Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH 44195, USA.
Paediatr Drugs. 2012 Jun 1;14(3):179-88. doi: 10.2165/11594880-000000000-00000.
Short bowel syndrome (SBS) reflects a state of malabsorption that occurs due to loss of a significant portion of the small bowel. The pathophysiology of SBS is determined largely by the process of adaptation, which is the innate attempt by the remnant portions of the intestine to increase fluid and nutrient reabsorption. In recent years, emphasis has been placed on intestinal rehabilitation with multidisciplinary teams as a comprehensive approach to the management of patients with SBS. In our institution, the multidisciplinary team members include pediatric gastroenterologists, pediatric surgeons, pediatric dieticians, physical therapists, occupational therapists, neonatologists (especially for patients still under their care), transplant surgeons, transplant coordinators and social workers. Parenteral nutrition plays a significant role in the management of SBS, but its use is associated with many potential complications, including cholestatic liver disease. Fish oil-based lipid emulsions have shown promise in their ability to reverse and also prevent the development of cholestasis in these patients. Clinical trials have shown that growth factors and other trophic hormones facilitate the process of adaptation. The most significant impact has been shown with the use of glucagon-like peptide-2 and its analog (teduglutide). Surgical interventions remain an important part of the management of SBS to facilitate adaptation and treat complications. Intestinal transplantation is a last resort option when the process of adaptation is unsuccessful. This review article is intended to provide an overview of the conventional and emerging therapies for pediatric SBS.
短肠综合征(SBS)反映了一种吸收不良的状态,这种状态是由于小肠的大部分丢失而发生的。SBS 的病理生理学主要由适应过程决定,这是肠道残余部分增加液体和营养吸收的固有尝试。近年来,强调多学科团队的肠道康复作为 SBS 患者管理的综合方法。在我们的机构中,多学科团队成员包括儿科胃肠病学家、儿科外科医生、儿科营养师、物理治疗师、职业治疗师、新生儿科医生(特别是仍在他们照顾下的患者)、移植外科医生、移植协调员和社会工作者。肠外营养在 SBS 的管理中起着重要作用,但它的使用与许多潜在的并发症有关,包括胆汁淤积性肝病。基于鱼油的脂肪乳剂在逆转这些患者的胆汁淤积并预防其发展方面显示出了希望。临床试验表明,生长因子和其他营养激素促进了适应过程。最显著的影响是使用胰高血糖素样肽-2 及其类似物(teduglutide)。手术干预仍然是 SBS 管理的重要组成部分,以促进适应和治疗并发症。当适应过程不成功时,肠移植是最后的选择。本文旨在概述儿科 SBS 的传统和新兴治疗方法。