Reinshagen K, Adam R, Trunk M, Wessel L M
Department of Pediatric Surgery, Mannheim, Germany.
Minerva Pediatr. 2009 Jun;61(3):273-81.
Short bowel syndrome is a life threatening disease with a high mortality and morbidity. Since home parenteral nutrition (PN) has been established, there is an increasing number of patients surviving the acute loss of bowel function. But on the long-time these patients suffer from different complications of PN, with loss of central venous access, recurrent sepsis and finally the syndrome of progressive cholestatic liver disease. Both loss of central venous access and especially the progressive cholestatic liver disease are the limiting factor for the long-term survival of patients suffering from intestinal failure. Interestingly, the pathophysiologic mechanisms of PN induced intrahepatic cholestasis have not been dissolved yet and seem to be of multifactorial genesis. Cholestasis has shown to be associated with prematurity, recurrent sepsis, enteral and PN, especially with lipid emulsions. Enteral feeding and a well-controlled regime of PN lower the incidence of end-stage liver disease and, therefore, has to be optimized in the therapy of these patients.
短肠综合征是一种危及生命的疾病，死亡率和发病率都很高。自从建立了家庭肠外营养（PN）以来，越来越多的患者在肠道功能急性丧失后存活下来。但长期来看，这些患者会遭受PN的不同并发症，如中心静脉通路丧失、反复发生败血症，最终出现进行性胆汁淤积性肝病综合征。中心静脉通路丧失，尤其是进行性胆汁淤积性肝病，是肠衰竭患者长期生存的限制因素。有趣的是，PN诱导的肝内胆汁淤积的病理生理机制尚未明确，似乎是多因素导致的。胆汁淤积已被证明与早产、反复败血症、肠内营养和PN有关，尤其是与脂肪乳剂有关。肠内喂养和精心控制的PN方案可降低终末期肝病的发生率，因此，在这些患者的治疗中必须进行优化。
