Nicholas L Webb1, Ashish Jiwane2,3, Chee Y Ooi2,4, Scott Nightinghale5,6, Susan E Adams2,3, Usha Krishnan2,4. 1. Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia. 2. School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia. 3. Department of Pediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia. 4. Department of Gastroenterology, Sydney Children's Hospital, Sydney, New South Wales, Australia. 5. Department of Gastroenterology, John Hunter Children's Hospital, Newcastle, New South Wales, Australia. 6. Discipline of Paediatrics and Child Health, School of Medicine and Public Health, University of Newcastle, Newcastle, New South Wales, Australia.
Abstract
AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014. Age at KP, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of PHT and ascending cholangitis post-KP were recorded and related to the primary post-KP outcome measures. RESULTS: BA was diagnosed in 29 patients. Twenty-four of 29 patients underwent KP. Median age at KP was 68 days (29-104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of KP. Median follow-up was 8.4 years (2.08-15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5-year NLS was 45.8% (11/24). Fourteen of 24 (58.3%) had PHT and 18 of 24 (75%) patients had ascending cholangitis post-KP. Absence of bridging fibrosis in liver histology at KP was the only factor to be significantly associated with improved 5-year NLS. None of the other variables examined had a significant association with either 5-year NLS or bilirubin normalisation by 6 months. CONCLUSION: Five-year NLS in this series was 45.8%. Absence of bridging fibrosis at time of KP was the only factor significantly associated with improved 5-year NLS.
AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014. Age at KP, liver biopsy results, use of ursodeoxycholic acid or prophylactic antibiotics and occurrence of PHT and ascending cholangitis post-KP were recorded and related to the primary post-KP outcome measures. RESULTS: BA was diagnosed in 29 patients. Twenty-four of 29 patients underwent KP. Median age at KP was 68 days (29-104). Fourteen of 24 (58.3%) had bridging fibrosis and 5 of 24 (20.8%) had cirrhosis at time of KP. Median follow-up was 8.4 years (2.08-15.58 years). Bilirubin normalisation within 6 months occurred in 7 of 24 (29.2%) patients and 5-year NLS was 45.8% (11/24). Fourteen of 24 (58.3%) had PHT and 18 of 24 (75%) patients had ascending cholangitis post-KP. Absence of bridging fibrosis in liver histology at KP was the only factor to be significantly associated with improved 5-year NLS. None of the other variables examined had a significant association with either 5-year NLS or bilirubin normalisation by 6 months. CONCLUSION: Five-year NLS in this series was 45.8%. Absence of bridging fibrosis at time of KP was the only factor significantly associated with improved 5-year NLS.
Authors: Ana M Calinescu; Omid Madadi-Sanjani; Cara Mack; Richard A Schreiber; Riccardo Superina; Deirdre Kelly; Claus Petersen; Barbara E Wildhaber Journal: J Clin Med Date: 2022-01-19 Impact factor: 4.241