Cooper A, Ross A J, O'Neill J A, Bishop H C, Templeton J M, Ziegler M M
J Pediatr Surg. 1985 Dec;20(6):772-4. doi: 10.1016/s0022-3468(85)80042-7.
Although the protracted use of total parenteral nutrition (TPN) in infants is known to predispose to cholestasis, which in certain instances may not be reversible, failure to understand the pathogenesis of this condition has hindered the development of an effective medical treatment. That surgical treatment may reverse this process in selected patients is the subject of the present report. Two premature infants and one term infant, with short bowel syndrome acquired in infancy, developed conjugated hyperbilirubinemia following institution of balanced TPN. Infectious and metabolic etiologies for the jaundice were ruled out. No excretion of 99mTc-labeled DISIDA from the biliary tree was demonstrable 24 hours following injection by nuclear scintigraphy, despite the absence of extrahepatic biliary obstruction by abdominal sonography. When all conventional medical methods of managing the problem failed, exploration was performed. Intraoperative cholangiography showed normal intrahepatic and extrahepatic biliary ducts in each instance. Irrigation of the biliary tree with normal saline solution was then performed. In all cases, liver biopsies were obtained that were consistent with TPN-associated cholestasis and in all cases, jaundice resolved promptly following operation. We conclude that intractable cholestasis associated with TPN, regardless of its etiology, is a functional abnormality of biliary excretion that may respond in selected patients to irrigation of the biliary tree with radiographic dye and/or normal saline solution.
尽管已知婴儿长期使用全胃肠外营养（TPN）易引发胆汁淤积，在某些情况下这种胆汁淤积可能无法逆转，但由于对该病症发病机制的认识不足，阻碍了有效药物治疗方法的开发。本报告的主题是手术治疗可能会使部分患者的这一过程得到逆转。两名早产儿和一名足月儿在婴儿期患短肠综合征，在接受均衡TPN治疗后出现结合胆红素血症。排除了黄疸的感染性和代谢性病因。经核素闪烁扫描，注射99mTc标记的二异丙基乙酰苯胺亚氨基二乙酸（DISIDA）24小时后，未显示有来自胆管树的排泄，尽管腹部超声检查未发现肝外胆管梗阻。当所有常规的处理该问题的医疗方法均告失败后，进行了探查。术中胆管造影显示每个病例的肝内和肝外胆管均正常。然后用生理盐水冲洗胆管树。所有病例均获取了与TPN相关胆汁淤积相符的肝活检组织，并且所有病例术后黄疸均迅速消退。我们得出结论，与TPN相关的顽固性胆汁淤积，无论其病因如何，都是一种胆汁排泄的功能异常，在部分患者中可能对用造影剂和/或生理盐水冲洗胆管树有反应。
