Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Box 281, FIN-00029 HUS Helsinki, Finland.
J Pediatr Surg. 2009 Nov;44(11):2139-44. doi: 10.1016/j.jpedsurg.2009.05.002.
We investigated whether mortality, intestinal adaptation, and liver function differ between intestinal failure (IF) patients with either short bowel (SB) or bowel dysmotility (DM).
Twenty-six consecutive patients with SB (n = 20) or DM (n = 6) treated between 2000 and 2007 were retrospectively assessed. Intestinal failure was defined as less than 25% of age-adjusted small intestinal length or dependence on parenteral nutrition (PN) more than 6 months.
Median age-adjusted small intestinal length (17% vs 45%) and gestational age (35 vs 40 weeks) were (P < .05) shorter, whereas proportion of the remaining colon (86% vs 0%) was (P < .05) higher in the SB group relative to the DM group. Overall survival was 92%. Median peak serum bilirubin (80 vs 25 micromol/L) and rate of cholestasis (11/20 vs 0/6) were higher (P < .05) in the SB group. Short bowel rather than DM as an etiology of IF predicted weaning off PN (RR, 39.3; 95% confidence interval [CI], 1.43-526; P < .01) and development of cholestasis (risk ration [RR], 18.3; 95% CI, 0.658-127; P < .05). Three SB children developed liver failure and two died, whereas neither of these occurred in the DM group.
Children with SB are more likely to wean off PN but more prone to cholestatic liver disease than those with DM as an etiology of IF.
本研究旨在探讨短肠(SB)或肠动力障碍(DM)引起的肠衰竭(IF)患者的死亡率、肠道适应和肝功能是否存在差异。
回顾性分析了 2000 年至 2007 年期间收治的 26 例连续 SB(n = 20)或 DM(n = 6)IF 患者。IF 定义为小肠长度不足年龄校正值的 25%或肠外营养(PN)依赖超过 6 个月。
SB 组患者的年龄校正小肠长度(17%对 45%)和胎龄(35 对 40 周)均较短(P <.05),而残留结肠的比例(86%对 0%)则较高(P <.05)。总体生存率为 92%。SB 组患者的血清胆红素峰值(80 对 25 μmol/L)和胆汁淤积发生率(11/20 对 0/6)均较高(P <.05)。SB 而非 DM 是 IF 患者停止 PN 和发生胆汁淤积的预测因素(RR,39.3;95%置信区间 [CI],1.43-526;P <.01)和胆汁淤积(RR,18.3;95% CI,0.658-127;P <.05)。3 例 SB 患儿发生肝功能衰竭,2 例死亡,而 DM 组未发生上述情况。
与 DM 相比,SB 是 IF 的病因,SB 患儿更有可能停用 PN,但更易发生胆汁淤积性肝病。