Abstract：
BACKGROUND: Short bowel syndrome (SBS) is a rare but serious form of organ failure, and patients with SBS depend on total parenteral nutrition (PN) to maintain growth and development. The present study aimed to evaluate the experiences and outcomes of children with SBS managed by a multidisciplinary intestinal rehabilitation programme in a tertiary paediatric centre.
METHODS: A retrospective single-centre analysis of all paediatric patients with a clinical diagnosis of SBS between 2001 and 2022 was performed. Clinical outcomes and their predictors were extracted and analysed.
RESULTS: Of the 64 children included in the study, 43 (67%) had extensive necrotising enterocolitis. The median bowel length was 45 cm (interquartile range (IQR) = 18-65) and 18.9% (IQR = 10-28.5) of the expected length based on age. Over a mean follow-up period of 8.9 years, 57 patients (89%) survived, and 50 (78%) weaned off PN. The presence of intestinal failure-associated liver disease (IFALD) (OR = 6.375, p = 0.02) and patients managed before the introduction of fish oil-based PN in 2007 (OR = 5.895, p = 0.001) were significant predictors of mortality. There was an overall improvement in survival over time (p = 0.003). Ultrashort bowel length was not associated with significantly higher mortality (OR = 1.1, p = 0.65) but was a poor prognostic factor for weaning off PN (OR = 3.57, p = 0.004). Among all patients who weaned off PN, two had bowel lengthening procedures and one received a glucagon-like peptide 2 (GLP-2) analogue.
CONCLUSIONS: A multidisciplinary intestinal rehabilitation programme offers a comprehensive approach for patients with SBS and has been shown to be effective with favourable outcomes. Improvements in the choice of PN and the development of new treatment strategies potentially improved the survival and enteral autonomy of SBS patients.
METHODS: III.
METHODS: A retrospective single-centre analysis of all paediatric patients with a clinical diagnosis of SBS between 2001 and 2022 was performed. Clinical outcomes and their predictors were extracted and analysed.
RESULTS: Of the 64 children included in the study, 43 (67%) had extensive necrotising enterocolitis. The median bowel length was 45 cm (interquartile range (IQR) = 18-65) and 18.9% (IQR = 10-28.5) of the expected length based on age. Over a mean follow-up period of 8.9 years, 57 patients (89%) survived, and 50 (78%) weaned off PN. The presence of intestinal failure-associated liver disease (IFALD) (OR = 6.375, p = 0.02) and patients managed before the introduction of fish oil-based PN in 2007 (OR = 5.895, p = 0.001) were significant predictors of mortality. There was an overall improvement in survival over time (p = 0.003). Ultrashort bowel length was not associated with significantly higher mortality (OR = 1.1, p = 0.65) but was a poor prognostic factor for weaning off PN (OR = 3.57, p = 0.004). Among all patients who weaned off PN, two had bowel lengthening procedures and one received a glucagon-like peptide 2 (GLP-2) analogue.
CONCLUSIONS: A multidisciplinary intestinal rehabilitation programme offers a comprehensive approach for patients with SBS and has been shown to be effective with favourable outcomes. Improvements in the choice of PN and the development of new treatment strategies potentially improved the survival and enteral autonomy of SBS patients.
METHODS: III.
摘要：
背景：短肠综合征（SBS）是一种罕见但严重的器官衰竭形式，SBS患者依靠全胃肠外营养(PN)维持生长发育。本研究旨在评估三级儿科中心多学科肠道康复计划管理的SBS儿童的经验和结果。
方法：对2001年至2022年临床诊断为SBS的所有儿科患者进行了回顾性单中心分析。提取并分析临床结果及其预测因子。
结果：在纳入研究的64名儿童中，43（67％）患有广泛的坏死性小肠结肠炎。根据年龄，中位肠长为45厘米（四分位距（IQR）=18-65）和预期长度的18.9％（IQR=10-28.5）。在平均8.9年的随访期内，57例患者（89%）存活，和50（78%）脱离PN。肠衰竭相关肝病（IFALD）的存在（OR=6.375，p=0.02）和2007年引入鱼油PN之前管理的患者（OR=5.895，p=0.001）是死亡率的重要预测因素。随着时间的推移，生存率总体上有所改善（p=0.003）。超短肠长与死亡率无关（OR=1.1，p=0.65），但却是脱机PN的不良预后因素（OR=3.57，p=0.004）。在所有脱离PN的患者中，其中2人接受了肠延长手术,1人接受了胰高血糖素样肽2(GLP-2)类似物.
结论：多学科肠道康复计划为SBS患者提供了一种全面的方法，并已被证明是有效的，具有良好的预后。PN选择的改进和新治疗策略的开发可能改善SBS患者的生存率和肠内自主性。
方法：III.
方法：对2001年至2022年临床诊断为SBS的所有儿科患者进行了回顾性单中心分析。提取并分析临床结果及其预测因子。
结果：在纳入研究的64名儿童中，43（67％）患有广泛的坏死性小肠结肠炎。根据年龄，中位肠长为45厘米（四分位距（IQR）=18-65）和预期长度的18.9％（IQR=10-28.5）。在平均8.9年的随访期内，57例患者（89%）存活，和50（78%）脱离PN。肠衰竭相关肝病（IFALD）的存在（OR=6.375，p=0.02）和2007年引入鱼油PN之前管理的患者（OR=5.895，p=0.001）是死亡率的重要预测因素。随着时间的推移，生存率总体上有所改善（p=0.003）。超短肠长与死亡率无关（OR=1.1，p=0.65），但却是脱机PN的不良预后因素（OR=3.57，p=0.004）。在所有脱离PN的患者中，其中2人接受了肠延长手术,1人接受了胰高血糖素样肽2(GLP-2)类似物.
结论：多学科肠道康复计划为SBS患者提供了一种全面的方法，并已被证明是有效的，具有良好的预后。PN选择的改进和新治疗策略的开发可能改善SBS患者的生存率和肠内自主性。
方法：III.
