic, Nedim
中科院分区：
文献类型：
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作者：
Davenport, Mark;Tizzard, Sarah A.;Hadzic, Nedim
我们对胆道闭锁脾脏畸形（BASM）患儿进行了回顾性研究。我们发现，在1977年1月至2004年12月的548例患儿中，有56例（10.2%）符合纳入标准。综合征患儿更可能为女性（P = 0.04），且产前疾病发生率更高（特别是母亲患糖尿病；12.5%对1.2%；P < 0.0001）。21例（37%）患儿存在内脏反位，25例（45%）患儿存在心脏异常。BASM患儿和非综合征患儿在肝脏组织学（例如肝脏纤维化程度）或人类白细胞抗原（HLA）基因型方面没有差异。估计的5年和10年自体肝脏存活率分别为46%和32%。有7例长期存活且保留自体肝脏的患儿，随访时间超过10年；所有患儿均无黄疸。BASM是一个独特的亚组，其发病可能在器官发育的胚胎期。
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 was no difference 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.