Défis cliniques prioritaires

En raison de sa forte association avec morbidité et mort prématurée, la fibrose est devenue le principal objet des soins secondaires. En particulier pour les patients atteints de stéatose hépatique non alcoolique (NAFLD) qui représente environ 25 % de toutes les maladies du foie.

  • Dans le cas des tumeurs primitives du foie (PLT), certaines cibles moléculaires pour la médecine de précision ont été identifiées. Les traitements à base d'inhibiteurs de points de contrôle immunitaires ont montré des résultats prometteurs. Pour affiner la sélection des traitements et favoriser la combinaison d'approches thérapeutiques systémiques et loco-régionales (telles que les nouveaux types d'embolisation tumorale, les injections directes dans la tumeur et la radiothérapie innovante), de nouvelles signatures immunitaires pronostiques d'efficacité et de toxicité doivent être définies.
  • Dans le contexte de maladies hépatiques en phase terminale, l'infection bactérienne est l'une des causes les plus importantes de décompensation aiguë et de décès. Il est d'une importance cruciale d'acquérir une meilleure connaissance de la résistance bactérienne chez les patients gravement atteints.
  • Malgré les efforts considérables qui ont été consacrés au développement de médicaments orphelins, de nombreuses maladies génétiques et rares du foie (GARLD) manquent encore de remèdes efficaces et deamndent de nouvelles thérapies. Étant donné que la majorité des GARLD proviennent de troubles génétiques, les progrès récents des thérapies pharmacologiques et géniques sur mesure pourraient offrir plus d'options pour les traiter.
Les objectifs

Processus pathologiques dans le compartiment extracellulaire et l'endothélium des patients atteints de maladies du foie.
Hépatinov entend :


identifier à un stade précoce de la NALFD l'ensemble des protéines du compartiment extracellulaire hépatique sensibles aux modifications oxydatives ;

Les lésions hépatiques chroniques sont caractérisées par le dépôt de matrice extracellulaire en réponse à des lésions hépatiques persistantes. Bien que le stress oxydatif soit reconnu comme le second évènement qui mène de la stéatose à la NASH et à la fibrose, les espèces réactives de l'oxygène dans le compartiment extracellulaire ont été peu décrites et leur importance dans la progression de la maladie est totalement inexplorée. Comme toutes les protéines ne sont pas également sensibles à l'oxydation, Hépatinov identifiera les protéines oxydées à différents niveaux de stress et analysera leur capacité à induire la progression de la maladie.

identifier les effets toxiques de la NAFLD sur les cellules endothéliales in vitro et in vivo ;

La dysfonction endothéliale vasculaire semble être un événement précoce dans la NAFLD et peut être impliquée à la fois dans la progression des lésions hépatiques et dans l'athérosclérose systémique. Cette hypothèse sera étudiée en évaluant l'association entre les profils lipidiques circulants pré et postprandiaux et la dysfonction endothéliale vasculaire dans une population de patients NAFLD.

évaluer la qualité des fibres de collagène dans la fibrose hépatique au cours des différents stades des troubles hépatiques chroniques ;

Les microscopies par génération de seconde harmonique et à rayonnement synchrotron dans l'ultraviolet profond permettent l'évaluation qualitative de la fibrose en identifiant les fibrilles de collagène individuelles et en quantifiant leurs propriétés physiques. Le diagnostic et le pronostic seront établis en évaluant la qualité des fibres de collagène dans une cohorte de patients présentant différents degrés de fibrose (F0-F4) dans le cadre de différentes étiologies (NAFLD, maladie alcoolique du foie et maladie du foie après la guérison du VHC).

Identification de signatures pour l'élaboration d'une évaluation des risques de l'immunothérapie chez les patients atteints de tumeurs primitives du foie (PLT).

Le développement de médicaments pour la PLT a été caractérisé par de nombreux échecs. Une analyse complète des biomarqueurs reste à faire notamment dans le cas du cholangio carcinome (CC). Hépatinov améliorera l'efficacité et la sécurité de l'immunothérapie
  • en identifiant de nouvelles signatures tumorales génomiques/phénotypiques ;
  • en identifiant les signatures spécifiques des sérums auto-immuns et leur évolutions dynamique au cours de l'immunothérapie ;
  • en identifiant les signatures globales de réponse immunitaire sérique contre le microbiote intestinal et leur modification sous immunothérapie (en utilisant des techniques pronostiques basées sur l'imagerie, y compris des combinaisons de stratégies d'imagerie morphologique et fonctionnelle - immunoTEP, élastométrie tumorale et imagerie dynamique vasculaire) et
  • en proposant de nouvelles méthodes de manipulation du microbiome intestinal afin d'étendre la thérapie par points de contrôle immunitaires aux patients présentant une résistance primaire ou secondaire.

Pour l'intégration des données, Hépatinov s'appuiera sur l'analyse de corrélation canonique généralisée régularisée et limitée (RGCCA) qui est un cadre général pour l'analyse conjointe de plusieurs sources.

Approches locorégionales et combinées innovantes basées sur l'immunothérapie pour les tumeurs primitives du foie.

Les progrès technologiques ont amélioré la sécurité et l'efficacité de l'embolisation tumorale et de la radiothérapie dans la prise en charge des tumeurs primitives. La combinaison de ces thérapies pourrait améliorer la survie des patients atteints de CC. Bien que de nombreuses études aient démontré que la présence de cellules tumorales circulantes (CTC) est associée de manière significative au pronostic des tumeurs primitives du foie, de nouvelles approches telles que l'identification de l'antigène de surface des cellules tumorales et des cellules de type macrophage associé au cancer (CAML) sont nécessaires, notamment dans le cadre de thérapies innovantes.

Les axes de recherche sont :
  • l'évaluation de nouveaux agents d’embolisation intravasculaire ;

c'est-à-dire radio-embolisation avec HO-166, embolies résorbables chargées de médicaments, administration intra-tumorale d'inhibiteurs de points de contrôle par administration intra-artérielle, intra-hépatique ou percutanée directe, et injection directe dans la tumeur des virus oncolytiques JX594-Transgene.

  • l'évaluation des effets immunomodulateurs de la chimio et de la radio-embolisation à l'aide de ces nouveaux agents et/ou des immunothérapies combinées ;
  • l’étude de la radiothérapie conformationnelle 3D et de la radiothérapie modulée en intensité (IMRT) chez les patients atteints de CC ;
  • l’étude des facteurs influençant la récidive précoce.

Etude de l'évolution in vivo de la résistance bactérienne chez des patients atteints d'affections hépatiques terminales.

Hépatinov effectuera une analyse complète des adaptations bactériennes aux protocoles antibiotiques utilisés pour traiter les patients cirrhotiques infectés et proposera à terme des thérapies alternatives pour contourner la résistance bactérienne aux antibiotiques.

Développement de thérapies pharmacologiques sur mesure pour la cholestase génétique.

Les cholestases intrahépatiques familiales progressives de type 2 (PFIC2) et de type 3 (PFIC3) sont des types autosomiques récessifs de cholestases hépatocellulaires qui se manifestent généralement au cours de la première année de vie et nécessitent une transplantation hépatique lors de l'enfance. Le besoin de thérapies alternatives est d'une importance cruciale. Les défauts de synthèse des acides biliaires (BASD) sont également des troubles graves de la cholestase infantile et génétique qui bénéficient d'un traitement à l'acide cholique. Or, les données concernant les résultats à l'âge adulte des patients traités par acide cholique ne sont pas satisfaisantes.

Développement d'essais de thérapie génique pour les maladies génétiques du foie.

La validation dans des modèles précliniques est nécessaire avant que la thérapie génique puisse être transposée aux humains. Hépatinov se concentre sur les maladies suivantes :
  • les maladies de stockage du glycogène (GSD) utilisant des vecteurs de virus adéno-associés ;
  • la maladie de Crigler-Najjar ;
  • PFIC2 et PFIC3 utilisant des vecteurs de virus adéno-associés tropiques hépatiques.

Développement d'une thérapie cellulaire pour les maladies du foie.

Le laboratoire d'hépatologie pédiatrique et de thérapie cellulaire de l'UC Louvain a une longue expérience de transplantation d'hépatocytes et de cellules souches pour le traitement des maladies hépatiques innées etacquises. La découverte et les études approfondies des propriétés anti-inflammatoires et anti-fibrotiques d'une cellule progénitrice spécifique dérivée du foie ont conduit à développer leur utilisation clinique dans les maladies fibro-inflammatoires du foie.

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HBV prophylaxis after liver transplantation: close to the full success but at the price of long-term prophylaxis adapted to the risk of HBV recurrence.
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Hepatitis E Virus Infection in Patients with Chronic Inflammatory Bowel Disease Treated with Immunosuppressive Therapy
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HLA-DP diversity is associated with improved response to SARS-Cov-2 vaccine in hematopoietic stem cell transplant recipients.
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Interim results from an ongoing, open-label, single-arm trial of odevixibat in progressive familial intrahepatic cholestasis.
Thompson RJ, Artan R, Baumann U, Calvo PL, Czubkowski P, Dalgic B, D'Antiga L, Di Giorgio A, Durmaz Ö, Gonzalès E, Grammatikopoulos T, Gupte G, Hardikar W, Houwen RHJ, Kamath BM, Karpen SJ, Lacaille F, Lachaux A, Lainka E, Loomes KM, Mack CL, Mattsson JP, McKiernan P, Ni Q, Özen H, Rajwal SR, Roquelaure B, Shteyer E, Sokal E, Sokol RJ, Soufi N, Sturm E, Tessier ME, van der Woerd WL, Verkade HJ, Vittorio JM, Wallefors T, Warholic N, Yu Q, Horn P, Kjems L.
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Liver Histology Before Treatment Withdrawal in Autoimmune Hepatitis: Is It Time to Review the ESPGHAN Recommendations?
Maggiore G, Bernard O, Mosca A, Jacquemin E.
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Molecular Mechanisms of Hepatotoxicity
Messina A, Duclos-Vallée JC.
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Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival
Kounis I, Sebagh M, Evain M, Cailliez V, Roche B, De Martin E, Sobesky R, Guettier C, Allard MA, Golse N, Azoulay D, Vibert E, Duclos Vallee JC, Feray C, Samuel D, Coilly A.
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Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study
Vandriel SM, Li LT, She H, Wang JS, Gilbert MA, Jankowska I, Czubkowski P, Gliwicz-Miedzińska D, Gonzales EM, Jacquemin E, Bouligand J, Spinner NB, Loomes KM, Piccoli DA, D'Antiga L, Nicastro E, Sokal É, Demaret T, Ebel NH, Feinstein JA, Fawaz R, Nastasio S, Lacaille F, Debray D, Arnell H, Fischler B, Siew S, Stormon M, Karpen SJ, Romero R, Kim KM, Baek WY, Hardikar W, Shankar S, Roberts AJ, Evans HM, Jensen MK, Kavan M, Sundaram SS, Chaidez A, Karthikeyan P, Sanchez MC, Cavalieri ML, Verkade HJ, Lee WS, Squires JE, Hajinicolaou C, Lertudomphonwanit C, Fischer RT, Larson-Nath C, Mozer-Glassberg Y, Arikan C, Lin HC, Bernabeu JQ, Alam S, Kelly DA, Carvalho E, Ferreira CT, Indolfi G, Quiros-Tejeira RE, Bulut P, Calvo PL, Önal Z, Valentino PL, Desai DM, Eshun J, Rogalidou M, Dezsőfi A, Wiecek S, Nebbia G, Pinto RB, Wolters VM, Tamara ML, Zizzo AN, Garcia J, Schwarz K, Beretta M, Sandahl TD, Jimenez-Rivera C, Kerkar N, Brecelj J, Mujawar Q, Rock N, Busoms CM, Karnsakul W, Lurz E, Santos-Silva E, Blondet N, Bujanda L, Shah U, Thompson RJ, Hansen BE, Kamath BM; Global ALagille Alliance (GALA) Study Group.
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New epidemiologic trends in cholangiocarcinoma.
Pascale A, Rosmorduc O, Duclos-Vallée JC.
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J Hepatol. 2023 Sep;79(3):853-866. doi: 10.1016/j.jhep.2023.04.033. Epub 2023 May 8. PMID: 37164270; PMCID: PMC10735171.
Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival.
Kounis I, Sebagh M, Evain M, Cailliez V, Roche B, De Martin E, Sobesky R, Guettier C, Allard MA, Golse N, Azoulay D, Vibert E, Duclos Vallee JC, Feray C, Samuel D, Coilly A.
Transplantation. 2023 Feb 1;107(2):410-419. doi: 10.1097/TP.0000000000004303. Epub 2023 Jan 26. PMID: 36117256.
Outcomes of 38 patients with PFIC3: Impact of genotype and of response to ursodeoxycholic acid therapy.
Gonzales E, Gardin A, Almes M, Darmellah-Remil A, Seguin H, Mussini C, Franchi-Abella S, Duché M, Ackermann O, Thébaut A, Habes D, Hermeziu B, Lapalus M, Falguières T, Combal JP, Benichou B, Valero S, Davit-Spraul A, Jacquemin E.
JHEP Rep. 2023 Jul 13;5(10):100844. doi: 10.1016/j.jhepr.2023.100844. PMID: 37701337; PMCID: PMC10494458.
Pathological spectrum of hereditary transthyretin renal amyloidosis and clinicopathologic correlation: a French observational study
Dang J, Ferlicot S, Misrahi M, Mussini C, Kounis I, Rémy P, Samuel D, Planté-Bordeneuve V, Adams D, Funalot B, Snanoudj R, Damy T, Moktefi A, Audard V, Zaidan M.
Nephrol Dial Transplant. 2023 Jan 16:gfad006. doi: 10.1093/ndt/gfad006. Epub ahead of print. PMID: 36646436
Pediatric hepatic hemangiomas: spectrum and prognostic significance of initial ultrasound findings.
Rutten C, Ackermann O, Lambert V, Durand P, Gonzales E, Kreindel TG, Guérin F, Franchi-Abella S.
Pediatr Radiol. 2023 Nov;53(12):2446-2457. doi: 10.1007/s00247-023-05769-5. Epub 2023 Sep 29. PMID: 37773445.
Post Living Donor Liver Transplantation Small-for-size Syndrome: Definitions, Timelines, Biochemical, and Clinical Factors for Diagnosis: Guidelines From the ILTS-iLDLT-LTSI Consensus Conference.
Kow AWC, Liu J, Patel MS, De Martin E, Reddy MS, Soejima Y, Syn N, Watt K, Xia Q, Saraf N, Kamel R, Nasralla D, McKenna G, Srinvasan P, Elsabbagh AM, Pamecha V, Palaniappan K, Mas V, Tokat Y, Asthana S, Cherukuru R, Egawa H, Lerut J, Broering D, Berenguer M, Cattral M, Clavien PA, Chen CL, Shah S, Zhu ZJ, Emond J, Ascher N, Rammohan A, Bhangui P, Rela M, Kim DS, Ikegami T; ILTS-iLDLT-LTSI SFSS Working Group.
Transplantation. 2023 Oct 1;107(10):2226-2237. doi: 10.1097/TP.0000000000004770. Epub 2023 Sep 25. PMID: 37749812.
Predictors of 6-year event-free survival in Alagille syndrome patients treated with maralixibat, an ileal bile acid transporter inhibitor.
Sokol RJ, Gonzales EM, Kamath BM, Baker A, Vig P, Mogul DB, Garner W, Hansen BE, Jacquemin E, Thompson RJ.
Hepatology. 2023 Dec 1;78(6):1698-1710. doi: 10.1097/HEP.0000000000000502. Epub 2023 Jun 7. PMID: 37278241; PMCID: PMC10653287.
The novel SALT-M score predicts 1-year post-transplant mortality in patients with severe acute-on-chronic liver failure.
Hernaez R, Karvellas CJ, Liu Y, Sacleux SC, Khemichian S, Stein LL, Shetty K, Lindenmeyer CC, Boike JR, Simonetto DA, Rahimi RS, Jalal PK, Izzy M, Kriss MS, Im GY, Lin MV, Jou JH, Fortune BE, Cholankeril G, Kuo A, Mahmud N, Kanwal F, Saliba F, Sundaram V, Artzner T, Jalan R; Multi-Organ Dysfunction and Evaluation for Liver Transplantation (MODEL) Consortium.
J Hepatol. 2023 Sep;79(3):717-727. doi: 10.1016/j.jhep.2023.05.028. Epub 2023 Jun 12. PMID: 37315809.
Treatment of donor-specific anti-HLA antibodies-mediated rejection after liver transplantation: A French nationwide retrospective study.
Dumortier J, Conti F, Hiriart JB, Dharancy S, Duvoux C, Besch C, Houssel-Debry P, Latournerie M, Chermak F, Meszaros M, Pageaux GP, Radenne S, Boillot O, Hardwigsen J, Kounis I, Kamar N, Saliba F, Erard D, Del Bello A.
Liver Transpl. 2023 Dec 1;29(12):1313-1322. doi: 10.1097/LVT.0000000000000200. Epub 2023 Jun 28. PMID: 37367954.

A Model for Early Endoscopic Detection of High-Risk Gastroesophageal Varices in Children With Biliary Atresia.
Ackermann O, de Boissieu P, Bernard O, Gonzales E, Jacquemin E, Duché M.
J Pediatr Gastroenterol Nutr. 2022 May 1;74(5):643-650. doi: 10.1097/MPG.0000000000003375. Epub 2021 Dec 28. PMID: 34984987.
ABC Transporters in Human Diseases: Future Directions and Therapeutic Perspectives.
Falguières T.
Int J Mol Sci. 2022 Apr 12;23(8):4250. doi: 10.3390/ijms23084250. PMID: 35457067; PMCID: PMC9028344.
Advanced epithelioid hemangioendothelioma of the liver: could lenvatinib offer a bridge treatment to liver transplantation?
Kounis I, Lewin M, Laurent-Bellue A , Poli E, Coilly A, Duclos-Vallée JC, Guettier C , Adam R, Lerut J, Samuel D, Rosmorduc O.
Ther Adv Med Oncol. 2022 Mar 23;14:17588359221086909. doi: 10.1177/17588359221086909. PMID: 35340695; PMCID: PMC8949775.
Alcohol-related hepatocellular carcinoma is a heterogenous condition: Lessons from a latent class analysis.
Costentin CE, Minoves M, Kotzki S, Farges O, Goutté N , Decaens T, Bailly S.
Liver Int. 2022 Jul;42(7):1638-1647. doi: 10.1111/liv.15256. Epub 2022 May 11. PMID: 35324065; PMCID: PMC9373918.
A Retrospective Multicentric Study of 34 Patients with Niemann-Pick Type C Disease and Early Liver Involvement in France.
Gardin A, Mussini C, Héron B, Schiff M, Brassier A, Dobbelaere D, Broué P, Sevin C, Vanier MT, Habes D, Jacquemin E, Gonzales E.
J Pediatr. 2022 Oct 17:S0022-3476(22)00902-7. doi: 10.1016/j.jpeds.2022.10.015. Epub ahead of print. PMID: 36265573.
Acute Hepatitis of Unknown Etiology Among Young Children: Research Agenda by the ESPGHAN Hepatology Committee.
Indolfi G, Czubkowski P, Fitzpatrick E, Gonzales E , Gupte G, Mancell S, Mozer-Glassberg Y, Nicastro E, Norman J, Stephenne X, Zellos A, Samyn M.
J Pediatr Gastroenterol Nutr. 2022 Oct 1;75(4):543-548. doi: 10.1097/MPG.0000000000003567. Epub 2022 Sep 20. PMID: 35848740.
Best practice guidelines for France regarding the diagnosis and management of autoimmune hepatitis.
Duclos-Vallée JC, Debray D, De Martin E, Beux EL, Louvet A.
Clin Res Hepatol Gastroenterol. 2022 Apr;46(4):101871. doi: 10.1016/j.clinre.2022.101871. Epub 2022 Jan 31. PMID: 35108657.
Cannabis Use Is Inversely Associated with Metabolic Disorders in Hepatitis C-Infected Patients (ANRS CO22 Hepather Cohort).
Barré T, Bourlière M, Ramier C, Carrat F, Di Beo V, Protopopescu C, Marcellin F, Bureau M, Cagnot C, Dorival C, Zoulim F, Zucman-Rossi J, Duclos-Vallée JC , Fontaine H, Carrieri P, The Anrs/Afef Hepather Study Group.
J Clin Med. 2022 Oct 18;11(20):6135. doi: 10.3390/jcm11206135. PMID: 36294456; PMCID: PMC9605108.
Clinical outcomes after treatment with direct antiviral agents: beyond the virological response in patients with previous HCV-related decompensated cirrhosis.
Pageaux GP, Nzinga CL, Ganne N, Samuel D, Dorival C, Zoulim F, Cagnot C, Decaens T, Thabut D, Asselah T, Mathurin P, Habersetzer F, Bronowicki JP, Guyader D, Rosa I, Leroy V, Chazouilleres O, de Ledinghen V, Bourliere M, Causse X, Cales P, Metivier S, Loustaud-Ratti V, Riachi G, Alric L, Gelu-Simeon M, Minello A, Gournay J, Geist C, Tran A, Abergel A, Portal I, d'Alteroche L, Raffi F, Fontaine H, Carrat F, Pol S; French ANRS CO22 Hepather Cohort.
BMC Infect Dis. 2022 Jan 27;22(1):94. doi: 10.1186/s12879-022-07076-0. PMID: 35086481; PMCID: PMC8796427.
Diagnosis and Outcomes of Late-Onset Wilson's Disease: A National Registry-Based Study.
Nilles C, Obadia MA, Sobesky R, Dumortier J, Guillaud O, Laurencin C, Moreau C, Vanlemmens C, Ory-Magne F, de Ledinghen V, Bardou-Jacquet E, Fluchère F, Collet C, Oussedik-Djebrani N, Woimant F, Poujois A.
Mov Disord. 2022 Dec 27. doi: 10.1002/mds.29292. Epub ahead of print. PMID: 36573661.
Effect of an enteral amino acid blend on muscle and gut functionality in critically ill patients: a proof-of-concept randomized controlled trial
Heming N, Carlier R, Prigent H, Mekki A, Jousset C, Lofaso F, Ambrosi X, Bounab R, Maxime V, Mansart A, Crenn P, Moine P, Foltzer F, Cuenoud B, Konz T, Corthesy J, Beaumont M, Hartweg M, Roessle C, Preiser JC, Breuillé D, Annane D.
Crit Care. 2022 Nov 17;26(1):358. doi: 10.1186/s13054-022-04232-5. PMID: 36397118; PMCID: PMC9670468.
Effects of immunosuppressive drugs on COVID-19 severity in patients with autoimmune hepatitis.
Efe C, Lammert C, Taşçılar K, Dhanasekaran R, Ebik B, Higuera-de la Tijera F, Calışkan AR, Peralta M, Gerussi A, Massoumi H, Catana AM, Purnak T, Rigamonti C, Aldana AJG, Khakoo N, Nazal L, Frager S, Demir N, Irak K, Melekoğlu-Ellik Z, Kacmaz H, Balaban Y, Atay K, Eren F, Alvares-da-Silva MR, Cristoferi L, Urzua Á, Eşkazan T, Magro B, Snijders R, Barutçu S, Lytvyak E, Zazueta GM, Demirezer-Bolat A, Aydın M, Heurgue-Berlot A, De Martin E, Ekin N, Yıldırım S, Yavuz A, Bıyık M, Narro GC, Kıyıcı M, Akyıldız M, Kahramanoğlu-Aksoy E, Vincent M, Carr RM, Günşar F, Reyes EC, Harputluoğlu M, Aloman C, Gatselis NK, Üstündağ Y, Brahm J, Vargas NCE, Güzelbulut F, Garcia SR, Aguirre J, Anders M, Ratusnu N, Hatemi I, Mendizabal M, Floreani A, Fagiuoli S, Silva M, Idilman R, Satapathy SK, Silveira M, Drenth JPH, Dalekos GN, N Assis D, Björnsson E, Boyer JL, Yoshida EM, Invernizzi P, Levy C, Montano-Loza AJ, Schiano TD, Ridruejo E, Wahlin S.
Liver Int. 2022 Mar;42(3):607-614. doi: 10.1111/liv.15121. Epub 2021 Dec 13. PMID: 34846800.
Effects of odevixibat on pruritus and bile acids in children with cholestatic liver disease: Phase 2 study.
Baumann U, Sturm E, Lacaille F, Gonzalès E, Arnell H, Fischler B, Jørgensen MH, Thompson RJ, Mattsson JP, Ekelund M, Lindström E, Gillberg PG, Torfgård K, Soni PN.
Clin Res Hepatol Gastroenterol. 2021 Sep;45(5):101751. doi: 10.1016/j.clinre.2021.101751. Epub 2021 Jun 26. PMID: 34182185.
Galaxy Is a Suitable Bioinformatics Platform for the Molecular Diagnosis of Human Genetic DisordersUsing High-Throughput Sequencing Data Analysis: Five Years of Experience in a Clinical Laboratory.
Chappell K, Francou B, Habib C, Huby T, Leoni M, Cottin A, Nadal F, Adnet E, Paoli E, Oliveira C, Verstuyft C, Davit-Spraul A, Gaignard P, Lebigot E, Duclos-Vallee JC, Young J, Kamenicky P, Adams D, Echaniz-Laguna A, Gonzales E, Bouvattier C, Linglart A, Picard V, Bergoin E, Jacquemin E, Guiochon-Mantel A, Proust A, Bouligand J.
Clin Chem. 2022 Feb 1;68(2):313-321. doi: 10.1093/clinchem/hvab220. PMID: 34871369.
Genotypic and phenotypic spectrum of infantile liver failure due to pathogenic TRMU variants.
Vogel GF, Mozer-Glassberg Y, Landau YE, Schlieben LD, Prokisch H, Feichtinger RG, Mayr JA, Brennenstuhl H, Schröter J, Pechlaner A, Alkuraya FS, Baker JJ, Barcia G, Baric I, Braverman N, Burnyte B, Christodoulou J, Ciara E, Coman D, Das AM, Darin N, Della Marina A, Distelmaier F, Eklund EA, Ersoy M, Fang W, Gaignard P, Ganetzky RD, Gonzales E , Howard C, Hughes J, Konstantopoulou V, Kose M, Kerr M, Khan A, Lenz D, McFarland R, Margolis MG, Morrison K, Müller T, Murayama K, Nicastro E, Pennisi A, Peters H, Piekutowska-Abramczuk D, Rötig A, Santer R, Scaglia F, Schiff M, Shagrani M, Sharrard M, Soler-Alfonso C, Staufner C, Storey I, Stormon M, Taylor RW, Thorburn DR, Teles EL, Wang JS, Weghuber D, Wortmann S.
Genet Med. 2022 Oct 28:S1098-3600(22)00953-4. doi: 10.1016/j.gim.2022.09.015. Epub ahead of print. PMID: 36305855.
HCV eradication does not protect from fibrosis progression in patients with fibrosing cholestatic hepatitis after liver transplantation.
Coilly A, Sebagh M, Fougerou-Leurent C, Pageaux GP, Leroy V, Radenne S, Silvain C, Lebray P, Houssel-Debry P, Cagnot C, Rossignol E, Danjou H, Veislinger A, Samuel D, Duclos-Vallée JC, Dumortier J.
Clin Res Hepatol Gastroenterol. 2022 Nov;46(9):102024. doi: 10.1016/j.clinre.2022.102024. Epub 2022 Sep 16. PMID: 36122871.
Impact of COVID - 19 in patients awaiting liver transplantation.
Kounis I, Duhaut L, Breard T, Samuel D, Coilly A.
Liver Int. 2022 Jan;42(1):256-257. doi: 10.1111/liv.15105. Epub 2021 Nov 29. PMID: 34797949; PMCID: PMC8661909.
Impact of sleeve gastrectomy on renal function in patients with morbid obesity: a 1-year prospective cohort study
Sanchez D, Lebrun A, Somda S, Lainas P, Lamouri K, Prevot S, Njike-Nakseu M, Tranchart H, Gaillard M, Zaidan M, Balian A, Dagher I, Naveau S, Perlemuter G, Voican CS.
Langenbecks Arch Surg. 2022 Aug 9. doi: 10.1007/s00423-022-02647-w. Epub ahead of print. PMID: 35943574.
In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators.
Mareux E, Lapalus M, Ben Saad A, Zelli R, Lakli M, Riahi Y, Almes M, Banet M, Callebaut I, Decout JL, Falguières T, Jacquemin E, Gonzales E.
Int J Mol Sci. 2022 Sep 15;23(18):10758. doi: 10.3390/ijms231810758. PMID: 36142670; PMCID: PMC9502978.
Indocyanine green fluorescence patterns of hepatocellular carcinoma correlate with pathological and molecular features.
Sebagh M, Desterke C, Feray C, Hamelin J, Habib M, Samuel D, Rosmorduc O, Vibert E, Golse N.
HPB (Oxford). 2022 Oct 20:S1365-182X(22)01631-8. doi: 10.1016/j.hpb.2022.10.008. Epub ahead of print. PMID: 36411232.
Integrated Genomic Analysis Identifies Driver Genes and Cisplatin-Resistant Progenitor Phenotype in Pediatric Liver Cancer.
Hirsch TZ, Pilet J, Morcrette G, Roehrig A, Monteiro BJE, Molina L, Bayard Q, Trépo E, Meunier L, Caruso S, Renault V, Deleuze JF, Fresneau B, Chardot C, Gonzales E, Jacquemin E, Guerin F, Fabre M, Aerts I, Taque S, Laithier V, Branchereau S, Guettier C, Brugières L, Rebouissou S, Letouzé E, Zucman-Rossi J.
Cancer Discov. 2021 Oct;11(10):2524-2543. doi: 10.1158/2159-8290.CD-20-1809. Epub 2021 Apr 23. PMID: 33893148; PMCID: PMC8916021.(available on 2022-10-01)
International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts.
Korff S, Mostaguir K, Beghetti M, D'Antiga L, Debray D, Franchi-Abella S, Gonzales E, Guerin F, Hachulla AL, Lambert V, Makrythanasis P, Roduit N, Savale L, Senat MV, Spaltenstein J, van Steenbeek F, Wildhaber BE, Zwahlen M, McLin VA.
Orphanet J Rare Dis. 2022 Jul 19;17(1):284. doi: 10.1186/s13023-022-02412-8. PMID: 35854389; PMCID: PMC9295381.
Liver transplantation for autoimmune hepatitis: pre-transplant does not predict early post-transplant outcome.
Chouik Y, Francoz C, De Martin E, Guillaud O, Abergel A, Altieri M, Barbier L, Besch C, Chazouillères O, Conti F, Corpechot C, Dharancy S, Durand F, Duvoux C, Gugenheim J, Hardwigsen J, Hilleret MN, Houssel-Debry P, Kamar N, Minello A, Neau-Cransac M, Pageaux GP, Radenne S, Roux O, Saliba F, Samuel D, Vanlemmens C, Woehl-Jaegle ML, Leroy V, Duclos-Vallée JC, Dumortier J.
Liver Int. 2022 Dec 28. doi: 10.1111/liv.15500. Epub ahead of print. PMID: 36577700.
Maralixibat treatment response in Alagille syndrome is associated with improved health-related quality of life.
Kamath BM, Goldstein A, Howard R, Garner W, Vig P, Marden JR, Billmyer E, Anderson A, Kirson N, Jacquemin E, Gonzales E.
J Pediatr. 2022 Sep 10:S0022-3476(22)00791-0. doi: 10.1016/j.jpeds.2022.09.001. Epub ahead of print. PMID: 36096175.
Metabolic Disorders in Patients with Chronic Hepatitis B Virus Infection: Coffee as a Panacea? (ANRS CO22 Hepather Cohort).
Barré T, Fontaine H, Pol S, Ramier C, Di Beo V, Protopopescu C, Marcellin F, Bureau M, Bourlière M, Dorival C, Petrov-Sanchez V, Asselah T, Delarocque-Astagneau E, Larrey D, Duclos-Vallée JC, Carrat F, Carrieri P, On Behalf Of The Anrs/Afef Hepather Study Group.
Antioxidants (Basel). 2022 Feb 14;11(2):379. doi: 10.3390/antiox11020379. PMID: 35204261; PMCID: PMC8869416.
Modern therapeutic approaches to liver-related disorders.
Gardin A, Remih K, Gonzales E, Andersson ER, Strnad P.
J Hepatol. 2022 Jun;76(6):1392-1409. doi: 10.1016/j.jhep.2021.12.015. PMID: 35589258.
MRCK-Alpha and Its Effector Myosin II Regulatory Light Chain Bind ABCB4 and Regulate Its Membrane Expression.
Bruneau A, Delaunay JL, Durand-Schneider AM, Vauthier V, Ben Saad A, Aoudjehane L, El Mourabit H, Morichon R, Falguières T, Gautheron J, Housset C, Aït-Slimane T.
Cells. 2022 Feb 10;11(4):617. doi: 10.3390/cells11040617. PMID: 35203270; PMCID: PMC8870398.
Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study
Vandriel SM, Li LT, She H, Wang JS, Gilbert MA, Jankowska I, Czubkowski P, Gliwicz-Miedzińska D, Gonzales EM, Jacquemin E, Bouligand J, Spinner NB, Loomes KM, Piccoli DA, D'Antiga L, Nicastro E, Sokal É, Demaret T, Ebel NH, Feinstein JA, Fawaz R, Nastasio S, Lacaille F, Debray D, Arnell H, Fischler B, Siew S, Stormon M, Karpen SJ, Romero R, Kim KM, Baek WY, Hardikar W, Shankar S, Roberts AJ, Evans HM, Jensen MK, Kavan M, Sundaram SS, Chaidez A, Karthikeyan P, Sanchez MC, Cavalieri ML, Verkade HJ, Lee WS, Squires JE, Hajinicolaou C, Lertudomphonwanit C, Fischer RT, Larson-Nath C, Mozer-Glassberg Y, Arikan C, Lin HC, Bernabeu JQ, Alam S, Kelly DA, Carvalho E, Ferreira CT, Indolfi G, Quiros-Tejeira RE, Bulut P, Calvo PL, Önal Z, Valentino PL, Desai DM, Eshun J, Rogalidou M, Dezsőfi A, Wiecek S, Nebbia G, Pinto RB, Wolters VM, Tamara ML, Zizzo AN, Garcia J, Schwarz K, Beretta M, Sandahl TD, Jimenez-Rivera C, Kerkar N, Brecelj J, Mujawar Q, Rock N, Busoms CM, Karnsakul W, Lurz E, Santos-Silva E, Blondet N, Bujanda L, Shah U, Thompson RJ, Hansen BE, Kamath BM; Global ALagille Alliance (GALA) Study Group.
Hepatology. 2022 Aug 29. doi: 10.1002/hep.32761. Epub ahead of print. PMID: 36036223.
Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival.
Kounis I, Sebagh M, Evain M, Cailliez V, Roche B, De Martin E, Sobesky R, Guettier C, Allard MA, Golse N, Azoulay D, Vibert E, Duclos Vallee JC, Feray C, Samuel D, Coilly A.
Transplantation. 2022 Sep 19. doi: 10.1097/TP.0000000000004303. Epub ahead of print. PMID: 36117256.
Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial.
Thompson RJ, Arnell H, Artan R, Baumann U, Calvo PL, Czubkowski P, Dalgic B, D'Antiga L, Durmaz Ö, Fischler B, Gonzalès E, Grammatikopoulos T, Gupte G, Hardikar W, Houwen RHJ, Kamath BM, Karpen SJ, Kjems L, Lacaille F, Lachaux A, Lainka E, Mack CL, Mattsson JP, McKiernan P, Özen H, Rajwal SR, Roquelaure B, Shagrani M, Shteyer E, Soufi N, Sturm E, Tessier ME, Verkade HJ, Horn P.
Lancet Gastroenterol Hepatol. 2022 Sep;7(9):830-842. doi: 10.1016/S2468-1253(22)00093-0. Epub 2022 Jul 1. PMID: 35780807.
Paediatric acute liver failure: Confirm the outbreak, find the cause and explore the mechanisms.
Coilly A, Samuel D.
United European Gastroenterol J. 2022 Oct;10(8):789-790. doi: 10.1002/ueg2.12306. Epub 2022 Sep 12. PMID: 36094884; PMCID: PMC9557952.
Peripheral Arrangement of Steatosis Microvacuoles in Wilson's Disease.
Laurent-Bellue A, Sobesky R, Guettier C.
Clin Gastroenterol Hepatol. 2022 Apr;20(4):A17. doi: 10.1016/j.cgh.2021.08.005. Epub 2021 Aug 5. PMID: 34365001.
Prediagnostic alterations in circulating bile acid profiles in the development of hepatocellular carcinoma.
Stepien M, Lopez-Nogueroles M, Lahoz A, Kühn T, Perlemuter G, Voican C, Ciocan D, Boutron-Ruault MC, Jansen E, Viallon V, Leitzmann M, Tjønneland A, Severi G, Mancini FR, Dong C, Kaaks R, Fortner RT, Bergmann MM, Boeing H, Trichopoulou A, Karakatsani A, Peppa E, Palli D, Krogh V, Tumino R, Sacerdote C, Panico S, Bueno-de-Mesquita HB, Skeie G, Merino S, Ros RZ, Sánchez MJ, Amiano P, Huerta JM, Barricarte A, Sjöberg K, Ohlsson B, Nyström H, Werner M, Perez-Cornago A, Schmidt JA, Freisling H, Scalbert A, Weiderpass E, Christakoudi S, Gunter MJ, Jenab M.
Int J Cancer. 2022 Apr 15;150(8):1255-1268. doi: 10.1002/ijc.33885. Epub 2022 Jan 11. PMID: 34843121.
Predictive factors for hepatocellular carcinoma in chronic hepatitis B using structural equation modeling: a prospective cohort study.
Lam L, Fontaine H, Bourliere M, Lusivika-Nzinga C, Dorival C, Thabut D, Zoulim F, Habersetzer F, Asselah T, Duclos-Vallee JC, Bronowicki JP, Mathurin P, Decaens T, Ganne N, Guyader D, Leroy V, Rosa I, De Ledinghen V, Cales P, Causse X, Larrey D, Chazouilleres O, Gelu-Simeon M, Loustaud-Ratti V, Metivier S, Alric L, Riachi G, Gournay J, Minello A, Tran A, Geist C, Abergel A, Raffi F, D'Alteroche L, Portal I, Lapidus N, Pol S, Carrat F; ANRS/AFEF Hepather study group.
Clin Res Hepatol Gastroenterol. 2021 Sep;45(5):101713. doi: 10.1016/j.clinre.2021.101713. Epub 2021 Apr 27. PMID: 33930591.
SARS-CoV-2 vaccination and risk of severe COVID-19 outcomes in patients with autoimmune hepatitis.
Efe C, Taşçılar K, Gerussi A, Bolis F, Lammert C, Ebik B, Stättermayer AF, Cengiz M, Gökçe DT, Cristoferi L, Peralta M, Massoumi H, Montes P, Cerda E, Rigamonti C, Yapalı S, Adali G, Çalışkan AR, Balaban Y, Eren F, Eşkazan T, Barutçu S, Lytvyak E, Zazueta GM, Kayhan MA, Heurgue-Berlot A, De Martin E, Yavuz A, Bıyık M, Narro GC, Duman S, Hernandez N, Gatselis NK, Aguirre J, Idilman R, Silva M, Mendizabal M, Atay K, Güzelbulut F, Dhanasekaran R, Montano-Loza AJ, Dalekos GN, Ridruejo E, Invernizzi P, Wahlin S.
J Autoimmun. 2022 Oct;132:102906. doi: 10.1016/j.jaut.2022.102906. Epub 2022 Sep 7. PMID: 36088883; PMCID: PMC9448709.
Spontaneous evolution patterns of focal congenital hepatic hemangiomas: a case series of 25 patients.
Rutten C, Ladarre D, Ackermann O, Gonzales E, Guettier C, Franchi-Abella S.
Pediatr Radiol. 2022 May;52(6):1048-1060. doi: 10.1007/s00247-021-05277-4. Epub 2022 Feb 28. PMID: 35229180.
Urgent Liver Transplantation for Acute Liver Failure in Pregnant Women: The Optimum Timing for Delivery.
Ichai P, Bouchghoul H, Laurent-Bellue A, Sacleux SC, Boudon M, Cherqui D, Tortajada P, Braun M, Lemaitre E, Pittau G, Ordan MA, Levi S, Azoulay D, Fernandez H, Guettier C, Samuel D, Saliba F.
Transplantation. 2022 Aug 31. doi: 10.1097/TP.0000000000004290. Epub ahead of print. PMID: 36042543.
Targeted-Capture Next-Generation Sequencing in Diagnosis Approach of Pediatric Cholestasis.
Almes M, Spraul A, Ruiz M, Girard M, Roquelaure B, Laborde N, Gottrand F, Turquet A, Lamireau T, Dabadie A, Bonneton M, Thebaut A, Rohmer B, Lacaille F, Broué P, Fabre A, Mention-Mulliez K, Bouligand J, Jacquemin E, Gonzales E.
Diagnostics (Basel). 2022 May 7;12(5):1169. doi: 10.3390/diagnostics12051169. PMID: 35626323; PMCID: PMC9140938.
The role of prehabilitation on short-term outcomes after liver transplantation - A review of the literature and expert panel recommendations.
Vinaixa C, Martínez Palli G, Miliken D, Sellers D, Dharancy S, Spiro M, Aristotle Raptis D, Samuel D; ERAS4OLT.org Working Group.
Clin Transplant. 2022 Apr 24:e14686. doi: 10.1111/ctr.14686. Epub ahead of print. PMID: 35462421.
The optimal immunosuppression management to prevent early rejection after liver transplantation: A systematic review of the literature and expert panel recommendations.
De Martin E, Londoño MC, Emamaullee J, Lerut J, Potts J, Aluvihare V, Spiro M, Raptis DA, McCaughan G; ERAS4OLT.org Working Group: Claus Niemann, San Francisco, CA, USA, Joerg-Matthias Pollok, London, UK, Marina Berenguer, Valencia, Spain, Pascale Tinguely, London, UK, Adrian Gustar, Milano, Italy, Camila Hidalgo, London, UK.
Clin Transplant. 2022 Feb 10:e14614. doi: 10.1111/ctr.14614. Epub ahead of print. PMID: 35143096.

A Model for Early Endoscopic Detection of High-Risk Gastroesophageal Varices in Children With Biliary Atresia.
Ackermann O, de Boissieu P, Bernard O, Gonzalès E, Jacquemin E, Duché M.
J Pediatr Gastroenterol Nutr. 2021 Dec 28. doi: 10.1097/MPG.0000000000003375. Epub ahead of print. PMID: 34984987.
Adenosine kinase deficiency: Three new cases and diagnostic value of hypermethioninemia.
Becker PH, Demir Z, Mozer Glassberg Y, Sevin C, Habes D, Imbard A, Mussini C, Rozenfeld Bar Lev M, Davit-Spraul A, Benoist JF, Thérond P, Slama A, Jacquemin E, Gonzales E, Gaignard P.
Mol Genet Metab. 2021 Jan;132(1):38-43. doi: 10.1016/j.ymgme.2020.11.007. Epub 2020 Nov 28. PMID: 33309011.
Assessment of hepatic steatosis by controlled attenuation parameter using the M and XL probes: an individual patient data meta-analysis.
Petroff D, Blank V, Newsome PN, Shalimar, Voican CS, Thiele M, de Lédinghen V, Baumeler S, Chan WK, Perlemuter G, Cardoso AC, Aggarwal S, Sasso M, Eddowes PJ, Allison M, Tsochatzis E, Anstee QM, Sheridan D, Cobbold JF, Naveau S, Lupsor-Platon M, Mueller S, Krag A, Irles-Depe M, Semela D, Wong GL, Wong VW, Villela-Nogueira CA, Garg H, Chazouillères O, Wiegand J, Karlas T
Lancet Gastroenterol Hepatol. 2021 Mar;6(3):185-198. doi: 10.1016/S2468-1253(20)30357-5. Epub 2021 Jan 16. PMID: 33460567.
ATP7B variant spectrum in a French pediatric Wilson disease cohort
Eduardo Couchonnal, Sophie Bouchard, Thomas Damgaard Sandahl, Cecile Pagan, Laurence Lion-François, Olivier Guillaud, Dalila Habes, Dominique Debray, Thierry Lamireau, Pierre Broué, Alexandre Fabre, Claire Vanlemmens, Rodolphe Sobesky, Frederic Gottrand, Laure Bridoux-Henno, Abdelouahed Belmali, Aurelia Poujois, Anne Sophie Brunet, Alain Lachaux, Muriel Bost
Eur J Med Genet. 2021 Oct;64(10):104305. doi: 10.1016/j.ejmg.2021.104305. Epub 2021 Aug 13. Erratum in: Eur J Med Genet. 2021 Sep 17;:104341. PMID: 34400371.
Blood microbiota and metabolomic signature of major depression before and after antidepressant treatment: a prospective case-control study.
Dragos Ciocan, Anne-Marie Cassard, Laurent Becquemont, Céline Verstuyft, Cosmin Sebastian Voican, Khalil El Asmar, Romain Colle, Denis David, Séverine Trabado, Bruno Feve, Philippe Chanson, Gabriel Perlemuter, Emmanuelle Corruble.
J Psychiatry Neurosci. 2021 May 19;46(3):E358-E368, PMID: 34008933, DOI: 10.1503/jpn.200159
Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre.
Lambert V, Ladarre D, Fortas F, Durand P, Hervé P, Gonzales E, Guérin F, Savale L, McLin VA, Ackermann O, Franchi-Abella S; International Registry of Congenital Portosystemic Shunts (IRCPSS).
Arch Cardiovasc Dis. 2021 Mar;114(3):221-231. doi: 10.1016/j.acvd.2020.10.003. Epub 2020 Dec 3. PMID: 33281106
Changing Trends in Liver Transplantation: Challenges and Solutions.
Ivanics T, Abreu P, De Martin E, Sapisochin G.
Transplantation. 2021 Apr 1;105(4):743-756. doi: 10.1097/TP.0000000000003454. PMID: 32910093.
Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.
Guérin F, Franchi Abella S, McLin V, Ackermann O, Girard M, Cervoni JP, Savale L, Hernandez-Gea V, Valla D, Hillaire S, Dutheil D, Bureau C, Gonzales E, Plessier A.
Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):452-459. doi: 10.1016/j.clinre.2020.03.004. Epub 2020 Apr 9. PMID: 32279979.
Donor HLA Class 1 Evolutionary Divergence Is a Major Predictor of Liver Allograft Rejection : A Retrospective Cohort Study.
Féray C, Taupin JL, Sebagh M, Allain V, Demir Z, Allard MA, Desterke C, Coilly A, Saliba F, Vibert E, Azoulay D, Guettier C, Chatton A, Debray D, Caillat-Zucman S, Samuel D.
Ann Intern Med. 2021 Aug 24. doi: 10.7326/M20-7957. Epub ahead of print. PMID: 34424731.
Early liver transplantation for corticosteroid non-responders with acute severe autoimmune hepatitis: The SURFASA score.
De Martin E, Coilly A, Chazouillères O, Roux O, Peron JM, Houssel-Debry P, Artru F, Silvain C, Ollivier-Hourmand I, Duvoux C, Heurgue A, Barge S, Ganne-Carrié N, Pageaux GP, Besch C, Bourlière M, Fontaine H, de Ledinghen V, Dumortier J, Conti F, Radenne S, Debette-Gratien M, Goria O, Durand F, Potier P, Di Martino V, Reboux N, Ichai P, Sebagh M, Mathurin P, Agostini H, Samuel D, Duclos-Vallée JC
FILFOIE consortium – France.
J Hepatol. 2021 Jun;74(6):1325-1334. doi: 10.1016/j.jhep.2020.12.033. Epub 2021 Jan 24. PMID: 33503489.
Effects of odevixibat on pruritus and bile acids in children with cholestatic liver disease: Phase 2 study.
Baumann U, Sturm E, Lacaille F, Gonzalès E, Arnell H, Fischler B, Jørgensen MH, Thompson RJ, Mattsson JP, Ekelund M, Lindström E, Gillberg PG, Torfgård K, Soni PN.
Clin Res Hepatol Gastroenterol. 2021 Jun 25:101751. doi: 10.1016/j.clinre.2021.101751. PMID: 34182185.
Endoscopic Retrograde Cholangiopancreatography in Infants: Availability Under Threat: A Survey on Availability, Need, and Clinical Practice in Europe and Israel.
Koot BGP, Kelly DA, Hadzic N, Gonzales E, Hierro L, Davenport M, Keil R, Fockens P, Baumann U.
J Pediatr Gastroenterol Nutr. 2020 Aug;71(2):e54-e58. doi: 10.1097/MPG.0000000000002752. PMID: 32304552.
External validation of LCR1-LCR2, a multivariable HCC risk calculator, in patients with chronic HCV.
Poynard T, Lacombe JM, Deckmyn O, Peta V, Akhavan S, de Ledinghen V, Zoulim F, Samuel D, Mathurin P, Ratziu V, Thabut D, Housset C, Fontaine H, Pol S, Carrat F.
JHEP Rep. 2021 Apr 24;3(4):100298. doi: 10.1016/j.jhepr.2021.100298. PMID: 34142073; PMCID: PMC8187244.
Functional rescue of an ABCB11 mutant by ivacaftor: A new targeted pharmacotherapy approach in bile salt export pump deficiency.
Mareux E, Lapalus M, Amzal R, Almes M, Aït-Slimane T, Delaunay JL, Adnot P, Collado-Hilly M, Davit-Spraul A, Falguières T, Callebaut I, Gonzales E, Jacquemin E.
Liver Int. 2020 Aug;40(8):1917-1925. doi: 10.1111/liv.14518. Epub 2020 Jun 8. PMID: 32433800.
Galaxy Is a Suitable Bioinformatics Platform for the Molecular Diagnosis of Human Genetic Disorders Using High-Throughput Sequencing Data Analysis. Five Years of Experience in a Clinical Laboratory.
Chappell K, Francou B, Habib C, Huby T, Leoni M, Cottin A, Nadal F, Adnet E, Paoli E, Oliveira C, Verstuyft C, Davit-Spraul A, Gaignard P, Lebigot E, Duclos-Vallee JC, Young J, Kamenicky P, Adams D, Echaniz-Laguna A, Gonzales E, Bouvattier C, Linglart A, Picard V, Bergoin E, Jacquemin E, Guiochon-Mantel A, Proust A, Bouligand J.
Clin Chem. 2021 Dec 6:hvab220. doi: 10.1093/clinchem/hvab220. Epub ahead of print. PMID: 34871369
Glycerol Phenylbutyrate Therapy in Progressive Familial Intrahepatic Cholestasis Type 2.
Almes M, Jobert A, Lapalus M, Mareux E, Gonzales E, Jacquemin E.
J Pediatr Gastroenterol Nutr. 2020 Jun;70(6):e139-e140. doi: 10.1097/MPG.0000000000002713. PMID: 32443059.
HCV Cure and Cannabis Abstinence Facilitate Tobacco Smoking Quit Attempts in HIV-HCV Co-Infected Patients (ANRS CO13 HEPAVIH Cohort Study).
Barré T, Mercié P, Marcellin F, Esterle L, Duvivier C, Teicher E, Bureau M, Chas J, Salmon-Céron D, Sogni P, Carrieri MP, Wittkop L, Protopopescu C; ANRS CO13 HEPAVIH Study Group.
AIDS Behav. 2021 Apr 27. doi: 10.1007/s10461-021-03277-x. Online ahead of print. PMID: 33903998
In vitro functional rescue by ivacaftor of an ABCB11 variant involved in PFIC2 and intrahepatic cholestasis of pregnancy.
Mareux E, Lapalus M, Ben-Saad A, Callebaut I, Falguières T, Gonzales E, Jacquemin E.
Orphanet J Rare Dis. 2021 Nov 18;16(1):484. doi: 10.1186/s13023-021-02125-4. PMID: 34794484; PMCID: PMC8600881.
Long term results of liver transplantation for alpha-1 antitrypsin deficiency.
Guillaud O, Jacquemin E, Couchonnal E, Vanlemmens C, Francoz C, Chouik Y, Conti F, Duvoux C, Hilleret MN, Kamar N, Houssel-Debry P, Neau-Cransac M, Pageaux GP, Gonzales E, Ackermann O, Gugenheim J, Lachaux A, Ruiz M, Radenne S, Debray D, Lacaille F, McLin V, Duclos-Vallée JC, Samuel D, Coilly A, Dumortier J.
Dig Liver Dis. 2021 May;53(5):606-611. doi: 10.1016/j.dld.2020.10.016. Epub 2020 Nov 1. PMID: 33139195.
Long-term results of pediatric liver transplantation for autoimmune liver disease.
Couchonnal E, Jacquemin E, Lachaux A, Ackermann O, Gonzales E , Lacaille F, Debray D, Boillot O, Guillaud O, Wildhaber BE, Chouik Y, McLin V, Dumortier J.
Clin Res Hepatol Gastroenterol. 2021 May;45(3):101537. doi: 10.1016/j.clinre.2020.08.013. Epub 2020 Oct 17. PMID: 33077391.
Molecular Regulation of Canalicular ABC Transporters.
Amel Ben Saad, Alix Bruneau, Elodie Mareux, Martine Lapalus, Jean-Louis Delaunay, Emmanuel Gonzales, Emmanuel Jacquemin, Tounsia Aït-Slimane, Thomas Falguières.
Review Int J Mol Sci. 2021 Feb 20;22(4):2113. doi: 10.3390/ijms22042113. PMID: 33672718.
Multikinase inhibitor-induced liver injury in patients with cancer: A review for clinicians.
Houron C, Danielou M, Mir O, Fromenty B, Perlemuter G, Voican CS.
Crit Rev Oncol Hematol. 2021 Jan;157:103127. doi: 10.1016/j.critrevonc.2020.103127. Epub 2020 Oct 21. PMID: 33161366.
Pediatric Wilson's Disease: Phenotypic, Genetic Characterization and Outcome of 182 Children in France.
Couchonnal E, Lion-François L, Guillaud O, Habes D, Debray D, Lamireau T, Broué P, Fabre A, Vanlemmens C, Sobesky R, Gottrand F, Bridoux-Henno L, Dumortier J, Belmalih A, Poujois A, Jacquemin E, Brunet AS, Bost M, Lachaux A.
J Pediatr Gastroenterol Nutr. 2021 Jun 1. doi: 10.1097/MPG.0000000000003196. Epub ahead of print. PMID: 34091542.
Peripheral Arrangement of Steatosis Microvacuoles in Wilson's Disease.
Laurent-Bellue A, Sobesky R, Guettier C.
Clin Gastroenterol Hepatol. 2021 Aug 5:S1542-3565(21)00839-9. doi: 10.1016/j.cgh.2021.08.005. Epub ahead of print. PMID: 34365001.
Pharmacological Premature Termination Codon Readthrough of ABCB11 in Bile Salt Export Pump Deficiency: An In Vitro Study.
Amzal R, Thébaut A, Lapalus M, Almes M, Grosse B, Mareux E, Collado-Hilly M, Davit-Spraul A, Bidou L, Namy O, Jacquemin E, Gonzales E.
Hepatology. 2021 Apr;73(4):1449-1463. doi: 10.1002/hep.31476. PMID: 32702170.
Phase 3, Multicenter Open-Label study to investigate the efficacy of elbasvir and grazoprevir fixed-dose combination for 8 weeks in treatment-naïve, HCV GT1b-infected patients, with non-severe fibrosis.
Abergel A, Asselah T, Mallat A, Chanteranne B, Faure F, Larrey D, Gournay J, Loustaud-Ratti V, Di Martino V, Fouchard-Hubert I, Pol S, Bailly F, Samuel D, Tran A, Dodel M, Andant N, Lamblin G, Muti L, Reymond M, Teilhet C, Pereira B, Buchard B.
Liver Int. 2020 Aug;40(8):1853-1859. doi: 10.1111/liv.14502. Epub 2020 Jun 1. PMID: 32383275.
Portopulmonary hypertension in the current era of pulmonary hypertension management
Savale L, Guimas M, Ebstein N, Fertin M, Jevnikar M, Renard S, Horeau-Langlard D, Tromeur C, Chabanne C, Prevot G, Chaouat A, Moceri P, Artaud-Macari É, Degano B, Tresorier R, Boissin C, Bouvaist H, Simon AC, Riou M, Favrolt N, Palat S, Bourlier D, Magro P, Cottin V, Bergot E, Lamblin N, Jaïs X, Coilly A, Durand F, Francoz C, Conti F, Hervé P, Simonneau G, Montani D, Duclos-Vallée JC, Samuel D, Humbert M, De Groote P, Sitbon O.
J Hepatol. 2020 Jul;73(1):130-139. doi: 10.1016/j.jhep.2020.02.021. Epub 2020 Mar 5. Erratum in: J Hepatol. 2020 Nov;73(5):1293-1294. PMID: 32145258.
Post-Liver Transplantation Sinusoidal Obstruction Syndrome With Refractory Ascites Induced by Mycophenolate Mofetil.
Poli E, Kounis I, Guettier C, Verstuyft C, Coilly A, Sobesky R, Feray C, Vibert E, Ciacio O, Samuel D, Bismuth H, Duclos-Vallée JC.
Hepatology. 2020 Apr;71(4):1508-1510. doi: 10.1002/hep.30984. Epub 2020 Mar 31. PMID: 31597193
Pre-diagnostic alterations in circulating bile acid profiles in the development of hepatocellular carcinoma.
Stepien M, Lopez-Nogueroles M, Lahoz A, Kühn T, Perlemuter G, Voican C, Ciocan D, Boutron-Ruault MC, Jansen E, Viallon V, Leitzmann M, Tjønneland A, Severi G, Mancini FR, Dong C, Kaaks R, Fortner RT, Bergmann MM, Boeing H, Trichopoulou A, Karakatsani A, Peppa E, Palli D, Krogh V, Tumino R, Sacerdote C, Panico S, Bueno-de-Mesquita HB, Skeie G, Merino S, Ros RZ, Sánchez MJ, Amiano P, Huerta JM, Barricarte A, Sjöberg K, Ohlsson B, Nyström H, Werner M, Perez-Cornago A, Schmidt JA, Freisling H, Scalbert A, Weiderpass E, Christakoudi S, Gunter MJ, Jenab M.
Int J Cancer. 2021 Nov 29. doi: 10.1002/ijc.33885. Epub ahead of print. PMID: 34843121.
Predictive Factors for Hepatocellular Carcinoma in Chronic Hepatitis B Using Structural Equation Modeling: A Prospective Cohort Study.
Lam L, Fontaine H, Bourliere M, Lusivika-Nzinga C, Dorival C, Thabut D, Zoulim F, Habersetzer F, Asselah T, Duclos-Vallee JC, Bronowicki JP, Mathurin P, Decaens T, Ganne N, Guyader D, Leroy V, Rosa I, Ledinghen V, Cales P, Causse X, Larrey D, Chazouilleres O, Gelu-Simeon M, Loustaud-Ratti V, Metivier S, Alric L, Riachi G, Gournay J, Minello A, Tran A, Geist C, Abergel A, Raffi F, D'alteroche L, Portal I, Lapidus N, Pol S, Carrat F; ANRS AFEF Hepather study group.
Clin Res Hepatol Gastroenterol. 2021 Apr 27:101713. doi: 10.1016/j.clinre.2021.101713. Online ahead of print. PMID: 33930591
RAB10 Interacts with ABCB4 and Regulates Its Intracellular Traffic
Amel Ben Saad, Virginie Vauthier, Martine Lapalus, Elodie Mareux, Evangéline Bennana, Anne-Marie Durand-Schneider, Alix Bruneau, Jean-Louis Delaunay, Emmanuel Gonzales, Chantal Housset, Tounsia Aït-Slimane, François Guillonneau, Emmanuel Jacquemin, Thomas Falguières.
Int J Mol Sci. 2021 Jun 30;22(13):7087. doi: 10.3390/ijms22137087. PMID: 34209301.
Reply to: "Validation of the SURFASA score to define steroid responsiveness in patients with acute autoimmune hepatitis".
De Martin E, Duclos-Vallée JC.
J Hepatol. 2021 Nov 10:S0168-8278(21)02167-X. doi: 10.1016/j.jhep.2021.11.002. Epub ahead of print. PMID: 34774640.
Seronegative Autoimmune Hepatitis-Associated Severe Aplastic Anemia: Looking for the Best Treatment
Giuseppe Maggiore, Andrea Pietrobattista, Daniela Liccardo, Marco Sciveres, Silvia Nastasio, Emmanuel Jacquemin, Olivier Bernard.
J Pediatr Gastroenterol Nutr. 2021 May 11, PMID: 34016885, DOI: 10.1097/MPG.0000000000003165
Specific microbiome profile in Takayasu's arteritis and giant cell arteritis
Anne Claire Desbois, Dragos Ciocan, David Saadoun, Gabriel Perlemuter, Patrice Cacoub.
Sci Rep. 2021; 11: 5926.Published online 2021 Mar 15. doi: 10.1038/s41598-021-84725-5. PMID: 33723291. PMCID: PMC7961033
Systemic inflammation and liver cirrhosis complications: Driving or secondary event? How to square the circle?
Samuel D.
J Hepatol. 2021 Mar;74(3):508-510. doi: 10.1016/j.jhep.2021.01.001. Epub 2021 Jan 18. PMID: 33478857.
Ursodeoxycholic acid therapy throughout pregnancy in women affected with chronic cholestasis of childhood: No evidence for teratogenicity.
Lykavieris P, Bernard O, Jacquemin E.
Clin Res Hepatol Gastroenterol. 2021 May;45(3):101472. doi: 10.1016/j.clinre.2020.05.020. Epub 2020 Jun 18. PMID: 32565202.

A Model to Identify Heavy Drinkers at High Risk for Liver Disease Progression.
Claire Delacôte, Pierre Bauvin, Alexandre Louvet, Flavien Dautrecque, Line Carolle Ntandja Wandji, Guillaume Lassailly, Cosmin Voican, Gabriel Perlemuter, Sylvie Naveau, Philippe Mathurin, Sylvie Deuffic-Burban.
Clin Gastroenterol Hepatol. 2020 Sep;18(10):2315-2323.e6. doi: 10.1016/j.cgh.2019.12.041. Epub 2020 Jan 11.
A Novel CFC1 Mutation in a Family with Heterotaxy and Biliary Atresia Splenic Malformation Syndromes.
Gonzales E, Davit-Spraul A, Jacquemin E.
J Pediatr Gastroenterol Nutr. 2020;70:e24-e25.DOI: 10.1097/MPG.0000000000002531
Adenosine kinase deficiency: Three new cases and diagnostic value of hypermethioninemia.
Becker PH, Demir Z, Mozer Glassberg Y, Sevin C, Habes D, Imbard A, Mussini C, Rozenfeld Bar Lev M, Davit-Spraul A, Benoist JF, Thérond P, Slama A, Jacquemin E, Gonzales E*, Gaignard P*.
Mol Genet Metab. 2021:132:38-43.doi: 10.1016/j.ymgme.2020.11.007
Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre.
Lambert V, Ladarre D, Fortas F, Durand P, Hervé P, Gonzales E, Guérin F, Savale L, McLin VA, Ackermann O, Franchi-Abella S.
International Registry of Congenital Portosystemic Shunts (IRCPSS). Arch Cardiovasc Dis. 2020: Dec 3:S1875-2136(20)30237-0. doi: 10.1016/j.acvd.2020.10.003. Online ahead of print.
Diagnostic protocol of neonatal and infantile cholestasis: can it be improved?
Marco Sciveres, Francesco Cirillo, Emmanuel Jacquemin, Giuseppe Maggiore.
Comment J Pediatr. 2020 Jan;216:247. doi: 10.1016/j.jpeds.2019.09.045. Epub 2019 Oct 9.PMID: 31606154
Endoscopic Retrograde Cholangiopancreatography in infants: availability under threat: A survey on availability, need and clinical practice in Europe and Israel.
Koot BG, Kelly DA, Hadžić D, Gonzales E, Hierro L, Davenport M, Keil R, Fockens P, Baumann U.
J Pediatr Gastroenterol Nutr. 2020; 71(2):e54-e58.10.1097/MPG.0000000000002752.
Functional rescue of an ABCB11 mutant by ivacaftor: a new targeted pharmacotherapy approach in bile salt export pump deficiency.
Mareux E*, Lapalus M*, Amzal R, Almès M, Aït Slimane T, Delaunay JL, Adnot P, Collado-Hilly M, Davit Spraul A, Callebaut I, Gonzales E*, Jacquemin E*.
Liver Int. 2020;40:1917-1925.DOI: 10.1111/liv.14518
Geographical Disparities of Outcomes of Hepatocellular Carcinoma in France: The Heavier Burden of Alcohol Compared to Hepatitis C.
Charlotte E Costentin, Philippe Sogni, Bruno Falissard, Jean-Claude Barbare, NoelleBendersky, Olivier Farges, Nathalie Goutte.
Comparative Study Dig Dis Sci. 2020 Jan;65(1):301-311. doi: 10.1007/s10620-019-05724-1. Epub 2019 Jul 25.PMID: 31346950
Glycerol Phenylbutyrate Therapy in Progressive Familial Intrahepatic Cholestasis Type 2.
Almes M, Jobert A, Lapalus M, Mareux E, Gonzales E, Jacquemin E.
J Pediatr Gastroenterol Nutr 2020;70:e139-e140.10.1097/MPG.0000000000002713
Long term results of liver transplantation for alpha-1 antitrypsin deficiency.
Guillaud O, Jacquemin E, Couchonnal E, Vanlemmens C, Francoz C, Chouik Y, Conti F, Duvoux C, Hilleret MN, Kamar N, Houssel-Debry P, Neau-Cransac M, Pageaux GP, Gonzales E, Ackermann O, Gugenheim J, Lachaux A, Ruiz M, Radenne S, Debray D, Lacaille F, McLin V, Duclos-Vallée JC, Samuel D, Coilly A, Dumortier J.
Dig Liver Dis. 2020:S1590-8658(20)30970-1. doi: 10.1016/j.dld.2020.10.016. Online ahead of print.
Long-term results of pediatric liver transplantation for autoimmune liver disease.
Couchonnal E, Jacquemin E, Lachaux A, Ackermann O, Gonzales E, Lacaille F, Debray D, Boillot O, Guillaud O, Wildhaber BE, Chouik Y, McLin V, Dumortier J.
Clin Res Hepatol Gastroenterol. 2020 Oct 16:S2210-7401(20)30275-8. doi: 10.1016/j.clinre.2020.08.013. Online ahead of print.
Management of liver failure in general intensive care unit.
C Paugam-Burtz, E Levesque, A Louvet, D Thabut, R Amathieu, C Bureau, C Camus, G Chanques, S Faure, M Ferrandière, C Francoz, A Galbois, T Gustot, C Ichai, P Ichai, S Jaber, T Lescot, R Moreau, S Roullet, F Saliba, T Thévenot, L Velly, E Weiss.
Review Anaesth Crit Care Pain Med. 2020 Feb;39(1):143-161. doi: 10.1016/j.accpm.2019.06.014. Epub 2019 Sep 13.PMID: 31525507. DOI: 10.1016/j.accpm.2019.06.014
Optimization of the transition process of youth with liver disease in adulthood: A position paper from FILFOIE, the French network for paediatric and adult rare liver diseases.
Teresa Maria Antonini, Muriel Girard, Dalila Habes, Odile Goria, Dominique Debray.
Practice Guideline Clin Res Hepatol Gastroenterol. 2020 Apr;44(2):135-141. doi:10.1016/j.clinre.2019.07.018. Epub 2019 Sep 26.PMID: 31564624
Parvovirus B19 infection: A challenging cause of fulminant hepatitis.
Gille N, Roque-Afonso AM, Sebagh M, Rudler M, Thabut D, Duclos-Vallée JC, Samuel D, Coilly A.
Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):e89-e91. doi: 10.1016/j.clinre.2019.12.004. Epub 2020 Jan 6. PMID: 31917176 No abstract available.
Pharmacological premature termination codon readthrough of ABCB11 in bile salt export pump deficiency: an in vitro study.
Amzal R, Thébaut A, Lapalus M, Almes M, Mareux E, Collado-Hilly M, Davit Spraul A, Bidou L, Namy O, Jacquemin E*, Gonzales E*.
Hepatology. 2020 Jul 23. doi: 10.1002/hep.31476.
Phase 3, Multicenter Open-Label study to investigate the efficacy of elbasvir and grazoprevir fixed-dose combination for 8 weeks in treatment-naïve, HCV GT1b-infected patients, with non-severe fibrosis.
Armand Abergel, Tarik Asselah, Arianne Mallat, Brigitte Chanteranne, Frederic Faure, Dominique Larrey, Jerome Gournay, Veronique Loustaud-Ratti, Vincent Di Martino, Isabelle Fouchard-Hubert, Stanislas Pol, Francois Bailly, Didier Samuel, Albert Tran, Marie Dodel, Nicolas Andant, Geraldine Lamblin, Leon Muti, Maud Reymond, Camille Teilhet, Bruno Pereira, Benjamin Buchard.
Liver Int. 2020 Aug;40(8):1853-1859. doi: 10.1111/liv.14502. Epub 2020 Jun 1.PMID: 32383275
Plasma citrulline correlates with basolateral amino acid transporter LAT4 expression in human small intestine.
Stefano Maric, Pascal Flüchter, Laura Chiara Guglielmetti, Ralph Fabian Staerkle, Tom Sasse, Tanja Restin, Christoph Schneider, Stefan Gerhard Holland-Cunz, Pascal Crenn, Raphael Nicolas Vuille-dit-Bille*
Clinical Nutrition, https://doi.org/10.1016/j.clnu.2020.10.003
Portopulmonary hypertension in the current era of pulmonary hypertension management.
Savale L, Guimas M, Ebstein N, Fertin M, Jevnikar M, Renard S, Horeau-Langlard D, Tromeur C, Chabanne C, Prevot G, Chaouat A, Moceri P, Artaud-Macari É, Degano B, Tresorier R, Boissin C, Bouvaist H, Simon AC, Riou M, Favrolt N, Palat S, Bourlier D, Magro P, Cottin V, Bergot E, Lamblin N, Jaïs X, Coilly A, Durand F, Francoz C, Conti F, Hervé P, Simonneau G, Montani D, Duclos-Vallée JC, Samuel D, Humbert M, De Groote P, Sitbon O.
J Hepatol. 2020 Jul;73(1):130-139. doi: 10.1016/j.jhep.2020.02.021. Epub 2020 Mar 5.PMID: 32145258
Corrigendum to: "Portopulmonary hypertension in the current era of pulmonary hypertension management" [J Hepatol (2020);73:130-139].
Savale L, Guimas M, Ebstein N, Fertin M, Jevnikar M, Renard S, Horeau-Langlard D, Tromeur C, Chabanne C, Prevot G, Chaouat A, Moceri P, Artaud-Macari É, Degano B, Tresorier R, Boissin C, Bouvaist H, Simon AC, Riou M, Favrolt N, Palat S, Bourlier D, Magro P, Cottin V, Bergot E, Lamblin N, Jaïs X, Coilly A, Durand F, Francoz C, Conti F, Hervé P, Simonneau G, Montani D, Duclos-Vallée JC, Samuel D, Humbert M, De Groote P, Sitbon O.
J Hepatol. 2020 Nov;73(5):1293-1294. doi: 10.1016/j.jhep.2020.08.023. Epub 2020 Sep 7. PMID: 32907706 No abstract available.
Post-Liver Transplantation Sinusoidal Obstruction Syndrome With Refractory Ascites Induced by Mycophenolate Mofetil.
Poli E, Kounis I, Guettier C, Verstuyft C, Coilly A, Sobesky R, Feray C, Vibert E, Ciacio O, Samuel D, Bismuth H, Duclos-Vallée JC.
Hepatology. 2020 Apr;71(4):1508-1510. doi: 10.1002/hep.30984. Epub 2020 Mar 31. PMID: 31597193 No abstract available.
Reply to "Letter to the Editor: Post-Liver Transplantation Sinusoidal Obstruction Syndrome and immunosuppressive drugs: causality of MMF or tacrolimus?".
Poli E, Verstuyft C, Guettier C, Duclos Vallée JC.
Hepatology. 2020 Nov 17. doi: 10.1002/hep.31635. Online ahead of print. PMID: 33200416
Ursodeoxycholic acid therapy throughout pregnancy in women affected with chronic cholestasis of childhood: No evidence for teratogenicity.
Panayotis Lykavieris, Olivier Bernard, Emmanuel Jacquemin
Clin Res Hepatol Gastroenterol. 2020 Jun 18;101472. doi: 10.1016/j.clinre.2020.05.020. Online ahead of print.PMID: 32565202

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