Long-term outcomes of various pediatric short bowel syndrome in China

Long-term outcomes of various pediatric short bowel syndrome in China
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中国各种儿童短肠综合征的长期结局

DOI:
10.1007/s00383-020-04797-8
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发表时间:
2021-01-04
影响因子:
1.8
通讯作者:
Cai, Wei
Cai, Wei
中科院分区:
医学3区
文献类型:
--
作者:
Zhang, Tian;Feng, Haixia;Cai, Wei

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目的本研究的目的是分析各种儿童短肠综合征(SBS)在中国肠道康复中心的长期结果。方法157例SBS儿童参加了这项研究,从1988年10月至2019年7月。根据发病年龄、肠外营养(PN)持续时间和短肠解剖类型分别分析其长期随访结果。的临床特征,其中包括人口统计学,残留小肠的长度,PN的持续时间,PN依赖,SBS相关的并发症,如IF相关的肝脏疾病(IFALD),导管相关的血流感染(CRBI),和死亡率进行了比较groups.ResultsThe SBS的主要病因是肠闭锁,NEC和肠扭转。157例患者中有5例未停用PN。IFALD和CRBI的发生率分别为24.2%和22.3%。16例患者因感染和肝功能衰竭死亡,8例患者失访。157例患者的生存率为84.7%。婴幼儿组PN持续时间较长(284 ± 457 d vs.110 ± 64 d,P= 0.021;R= 0.264,P = 0.001),未脱离PN者多于新生儿组(11.6% vs.0,P= 0.001;R= 0.295,P < 0.001)。PN时间> 90 d的患者CRBI发生率较高(30.6%,P = 0.025;R= 0.236,P = 0.003)。有吻合口的患者CRBI发生率较高(30.0%,P = 0.032)。各组之间的死亡率没有差异。在五个PN依赖患者,没有SBS发生在news.Conclusionpediatric SBS患者可以实现良好的长期生存和肠内自主性。不同的SBS分类标准,如发病年龄、PN持续时间和短肠的解剖类型,对儿童SBS的总体死亡率没有影响。PN持续时间延长与发病年龄和CRBI发生率呈正相关。肠道完整者CRBI发生率低。
PurposeThe goal of this study was to analyze long-term outcome of various pediatric short bowel syndrome (SBS) at an intestinal rehabilitation center in China.MethodsOne hundred and fifty-seven children with SBS were enrolled in this study from October 1988 to July 2019. Their long-term follow-up outcome was analyzed according to the age of disease onset, parenteral nutrition (PN) duration, and anatomic types of short bowel, respectively. The clinical characteristics, which included demographics, the length of residual small bowel, PN duration, PN dependence, SBS-related complications such as IF-related liver disease (IFALD), catheter-related bloodstream infection (CRBI), and mortality were compared among the groups.ResultsThe main etiology for SBS were intestinal atresia, NEC, and volvulus. Five of 157 patients did not wean off PN. The incidence of IFALD and CRBI was 24.2 and 22.3%, respectively. Sixteen cases died because of infection and liver failure and eight patients lost to follow-up. The survival rate of the 157 patients was 84.7%. PN duration was longer in the infants and children group (284 ± 457 d vs. 110 ± 64 d,P= 0.021;R= 0.264,P= 0.001) and more patients did not wean off PN than in the neonates group (11.6% vs. 0,P= 0.001;R= 0.295,P< 0.001). Patients with PN with a duration of longer than 90 days had more CRBIs (30.6%,P= 0.025;R= 0.236,P= 0.003). Additionally, the rate of CRBI was higher in patients with stoma (30.0%,P= 0.032). There was no difference in mortality among the groups. In five PN dependence patients, none was SBS onset in neonates.ConclusionPediatric patients with SBS could achieve favorable long-term survival and enteral autonomy. Different standards of SBS classification such as the age of disease onset, PN duration, and anatomic types of short bowel did not impact the overall mortality of pediatric SBS. Prolonged PN duration positively correlated with the age of disease onset and the incidence of CRBI. Patients with the complete continuity of intestinal tract suffered less from CRBI.