Emmanuel Gonzales

Emmanuel Gonzales

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Emmanuel Gonzales

Publications by authors named "Emmanuel Gonzales"

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mTOR inhibitors in pediatric liver transplant recipients.

Clin Res Hepatol Gastroenterol 2019 Aug 6;43(4):403-409. Epub 2018 Dec 6.

Swiss center for liver disease in children, university hospitals Geneva, 1205 Geneva, Switzerland.

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http://dx.doi.org/10.1016/j.clinre.2018.11.010DOI Listing
August 2019

Congenital Portosystemic Shunts: Current Diagnosis and Management.

J Pediatr Gastroenterol Nutr 2019 May;68(5):615-622

Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference Centre for Pediatric Liver Diseases and Folfoie-DHU Hepatinov, Assistance Publique-Hôpitaux de Paris and INSERM UMR-S 1174, Université Paris-Sud, Paris-Saclay, Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1097/MPG.0000000000002263DOI Listing
May 2019

Cholic acid for primary bile acid synthesis defects: a life-saving therapy allowing a favorable outcome in adulthood.

Orphanet J Rare Dis 2018 10 29;13(1):190. Epub 2018 Oct 29.

Pediatric Hepatology and Pediatric Liver Transplantation Unit, National Reference Centre for rare pediatric liver diseases and Filfoie, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Paris, France.

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0
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http://dx.doi.org/10.1186/s13023-018-0920-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206929PMC
October 2018

Congenital portosystemic shunts: diagnosis and treatment.

Abdom Radiol (NY) 2018 08;43(8):2023-2036

Pediatric Surgery Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

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http://link.springer.com/10.1007/s00261-018-1619-8
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http://dx.doi.org/10.1007/s00261-018-1619-8DOI Listing
August 2018

First liver transplantation for biliary atresia in children: The hidden effects of non-centralization.

Pediatr Transplant 2018 Jun 4:e13232. Epub 2018 Jun 4.

Department of Pediatric Surgery, Hôpitaux Universitaires Paris-Sud, Bicêtre Hospital, AP-HP, Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1111/petr.13232DOI Listing
June 2018

Diagnosis, treatment and outcome of hepatic venous outflow obstruction in paediatric liver transplantation: 24-year experience at a single centre.

Pediatr Radiol 2018 05 21;48(5):667-679. Epub 2018 Feb 21.

AP-HP, Bicêtre Hospital, Pediatric Radiology Department, 78 rue du Gal Leclerc, 94270, Le Kremlin Bicêtre, France.

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http://dx.doi.org/10.1007/s00247-018-4079-yDOI Listing
May 2018

Cholic Acid to Treat HSD3B7 and AKR1D1 Deficiencies.

J Pediatr Gastroenterol Nutr 2017 12;65(6):e134

Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference Centre for Pediatric Liver Diseases-DHU Hépatinov, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre.

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http://dx.doi.org/10.1097/MPG.0000000000001693DOI Listing
December 2017

Hepatic Portocholecystostomy: 97 Cases From a Single Institution.

J Pediatr Gastroenterol Nutr 2017 10;65(4):375-379

*Department of Pediatric Surgery, Bicetre's Hospital †Department of Pediatric Hepatology, Bicetre's Hospital, Le Kremlin-Bicètre, France.

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http://dx.doi.org/10.1097/MPG.0000000000001685DOI Listing
October 2017

Attempt to Determine the Prevalence of Two Inborn Errors of Primary Bile Acid Synthesis: Results of a European Survey.

J Pediatr Gastroenterol Nutr 2017 06;64(6):864-868

*Department of Paediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria †Department of Paediatrics, CLINTEC, Karolinska University Hospital, Stockholm, Sweden ‡Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy §Pediatric Hepatology Unit, Hôpital Necker-Enfants-Malades, Paris, France ||First Department of Paediatrics, Semmelweis University, Budapest, Hungary ¶Division of Neuropediatrics and Metabolic Medicine, University Children's Hospital Heidelberg, Heidelberg, Germany #Paediatric Centre for Hepatology, Gastroenterology and Nutrition, King's College Hospital, London, UK **Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference Centre for Pediatric Liver Diseases-DHU Hepatinov, Assistance Publique-Hôpitaux de Paris and INSERM UMR-S 1174, Université Paris-Sud 11, Le Kremlin-Bicêtre ††Pediatric Hepatology and Gastroenterology Unit, University Children's Hospital, Bordeaux, France ‡‡Department of Medical Sciences, Paediatric Section, University Hospital Arcispedale Sant'Anna, University of Ferrara, Ferrara, Italy §§Division of Gastroenterology/Hepatology/Nutrition, Children's Hospital of Pittsburgh of UPMC, University of Pittsburgh, Pittsburgh, PA ||||Department of Pediatrics, Pediatric Gastroenterology Unit, Azienda Ospedaliera-Universitaria Citta[Combining Grave Accent] della Salute e della Scienza di Torino, University of Torino, Torino, Italy ¶¶Department of Paediatrics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands ##Paediatric Hepatology Service, Hospital Infantil Universitario "La Paz," Madrid, Spain ***Paediatric Gastroenterology Unit, Department of Pediatrics, University Hospitals Geneva, Geneva, Switzerland †††Division of Paediatric Gastroenterology and Hepatology, Department of Paediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany.

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http://dx.doi.org/10.1097/MPG.0000000000001546DOI Listing
June 2017

Sertraline as an Additional Treatment for Cholestatic Pruritus in Children.

J Pediatr Gastroenterol Nutr 2017 03;64(3):431-435

*Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference Centre for Pediatric Liver Diseases-DHU Hepatinov, Assistance Publique-Hôpitaux de Paris, Université Paris Sud, Le Kremlin-Bicêtre †INSERM UMR-S 1174, University Paris-Sud, Orsay ‡Pediatric Gastroenterology and Hepatology Department, CHU-Lille, Univ Lille, Lille §Pediatric Gastroenterology, Hepatology and Nutrition Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron ||Pediatric Hepatology Unit, Hôpital Necker-Enfants-Malades, Paris, France.

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http://dx.doi.org/10.1097/MPG.0000000000001385DOI Listing
March 2017

MYO5B mutations cause cholestasis with normal serum gamma-glutamyl transferase activity in children without microvillous inclusion disease.

Hepatology 2017 01 5;65(1):164-173. Epub 2016 Oct 5.

Pediatric Hepatology and Pediatric Liver Transplantation Unit and National Reference Centre for Rare Pediatric Liver Diseases, Bicêtre University Hospital, University of Paris-Sud, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, France.

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http://dx.doi.org/10.1002/hep.28779DOI Listing
January 2017

Long-term successful liver-kidney transplantation in a child with atypical hemolytic uremic syndrome caused by homozygous factor H deficiency.

Pediatr Nephrol 2016 12 15;31(12):2375-2378. Epub 2016 Oct 15.

Pediatric Nephrology, AP-HP Hôpital Robert-Debré, 48 Bd Sérurier, 75019, Paris, France.

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http://dx.doi.org/10.1007/s00467-016-3511-5DOI Listing
December 2016

Serum Autotaxin Activity Correlates With Pruritus in Pediatric Cholestatic Disorders.

J Pediatr Gastroenterol Nutr 2016 Apr;62(4):530-5

*Department of Medicine I, Friedrich-Alexander-University of Erlangen, Erlangen, Germany †Tytgat Institute for Liver and Intestinal Research and Department of Hepatology & Gastroenterology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands ‡Pediatric Hepatology Unit and National Reference Centre for Biliary Atresia, DHU Hepatinov, CHU Bicêtre, Assistance Publique-Hôpitaux de Paris, University Paris-Sud, Paris, and Inserm UMR-S1174, Orsay, France §Department of Surgery, University of Maastricht, Maastricht, The Netherlands.

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http://dx.doi.org/10.1097/MPG.0000000000001044DOI Listing
April 2016

Feasibility and Diagnostic Accuracy of Supersonic Shear-Wave Elastography for the Assessment of Liver Stiffness and Liver Fibrosis in Children: A Pilot Study of 96 Patients.

Radiology 2016 Feb 21;278(2):554-62. Epub 2015 Aug 21.

From the Departments of Pediatric Radiology (S.F.A., L.C., D.P.), Pediatric Hepatology (E.G.), and Epidemiology (G.A., B.D.), Hôpital Bicêtre, Hôpitaux Universitaires Paris Sud, Assistance Publique Hôpitaux de Paris, Université Paris Sud, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France; Departments of Pathology (M.F.) and Adult Radiology (J.M.C.), Hôpital Necker, Assistance Publique Hôpitaux de Paris, Université Paris V, Assistance Publique Hôpitaux de Paris, Paris, France; and Institut Langevin, ESPCI ParisTech, CNRS UMR7587, INSERM U979, Paris, France (J.L.G., M.T.).

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http://dx.doi.org/10.1148/radiol.2015142815DOI Listing
February 2016

Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate.

Hepatology 2015 Aug 8;62(2):558-66. Epub 2015 Apr 8.

Pediatric hepatology and pediatric liver transplantation unit and National Reference Centre for rare pediatric liver diseases, Bicêtre Universitary Hospital, Faculty of Medicine Paris-Sud, University of Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Le Kremlin Bicêtre, Paris, France.

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http://dx.doi.org/10.1002/hep.27767DOI Listing
August 2015

Liver transcript analysis reveals aberrant splicing due to silent and intronic variations in the ABCB11 gene.

Mol Genet Metab 2014 Nov 15;113(3):225-9. Epub 2014 Jul 15.

Pediatric Hepatology and Liver Transplantation Unit, Hôpital Bicêtre, Reference centre for pediatric liver diseases - DHU Hepatinov, Assistance Publique - Hôpitaux de Paris, Université Paris Sud 11, Le Kremlin-Bicêtre, France; INSERM U757, University Paris-Sud 11, Orsay, France. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S10967192140021
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http://dx.doi.org/10.1016/j.ymgme.2014.07.006DOI Listing
November 2014

Clinical utility gene card for: progressive familial intrahepatic cholestasis type 2.

Eur J Hum Genet 2014 Apr 28;22(4). Epub 2013 Aug 28.

1] INSERM, UMR-S757, University of Paris-Sud 11, Orsay, France [2] Pediatric Hepatology and Liver transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases, Bicêtre Universitary Hospital, Faculty of Medicine Paris-Sud, University of Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1038/ejhg.2013.187DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3953913PMC
April 2014

Clinical utility gene card for: progressive familial intrahepatic cholestasis type 1.

Eur J Hum Genet 2014 Apr 28;22(4). Epub 2013 Aug 28.

1] INSERM, UMR-S757, University of Paris-Sud 11, Orsay, France [2] Pediatric Hepatology and Liver transplantation Unit and National Reference Centre for Rare Pediatric Liver Diseases, Faculty of Medicine Paris-Sud, Bicêtre Universitary Hospital, University of Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1038/ejhg.2013.186DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3953912PMC
April 2014

Clinical utility gene card for: progressive familial intrahepatic cholestasis type 3.

Eur J Hum Genet 2014 Apr 4;22(4). Epub 2013 Sep 4.

1] INSERM, UMR-S757, University of Paris-Sud 11, Orsay, France [2] Pediatric Hepatology and Liver transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases, Bicêtre Universitary Hospital, Faculty of Medicine Paris-Sud, University of Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1038/ejhg.2013.188DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3953914PMC
April 2014

Arthrogryposis, renal dysfunction, and cholestasis syndrome caused by VIPAR mutation.

J Pediatr Gastroenterol Nutr 2014 Mar;58(3):e29-32

*Pediatric Hepatology and Pediatric Liver Transplantation Unit, National Reference Centre for Biliary Atresia †Pathology Unit, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris Sud 11, Le Kremlin-Bicêtre, France ‡Laboratory for Molecular Cell Biology, Great Ormond Street Hospital, London, UK.

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http://dx.doi.org/10.1097/MPG.0b013e318298108fDOI Listing
March 2014

Mitochondrial Infantile Liver Disease due to TRMU Gene Mutations: Three New Cases.

JIMD Rep 2013 27;11:117-23. Epub 2013 Apr 27.

Laboratoire de biochimie, AP-HP, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Sud, 78 rue du Général Leclerc, Le Kremlin-Bicêtre Cedex, 94275, France.

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http://dx.doi.org/10.1007/8904_2013_230DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3755544PMC
August 2013

NR1H4 analysis in patients with progressive familial intrahepatic cholestasis, drug-induced cholestasis or intrahepatic cholestasis of pregnancy unrelated to ATP8B1, ABCB11 and ABCB4 mutations.

Clin Res Hepatol Gastroenterol 2012 Dec 9;36(6):569-73. Epub 2012 Nov 9.

Biochemistry Laboratory, Bicêtre Hospital, University of Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Paris, France.

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http://dx.doi.org/10.1016/j.clinre.2012.08.008DOI Listing
December 2012

Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2.

J Hepatol 2012 Sep 16;57(3):695-8. Epub 2012 May 16.

Pediatric Hepatology and National Reference Centre for Rare Pediatric Liver Diseases, Bicêtre Universitary Hospital, Faculty of Medicine Paris-Sud, University of Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1016/j.jhep.2012.04.017DOI Listing
September 2012

Role of purinergic P2X receptors in the control of liver homeostasis.

Wiley Interdiscip Rev Membr Transp Signal 2012 May 11;1(3):341-348. Epub 2012 Jan 11.

Division of Gastroenterology & Hepatology, Department, of Internal Medicine, College of Medicine, University of Arkansas for, Medical Sciences, Little Rock, AR, USA.

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http://dx.doi.org/10.1002/wmts.32DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3364537PMC
May 2012

Claudin-1 involved in neonatal ichthyosis sclerosing cholangitis syndrome regulates hepatic paracellular permeability.

Hepatology 2012 Apr 1;55(4):1249-59. Epub 2012 Mar 1.

INSERM UMR-S757, Orsay, University Paris-Sud 11, Paris, France.

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http://dx.doi.org/10.1002/hep.24761DOI Listing
April 2012

Primary bile acid therapy during pregnancy in patients with 3β-hydroxy-Δ5 -C27 -steroid oxidoreductase deficiency.

Pediatr Int 2011 Oct;53(5):792

Pediatric Hepatology Unit and National Reference Centre for Biliary Atresia, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris; Faculty of Medicine Paris-Sud, University Paris-Sud 11, Paris, INSERM U757, University Paris-Sud 11, Orsay, France.

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http://doi.wiley.com/10.1111/j.1442-200X.2011.03416.x
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http://dx.doi.org/10.1111/j.1442-200X.2011.03416.xDOI Listing
October 2011

Delayed postnatal presentation of biliary atresia in 2 premature neonates.

J Pediatr Gastroenterol Nutr 2011 Apr;52(4):489-91

Pediatric Hepatology Unit, National Reference Centre for Biliary Atresia, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris Sud 11, Le Kremlin Bicêtre, France.

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http://dx.doi.org/10.1097/MPG.0b013e3182034cecDOI Listing
April 2011

Relapsing features of bile salt export pump deficiency after liver transplantation in two patients with progressive familial intrahepatic cholestasis type 2.

J Hepatol 2010 Nov 29;53(5):981-6. Epub 2010 Jul 29.

Department of Pediatrics, Division of Gastroenterology and Hepatology, University Hospital Santa Chiara, Pisa, Italy.

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http://dx.doi.org/10.1016/j.jhep.2010.05.025DOI Listing
November 2010

Mutation specific drug therapy for progressive familial or benign recurrent intrahepatic cholestasis: a new tool in a near future?

J Hepatol 2010 Aug 29;53(2):385-7. Epub 2010 Apr 29.

Pediatric Hepatology and National Reference Centre for Biliary Atresia, Bicêtre Hospital, Assistance Publique - Hôpitaux de Paris, University Paris Sud 11, Paris, France.

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http://dx.doi.org/10.1016/j.jhep.2010.03.012DOI Listing
August 2010

The spectrum of liver diseases related to ABCB4 gene mutations: pathophysiology and clinical aspects.

Semin Liver Dis 2010 May 26;30(2):134-46. Epub 2010 Apr 26.

Biochemistry, University of Paris - Sud 11, Assistance Publique - Hôpitaux de Paris, Le Kremlin Bicêtre, France.

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https://www.thieme-connect.com/products/ejournals/pdf/10.105
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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0030-1253223
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http://dx.doi.org/10.1055/s-0030-1253223DOI Listing
May 2010

Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long-term therapy.

Gastroenterology 2009 Oct 19;137(4):1310-1320.e1-3. Epub 2009 Jul 19.

Pediatric Hepatology Unit, Hôpital Bicêtre, Assistance Publique - Hôpitaux de Paris, Paris, France.

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http://dx.doi.org/10.1053/j.gastro.2009.07.043DOI Listing
October 2009

Progressive familial intrahepatic cholestasis.

Orphanet J Rare Dis 2009 Jan 8;4. Epub 2009 Jan 8.

Biochemistry, Bicêtre Hospital, University of Paris-sud XI, Assistance Publique-Hôpitaux de Paris, Paris, France.

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http://gut.bmj.com/content/42/6/766.full.pdf
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http://link.springer.com/content/pdf/10.1186/1750-1172-4-1.p
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https://childrennetwork.org/download.aspx?f=pfic.pdf
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http://www.ojrd.com/content/4/1/1
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http://dx.doi.org/10.1186/1750-1172-4-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2647530PMC
January 2009

Liver diseases related to MDR3 (ABCB4) gene deficiency.

Front Biosci (Landmark Ed) 2009 Jan 1;14:4242-56. Epub 2009 Jan 1.

Pediatric Hepatology and National Reference Centre for Biliary Atresia, Bicêtre Hospital, University of Paris - South 11, AP-HP, Paris, France.

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http://dx.doi.org/10.2741/3526DOI Listing
January 2009

Rat hepatocytes express functional P2X receptors.

FEBS Lett 2007 Jul 19;581(17):3260-6. Epub 2007 Jun 19.

INSERM, Université Paris-Sud, UMR-S757, Bâtiment 443, 15 rue Georges Clémenceau, Orsay Cedex, France.

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http://dx.doi.org/10.1016/j.febslet.2007.06.016DOI Listing
July 2007