van der Woerd Wendy L, Kokke Freddy T, van der Zee David C, Houwen Roderick H
Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
J Pediatr Surg. 2015 Nov;50(11):1846-9. doi: 10.1016/j.jpedsurg.2015.07.016. Epub 2015 Jul 27.
Progressive familial intrahepatic cholestasis (PFIC) with low gamma-glutamyl transpeptidase (GGT) and Alagille syndrome are associated with persistent cholestasis and severe pruritus. Various types of biliary diversion have been used to reduce this pruritus and prevent liver dysfunction. We report our experience concerning the efficacy and safety of total biliary diversion (TBD) as an additional treatment option.
TBD was performed in four PFIC patients and one patient with Alagille syndrome, and was accomplished by anastomosing a jejunal segment to the choledochal duct terminating as an end stoma, or by disconnecting the choledochal duct after previous cholecystojejunocutaneostomy.
TBD resulted in a marked improvement of symptoms and biochemical parameters in all PFIC patients. Despite relief of pruritus, cholestasis persisted in the Alagille patient. During 5-15years of follow-up, no clinical signs of fat malabsorption such as diarrhea or weight loss were encountered. However, to maintain adequate levels of fat-soluble vitamins, especially of vitamin K, substantial supplementation was necessary.
Total biliary diversion can be a useful surgical treatment option for patients with low-GGT PFIC and possibly also Alagille syndrome, when partial biliary diversion is insufficient. It can be performed without inducing clinical signs of fat malabsorption although individualized supplementation of fat-soluble vitamins with careful monitoring is warranted.
低γ-谷氨酰转肽酶（GGT）的进行性家族性肝内胆汁淤积症（PFIC）和阿拉吉耶综合征与持续性胆汁淤积和严重瘙痒有关。已采用各种类型的胆汁转流术来减轻这种瘙痒并预防肝功能障碍。我们报告了我们关于全胆汁转流术（TBD）作为一种额外治疗选择的疗效和安全性的经验。
对4例PFIC患者和1例阿拉吉耶综合征患者实施了TBD，通过将一段空肠与胆总管吻合，以端侧造口结束，或在先前的胆囊空肠造口术后切断胆总管来完成。
TBD使所有PFIC患者的症状和生化指标有显著改善。尽管瘙痒缓解，但阿拉吉耶综合征患者的胆汁淤积仍持续存在。在5至15年的随访期间，未出现腹泻或体重减轻等脂肪吸收不良的临床体征。然而，为维持脂溶性维生素尤其是维生素K的适当水平，大量补充是必要的。
当部分胆汁转流术不足时，全胆汁转流术对于低GGT PFIC患者以及可能的阿拉吉耶综合征患者可能是一种有用的手术治疗选择。尽管需要仔细监测并个体化补充脂溶性维生素，但该手术可在不引发脂肪吸收不良临床体征情况下进行。
