BACKGROUND: We reported a modification of the hepatic portoenterostomy (HPE) for biliary atresia with favorable results. HPE is associated with a risk of hepatic impairment, so we adopted a novel steroid therapy regimen well suited to our procedure. This paper reports the results of our experience. PATIENTS AND METHODS: Between 1991 and 2009, 53 patients (18 boys, 35 girls) underwent modified HPE with novel steroid therapy, which consisted of administering hydrocortisone immediately after surgery, followed by intravenous administration of prednisolone. The number of patients who became normal total bilirubin (TB) levels, frequency of early onset cholangitis and other postoperative complications, and outcomes were retrospectively studied. RESULTS: The TB levels in 43 of the 53 patients became normal. Cholangitis was observed in seven, but all of them recovered. Other postoperative complications were noted in eight, but with no fatal cases. Of the 11 patients who underwent living-donor liver transplantation, 3 died after the transplant. Of the 53 patients, 39 are alive without liver transplantation and 34 have normal TB (range of observation period: 18 years and 9-2 months). CONCLUSIONS: The novel steroid regimen may have contributed to the outcome and appears to be well suited to the modified HPE.
BACKGROUND: We reported a modification of the hepatic portoenterostomy (HPE) for biliary atresia with favorable results. HPE is associated with a risk of hepatic impairment, so we adopted a novel steroid therapy regimen well suited to our procedure. This paper reports the results of our experience. PATIENTS AND METHODS: Between 1991 and 2009, 53 patients (18 boys, 35 girls) underwent modified HPE with novel steroid therapy, which consisted of administering hydrocortisone immediately after surgery, followed by intravenous administration of prednisolone. The number of patients who became normal total bilirubin (TB) levels, frequency of early onset cholangitis and other postoperative complications, and outcomes were retrospectively studied. RESULTS: The TB levels in 43 of the 53 patients became normal. Cholangitis was observed in seven, but all of them recovered. Other postoperative complications were noted in eight, but with no fatal cases. Of the 11 patients who underwent living-donor liver transplantation, 3 died after the transplant. Of the 53 patients, 39 are alive without liver transplantation and 34 have normal TB (range of observation period: 18 years and 9-2 months). CONCLUSIONS: The novel steroid regimen may have contributed to the outcome and appears to be well suited to the modified HPE.
Authors: Mark Davenport; Mark D Stringer; Sarah A Tizzard; Patricia McClean; Giorgina Mieli-Vergani; Nedim Hadzic Journal: Hepatology Date: 2007-12 Impact factor: 17.425
Authors: T Hashimoto; Y Otobe; Y Shimizu; T Suzuki; T Nakamura; S Hayashi; Y Matsuo; M Sato; T Manabe Journal: J Am Coll Surg Date: 1997-12 Impact factor: 6.113
Authors: Ho Yu Chung; Kenneth Kak Yuen Wong; Lawrence Cheun Leung Lan; Paul Kwong Hang Tam Journal: Pediatr Surg Int Date: 2008-08-05 Impact factor: 1.827
Authors: Rebecka L Meyers; Linda S Book; Molly A O'Gorman; W Daniel Jackson; Richard E Black; Dale G Johnson; Michael E Matlak Journal: J Pediatr Surg Date: 2003-03 Impact factor: 2.545
Authors: Yu-Wen Chung-Davidson; Peter J Davidson; Anne M Scott; Erin J Walaszczyk; Cory O Brant; Tyler Buchinger; Nicholas S Johnson; Weiming Li Journal: J Vis Exp Date: 2014-06-06 Impact factor: 1.355