Short bowel syndrome (SBS) is a rare condition characterised by extensive loss of intestinal mass secondary to congenital or acquired disease. The outcomes are determined by dependency on parenteral nutrition (PN), its possible complications and factors that influence intestinal adaptation. In order to achieve the best results, patients should be managed by a specialised multidisciplinary team with the aims of promoting growth and development, stimulating intestinal adaptation and preventing possible complications. This involves timely surgical management aimed at rescuing maximum bowel length and eventually re-establishing intestinal continuity where appropriate. A combination of enteral and parenteral nutrition needs to be targeted towards maintaining a balance between fulfilling the nutritional and metabolic needs of the child while preventing or at least minimising potential complications. Enteral nutrition and establishment of oral feeding play a fundamental role in stimulating bowel adaptation and promoting enteral autonomy. Other measures to promote enteral autonomy include the chyme recycling in patients where bowel is not in continuity, autologous gastrointestinal reconstruction and pharmacological treatments, including promising new therapies like teduglutide. Strategies such as lipid reduction, changing the type of lipid emulsion and cycling PN are associated with a reduction in the rates of intestinal failure–associated liver disease. Even though vast improvements have been made in the surgical and medical management of SBS, there is still lack of consensus in many aspects and collaboration is essential.

中文翻译：

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婴儿期短肠综合征：最新进展和实际管理


短肠综合征 (SBS) 是一种罕见疾病，其特征是继发于先天性或后天性疾病的肠道质量大量损失。结果取决于对肠外营养（PN）的依赖、其可能的并发症以及影响肠道适应的因素。为了达到最佳效果，患者应由专门的多学科团队管理，旨在促进生长发育、刺激肠道适应并预防可能的并发症。这涉及及时的手术治疗，旨在挽救最大肠道长度并最终在适当的情况下重新建立肠道连续性。肠内和肠外营养的结合需要旨在保持满足儿童的营养和代谢需求与预防或至少最大限度地减少潜在并发症之间的平衡。肠内营养和建立经口喂养对于刺激肠道适应和促进肠自主权发挥着基础性作用。促进肠内自主性的其他措施包括肠道不连续的患者的食糜回收、自体胃肠重建和药物治疗，包括有前途的新疗法，如替度鲁肽。降脂、改变脂肪乳剂类型和循环 PN 等策略可降低肠衰竭相关肝病的发生率。尽管 SBS 的手术和医疗管理已经取得了巨大进步，但在许多方面仍然缺乏共识，合作至关重要。