Long term results of liver transplantation for alpha-1 antitrypsin deficiency

被引：13

作者：

Guillaud, Olivier ^{[1
,2
]}

Jacquemin, Emmanuel ^{[3
,4
]}

Couchonnal, Eduardo ^{[5
]}

Vanlemmens, Claire ^{[6
]}

Francoz, Claire ^{[7
]}

Chouik, Yasmina ^{[1
]}

Conti, Filomena ^{[8
]}

Duvoux, Christophe ^{[9
]}

Hilleret, Marie-Noelle ^{[10
]}

Kamar, Nassim ^{[11
]}

Houssel-Debry, Pauline ^{[12
]}

Neau-Cransac, Martine ^{[13
]}

Pageaux, Georges-Philippe ^{[14
]}

Gonzales, Emmanuel ^{[3
,4
]}

Ackermann, Oanez ^{[3
,4
]}

Gugenheim, Jean ^{[15
]}

Lachaux, Alain ^{[5
,16
]}

Ruiz, Mathias ^{[5
]}

Radenne, Sylvie ^{[17
]}

Debray, Dominique ^{[18
]}

Lacaille, Florence ^{[18
]}

McLin, Valerie ^{[19
]}

Duclos-Vallee, Jean-Charles ^{[4
,20
]}

Samuel, Didier ^{[4
,20
]}

Coilly, Audrey ^{[4
,20
]}

Dumortier, Jerome ^{[1
,16
]}

机构：

[1] Hosp Civils Lyon, Hop Edouard Herriot, Federat Specialites Digest, Lyon, France

[2] Ramsay Gen Sante, Clin Sauvegarde, Lyon, France

[3] Univ Paris Saclay, Hop Bicetre, AP HP, Hepatol & Transplantat Hepat Pediat,Ctr Natl Refe, Le Kremlin Bicetre, France

[4] Univ Paris Saclay, Hepatinov, Inserm, U1193, Orsay, France

[5] Hosp Civils Lyon, Serv Hepatogastroenterol & Nutr Pediat, Hop Femme Mere Enfant, Bron, France

[6] CHU Besancon, Hop Jean Minjoz, Serv Hepatol, Besancon, France

[7] Hop Beaujon, AP HP, Serv Hepatol, Clichy, France

[8] Hop La Pitie Salpetriere, AP HP, Serv Hepatogastroenterol, Paris, France

[9] Hop Henri Mondor, AP HP, Serv Hepatol, Creteil, France

[10] CHU Grenoble, Hop Michalon, Serv Hepatogastroenterol, La Tronche, France

[11] CHU Toulouse, Hop Rangueil, Serv Nephrol Hypertens Arterielle Dialyse Transpl, Toulouse, France

[12] CHU Rennes, Hop Pontchaillou, Serv Hepatol, Rennes, France

[13] CHU Bordeaux, Hop Haut Leveque, Serv Chirurg Hepatobiliaire & Transplantat Hepat, Bordeaux, France

[14] CHU Montpellier, Federat Medicochirurg Malad Appareil Digestif, Hop St Eloi, Montpellier, France

[15] CHU Nice, Serv Chirurg Digest, Hop Archet 2, Nice, France

[16] Univ Lyon, Lyon, France

[17] Hosp Civils Lyon, Serv Hepatol, Hop Croix Rousse, Lyon, France

[18] Hop Necker Enfants Malad, AP HP, Unite Hepatol Pediat, Ctr Reference Atresie Voies Biliaires & Cholestas, Paris, France

[19] Hop Univ Geneve, Ctr Suisse Foie Enfant, Dept Pediat Gynecol & Obstet, Geneva, Switzerland

[20] Hop Paul Brousse, AP HP, Ctr Hepatobiliaire, Villejuif, France

来源：

DIGESTIVE AND LIVER DISEASE | 2021年 / 53卷 / 05期

关键词：

Alpha1; antitrypsin; Liver transplantation; Outcome; Survival; ALPHA1-ANTITRYPSIN DEFICIENCY; NATURAL-HISTORY; COMBINED LUNG; DISEASE; ADULTS; OUTCOMES; CHILDREN; INDIVIDUALS; EXPERIENCE;

D O I：

10.1016/j.dld.2020.10.016

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Introduction: Liver transplantation (LT) is the therapeutic option for end-stage liver disease associated with alpha1 antitrypsin (A1AT) deficiency. The aim of the present retrospective study was to report on long-term outcomes following LT for A1AT deficiency. Methods: The medical records of 90 pediatric and adult patients transplanted between 1982 and 2017 in France and Geneva (Switzerland) were reviewed. Results: The study population consisted of 32 adults and 58 children; median age at transplant was 13.0 years (range: 0.2?65.1), and 65 were male (72.2%). Eighty-two patients (94.8% of children and 84.4% of adults) had the PI *ZZ genotype/phenotype and eight patients (8.9%) had the Pi *SZ genotype/phenotype. Eighty-four patients (93.3%) were transplanted for end-stage liver disease and six (all Pi *ZZ adults) for HCC. Median follow-up after LT was 13.6 years (0.1?31.7). The overall cumulative patient survival rates post-transplant were 97.8% at 1 year, and 95.5%, 95.5%, 92.0%, 89.1% at 5, 10, 15, 20 years respectively. The overall cumulative graft survival rates were 92.2% at 1 year, and 89.9%, 89.9%, 84.4%, 81.5% at 5, 10, 15 and 20 years, respectively. Conclusions: In a representative cohort of patients having presented with end-stage-liver disease or HCC secondary to A1AT, liver transplantation offered very good patient and graft survival rates. (c) 2020 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

引用

页码：606 / 611

页数：6

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