Division of General Pediatrics, Department of Emergency Medicine, Children's Hospital of Philadelphia, 3401 Civic Center boulevard, 8W22, Philadelphia, PA, 19104, USA.
Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
BMC Pediatr. 2019 Mar 8;19(1):72. doi: 10.1186/s12887-019-1446-2.
Total parenteral nutrition (TPN) and biliary atresia (BA) are common causes of cholestasis in infancy. The diagnosis of BA is time sensitive due to an inverse correlation between age at intervention (hepatic portoenterostomy - HPE) and survival without liver transplantation. Clinical, laboratory, and histologic features of BA and parenteral nutrition associated cholestasis (PNAC) are similar, creating a diagnostic dilemma for cholestatic infants on parenteral nutrition. There is limited published information about the natural history of PNAC including time to resolution, or diagnostic tests that distinguish BA from other etiologies of cholestasis.
We present a case of a child diagnosed with BA whose cholestasis began while receiving TPN. His clinical course was notable for transient resolution of his cholestasis after stopping parenteral nutrition and ultimate intraoperative diagnosis.
Clinicians who care for patients who frequently receive TPN should be aware that clinical, laboratory, imaging, and biopsy findings can be similar between BA and PNAC.
全胃肠外营养（TPN）和胆道闭锁（BA）是婴儿胆汁淤积的常见原因。由于干预（肝门空肠吻合术-HPE）年龄与肝移植后无生存时间之间存在反比关系，因此 BA 的诊断具有时间紧迫性。BA 和肠外营养相关胆汁淤积（PNAC）的临床、实验室和组织学特征相似，这给接受肠外营养的胆汁淤积婴儿的诊断带来了困境。关于 PNAC 的自然病程，包括缓解时间或可将 BA 与其他胆汁淤积病因区分开来的诊断检测，发表的信息有限。
我们介绍了一例在接受 TPN 时被诊断为 BA 的患儿，其胆汁淤积始于 TPN 期间。他的临床病程值得注意的是，在停止 TPN 后，他的胆汁淤积短暂缓解，最终术中诊断为 BA。
经常接受 TPN 的患者的临床医生应注意到，BA 和 PNAC 之间的临床、实验室、影像学和活检结果可能相似。
