Alrabadi Leina S, Morotti Raffaella A, Valentino Pamela L, Rodriguez-Davalos Manuel I, Ekong Udeme D, Emre Sukru H
Department of Pediatrics, Section of Pediatric Gastroenterology and Hepatology, Yale University School of Medicine, New Haven, CT, USA.
Department of Pathology, Yale University School of Medicine, New Haven, CT, USA.
Pediatr Transplant. 2018 Jun;22(4):e13184. doi: 10.1111/petr.13184. Epub 2018 Apr 14.
Development of macrovesicular steatosis post-LT in patients with PFIC-1 is increasingly being observed, with the etiology not fully understood. We highlight successful and effective EBD for reversal of allograft steatosis in 2 patients with PFIC-1 disease and discuss our experience with internal biliary diversion in this patient population.
越来越多地观察到PFIC-1患者肝移植后出现大泡性脂肪变性,其病因尚未完全明确。我们重点介绍了2例PFIC-1疾病患者通过有效的内镜下胆管引流成功逆转移植肝脂肪变性的情况,并讨论了我们在这一患者群体中进行内胆汁转流术的经验。