Wada Motoshi, Nishi Kotaro, Nakamura Megumi, Kudo Hironori, Yamaki Satoshi, Sasaki Hideyuki, Sato Tomoyuki, Fukuzawa Taichi, Tanaka Hiromu, Kazama Takuro, Amae Shintaro, Nio Masaki
Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan,
Pediatr Surg Int. 2013 Nov;29(11):1115-8. doi: 10.1007/s00383-013-3375-8.
We elucidated the life-threatening risk factors for intestinal failure (IF) and characterized the role of intestinal transplantation (ITx) in affected patients.
We conducted a retrospective review of 38 patients with short bowel (SB) and 19 with motility disorders (MD). The SB patients were divided into three categories according to the length of their residual small bowel and the presence of the ileocecal valve. The four disease subcategories were grouped into two categories: low-risk category (mild and moderated SB) and high-risk category (extensive SB and MD). The age at the introduction of parenteral nutrition (PN) was <1 year in 50 patients (infant group, IG) and 1-15 years in 7 patients (pediatric group, PG).
Enteral autonomy was rarely achieved in the high-risk category (p < 0.0001). IG was associated with a higher incidence of developing intestinal failure-associated liver disease (IFALD) (p = 0.004). Eight patients died, due to IFALD in four, sepsis in three and acute heart failure in one. Twenty-eight patients (49 %) are currently alive without PN, including four after ITx.
The treatment of high-risk IF is still challenging. Inclusion of ITx in appropriate timing, along with aggressive medical, nutritional and surgical management, may reduce advanced morbidity and mortality of high-risk IF.
我们阐明了肠衰竭（IF）的危及生命的风险因素，并描述了肠移植（ITx）在受影响患者中的作用。
我们对38例短肠（SB）患者和19例运动障碍（MD）患者进行了回顾性研究。SB患者根据残余小肠长度和回盲瓣的存在分为三类。四个疾病亚类分为两类：低风险类别（轻度和中度SB）和高风险类别（广泛SB和MD）。50例患者（婴儿组，IG）开始肠外营养（PN）的年龄<1岁，7例患者（儿科组，PG）为1 - 15岁。
高风险类别中很少能实现肠道自主（p < 0.0001）。IG与发生肠衰竭相关肝病（IFALD）的较高发生率相关（p = 0.004）。8例患者死亡，4例死于IFALD，3例死于败血症，1例死于急性心力衰竭。28例患者（49%）目前无需PN存活，其中4例接受了ITx。
高风险IF的治疗仍然具有挑战性。在适当的时候纳入ITx，同时进行积极的医学、营养和手术管理，可能会降低高风险IF的晚期发病率和死亡率。
