Biliary atresia. Where are we now and what is next?

被引:0
作者
Petersen, C. [1 ]
Madadi-Sanjani, O. [1 ]
机构
[1] Hannover Med Sch, Klin Kinderchirurg, Carl Neuberg Str 1, D-30625 Hannover, Germany
关键词
Rare diseases; Kasai procedure; Liver transplantation; Transition; Interdisciplinary communication; KASAI PORTOENTEROSTOMY; LIVER-TRANSPLANTATION; INFANTS; PATHOGENESIS; CORTICOSTEROIDS; BILIATRESONE; OUTCOMES; STOOL;
D O I
10.1007/s00112-019-00768-z
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Interdisciplinary networking and centralization particularly for rare diseases as well as transition are characteristics of modern pediatric medicine. This development can be presented using the example of biliary atresia (BA) because the early diagnosis and treatment are prerequisites for a favorable outcome, which in this case is long-term survival with a native liver; however, BA is still the most common indication for liver transplantation during childhood. Even after successful liver transplantation, every patient with BA remains a so-called liver patient and with advancing age young patients face transition into adult care. To be able to understand the clinical dimension of BA, the treating pediatrician needs to realize that even now little is known about BA. In other words: the symptoms of an essentially unknown disease must be treated. For this reason, during the last decadesmany working groups were formed worldwide, focusing on clinical and translational basic research. Therefore, this article presents a summary of the current situation and perspectives for treatment of BA in the coming years.
引用
收藏
页码:1010 / 1017
页数:8
相关论文
共 43 条
[21]   Long-term outlook in biliary atresia [J].
Kelay, Arun ;
Davenport, Mark .
SEMINARS IN PEDIATRIC SURGERY, 2017, 26 (05) :295-300
[22]   Update on investigations pertaining to the pathogenesis of biliary atresia [J].
Kilgore, Alexandra ;
Mack, Cara L. .
PEDIATRIC SURGERY INTERNATIONAL, 2017, 33 (12) :1233-1241
[23]  
KLEMANN C, 2016, BAILLIERE CLIN GAST, V150, P229, DOI DOI 10.1053/J.GASTRO.2015.09.008
[24]   Biliary atresia: A comprehensive review [J].
Lakshminarayanan, Bhanumathi ;
Davenport, Mark .
JOURNAL OF AUTOIMMUNITY, 2016, 73 :1-9
[25]   The pathogenesis of biliary atresia: Evidence for a virus-induced autoimmune disease [J].
Mack, Cara L. .
SEMINARS IN LIVER DISEASE, 2007, 27 (03) :233-242
[26]  
Mack Cara L, 2019, J Pediatr Gastroenterol Nutr, V68, P495, DOI 10.1097/MPG.0000000000002256
[27]   The Role of Endoscopic Retrograde Cholangiopancreatography in the Diagnosis of Biliary Atresia: 14 Years' Experience [J].
Negm, Ahmed A. ;
Petersen, Claus ;
Markowski, Andrea ;
Luettig, Birgit ;
Ringe, Kristina I. ;
Lankisch, Tim O. ;
Manns, Michael P. ;
Ure, Benno ;
Schneider, Andrea S. .
EUROPEAN JOURNAL OF PEDIATRIC SURGERY, 2018, 28 (03) :261-267
[28]   Effects of age at Kasai portoenterostomy on the surgical outcome: a review of the literature [J].
Nio, Masaki ;
Wada, Motoshi ;
Sasaki, Hideyuki ;
Tanaka, Hiromu .
SURGERY TODAY, 2015, 45 (07) :813-818
[29]   SEQUENTIAL TREATMENT OF BILIARY ATRESIA WITH KASAI PORTOENTEROSTOMY AND LIVER-TRANSPLANTATION - A REVIEW [J].
OTTE, JB ;
DEGOYET, JD ;
REDING, R ;
HAUSLEITHNER, V ;
SOKAL, E ;
CHARDOT, C ;
DEBANDE, B .
HEPATOLOGY, 1994, 20 (01) :S41-S48
[30]   Progress in developing animal models for biliary atresia [J].
Petersen, C ;
Kuske, M ;
Bruns, E ;
Biermanns, D ;
von Wussow, P ;
Mildenberger, H .
EUROPEAN JOURNAL OF PEDIATRIC SURGERY, 1998, 8 (03) :137-141