de Agustin J C, Vázquez J J, Rodríguez-Arnao D, Canals M J, Soler J, Alvarez E
Department of Pediatric Surgery, Hospital General Universitario Gregorio Marañón, Universidad Complutense de Madrid, Spain.
Eur J Pediatr Surg. 1999 Aug;9(4):236-41. doi: 10.1055/s-2008-1072252.
Innovative surgical and pharmacological therapeutic measures in short-bowel syndrome (SBS) are constantly changing the prognosis of this devastating condition. The aim of this paper is to present our most recent experience in the treatment of this disease, with particular emphasis on the impact of home parenteral nutrition (HPN) and the use of growth hormone (GH).
A group of 8 patients with severe SBS have been studied for the past 4 years. Intestinal length of less than 25% normal at the time of bowel resection was the criterion for inclusion in this study.
Mean age at the time of diagnosis was 2 years (ranging from 1 day to 9 years). The etiology of the SBS was Hirschsprung's disease (n = 3), midgut volvulus (n = 2), gastroschisis (n = 1), omphalocele with ileal atresia and necrotizing enterocolitis (n = 1) and Crohn's disease (n = 1). Length of the residual bowel was 8 and 50 cm with ileocecal valve (ICV) preservation and 23, 27, 30, 50, 70, 100 cm without ICV. Sixty percent of the patients survived. Two patients died due to fulminant gram-negative sepsis and one due to cardiac malformation. Two patients are still on parenteral nutrition (PN) providing 30 and 60% of total calories. Human GH (0.3 U/kg/day) was used in two patients over a period of 28 days. In these patients, an increased tolerance to enteral feeding was observed. HPN was provided in 5 cases, allowing regular school attendance in 3 patients. In 3 cases, discontinuation of the PN was achieved at 24, 25 and 35 months respectively.
Human GH can improve tolerance of enteral feeding. HPN has a beneficial effect on child behaviour. Intestinal transplantation must be considered when no other surgical or medical measures are available.
短肠综合征（SBS）的创新性手术和药物治疗措施不断改变着这种毁灭性疾病的预后。本文旨在介绍我们在治疗该疾病方面的最新经验，尤其强调家庭肠外营养（HPN）的影响以及生长激素（GH）的使用。
在过去4年里对一组8例严重SBS患者进行了研究。肠切除时肠道长度小于正常的25%是纳入本研究的标准。
诊断时的平均年龄为2岁（范围从1天至9岁）。SBS的病因包括先天性巨结肠（n = 3）、中肠扭转（n = 2）、腹裂（n = 1）、脐膨出合并回肠闭锁和坏死性小肠结肠炎（n = 1）以及克罗恩病（n = 1）。保留回盲瓣（ICV）时残留肠管长度为8至50厘米，未保留ICV时为23、27、30、50、70、100厘米。60%的患者存活。2例患者死于暴发性革兰氏阴性菌败血症，1例死于心脏畸形。2例患者仍在接受肠外营养（PN），分别提供总热量的30%和60%。2例患者在2个月的时间内使用了人生长激素（0.3 U/kg/天）。在这些患者中，观察到对肠内喂养的耐受性增加。5例患者接受了HPN，3例患者能够正常上学。3例患者分别在24、25和35个月时停止了PN。
人生长激素可提高对肠内喂养的耐受性。HPN对儿童行为有有益影响。当没有其他手术或药物措施可用时，必须考虑肠道移植。
