Omid Madadi-Sanjani1, David Fortmann1, Udo Rolle2, Burkhard Rodeck3, Ekkehard Sturm4, Eva-Doreen Pfister5, Joachim F Kuebler1, Ulrich Baumann5,6, Peter Schmittenbecher7, Claus Petersen1. 1. Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany. 2. Department of Pediatric Surgery and Pediatric Urology, Goethe University Frankfurt /M., Germany. 3. Department of Pediatric Gastroenterology, Christliches Kinderhospital Osnabrueck, Osnabrueck, Germany. 4. Department of Paediatric Gastroenterology and Hepatology, University Hospital for Children and Adolescents, University of Tuebingen, Baden-Württemberg, Germany. 5. Division of Pediatric Gastroenterology and Hepatology, Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany. 6. Liver Unit, Birmingham Women's and Children's Hospital, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham. 7. Department of Pediatric Surgery, Municipal Hospital, Karlsruhe, Baden-Wuerttemberg, Germany.
Abstract
INTRODUCTION: The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. MATERIALS AND METHODS: In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. RESULT: Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001-2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001-2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001-2005 [p = 0.5]). Compared with the 2001-2005 analysis, all criteria showed improvement but the differences are statistically not significant. CONCLUSION: Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction. Thieme. All rights reserved.
INTRODUCTION: The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. MATERIALS AND METHODS: In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. RESULT: Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001-2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001-2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001-2005 [p = 0.5]). Compared with the 2001-2005 analysis, all criteria showed improvement but the differences are statistically not significant. CONCLUSION: Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction. Thieme. All rights reserved.
Authors: Jan B F Hulscher; Joachim F Kuebler; Janneke M Bruggink; Mark Davenport; Stefan Scholz; Claus Petersen; Omid Madadi-Sanjani; Nagoud Schukfeh Journal: J Clin Med Date: 2022-02-21 Impact factor: 4.241