Prognosis of Children Undergoing Liver Transplantation: A 30-Year European Study

被引:35
作者
Baumann, Ulrich [1 ,2 ,3 ,4 ,5 ,6 ,7 ]
Karam, Vincent [5 ,6 ]
Adam, Rene [5 ,6 ]
Fondevila, Constantino [5 ,8 ]
Dhawan, Anil [9 ]
Sokal, Etienne [10 ]
Jacquemin, Emmanuel [11 ,12 ]
Kelly, Deirdre A. [3 ,4 ]
Grabhorn, Enke [7 ,13 ]
Pawlowska, Joanna [7 ,14 ]
D'Antiga, Lorenzo [7 ,15 ]
Vega, Paloma Jara [7 ,16 ]
Debray, Dominique [7 ,17 ,18 ]
Polak, Wojciech G. [6 ,19 ]
de Goyet, Jean de Ville [20 ]
Verkade, Henkjan J. [6 ,21 ]
机构
[1] Hannover Med Sch, Dept Paediat Kidney Liver & Metab Dis, Div Paediat Gastroenterol, Carl Neuberg Str 1, D-30625 Hannover, Germany
[2] Hannover Med Sch, Dept Paediat Kidney Liver & Metab Dis, Div Hepatol, Hannover, Germany
[3] Univ Birmingham, Inst Immunol & Immunotherapy, Birmingham, W Midlands, England
[4] Birmingham Women & Childrens Hosp, Liver Unit, Birmingham, W Midlands, England
[5] Univ Paris Saclay, Hop Paul Brousse, AP HP, European Liver Transplant Registry,Res Unit Chron, Villejuif, France
[6] European Liver & Intestine Transplant Assoc, Padua, Italy
[7] La Paz Univ Hosp, European Reference Network TransplantChild, Madrid, Spain
[8] Hosp Univ La Paz, CIBERehd, IDIPAZ, Dept Gen & Digest Surg, Madrid, Spain
[9] Kings Coll Hosp London, London, England
[10] Catholic Univ Louvain, Clin Univ St Luc, Brussels, Belgium
[11] Univ Paris Saclay, Bicetre Hosp, AP HP,FILFOIE,ERN RARE LIVER, Pediat Hepatol & Liver Transplantat Unit,Natl Ref, Le Kremlin Bicetre, France
[12] Univ Paris Saclay, Inserm U1193, Hepatinov, Orsay, France
[13] Univ Med Ctr Hamburg Eppendorf, Childrens Hosp, Hamburg, Germany
[14] Childrens Mem Hlth Inst, Dept Gastroenterol Hepatol Nutr Disorders & Pedia, Warsaw, Poland
[15] Hosp Papa Giovanni XXIII, Paediat Hepatol Gastroenterol & Transplantat, Bergamo, Italy
[16] Hosp Infantil Univ La Paz, Paediat Hepatol Serv, Coordinator ERN TransplantChild, Madrid, Spain
[17] Univ Paris, Hop Necker, AP HP, Pediat Liver Unit, Paris, France
[18] Univ Paris, Hop Necker, AP HP, Reference Ctr Biliary Atresia & Genet Cholestasis, Paris, France
[19] Erasmus MC, Univ Med Ctr Rotterdam, Dept Surg, Div Hepatopancreatobiliary & Transplant Surg,Tran, Rotterdam, Netherlands
[20] ISMETT, Dept Treatment & Study Pediat Abdominal Dis & Abd, Palermo, Italy
[21] Univ Groningen, Univ Med Ctr Groningen, ERN RareLiver, Dept Pediat,Beatrix Childrens Hosp, Groningen, Netherlands
关键词
SURVIVAL; OUTCOMES; REGISTRY;
D O I
10.1542/peds.2022-057424
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
OBJECTIVES: The European Liver Transplant Registry has been collecting data on virtually all pediatric liver transplant (PLT) procedures in Europe since 1968. We analyzed patient outcome over time and identified parameters associated with long-term patient outcome. METHODS: Participating centers and European organ-sharing organizations provided retrospective data to the European Liver Transplant Registry. To identify trends, data were grouped into consecutive time spans: era A: before 2000, era B: 2000 to 2009, and the current era, era C: since 2010. RESULTS: From June 1968 until December 2017, 16 641 PLT were performed on 14 515 children by 133 centers. The children <7 years of age represented 58% in era A, and 66% in the current era (P <.01). The main indications for PLT were congenital biliary diseases (44%) and metabolic diseases (18%). Patient survival at 5 years is currently 86% overall and 97% in children who survive the first year after PLT. The survival rate has improved from 74% in era A to 83% in era B and 85% in era C (P <.0001). Low-volume centers (<5 PLT/year) represented 75% of centers but performed only 19% of PLT and were associated with a decreased survival rate. In the current era, however, survival rates has become irrespective of volume. Infection is the leading cause of death (4.1%), followed by primary nonfunction of the graft (1.4%). CONCLUSIONS: PLT has become a highly successful medical treatment that should be considered for all children with end-stage liver disease. The main challenge for further improving the prognosis remains the early postoperative period.
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页数:11
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