Journal ArticleDOI
Liver Transplantation for Children With Primary Sclerosing Cholangitis and Autoimmune Hepatitis: UNOS Database Analysis
TLDR
Children with AIH transplanted as status 1 had significantly lower patient survival rates but similar graft survival rates to children with chronic AIH, and children transplanted for AIH versus PSC showed no significant differences in patient or graft survival at both 1 and 5 years.Abstract:
Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the pediatric end-stage liver disease era. We aimed to characterize the outcome of LT in children with AIH and PSC.
Children 18 years or younger with PSC or AIH who had a first, isolated LT from 2002 to 2012 were identified from the United Network for Organ Sharing database. Graft and patient outcomes were studied.
A total of 174 children with AIH and 113 with PSC were transplanted in the study period. One-year patient survival was 95.4% for AIH and 97.3% for PSC. Five-year patient survival was 91.4% for AIH and 92.9% for PSC. Patient survival was not significantly different between the 2 groups. Forty-four (25.2%) children with AIH were listed as status 1 for transplant (fulminant hepatic failure at presentation or acute-on-chronic disease). Patients transplanted as status 1 had significantly lower patient survival compared with patients transplanted with AIH and end-stage liver disease. The one- and five-year graft survival rates were not significantly different between patients with AIH and PSC.
Children with AIH transplanted as status 1 had significantly lower patient survival rates but similar graft survival rates to children with chronic AIH. Children transplanted for AIH versus PSC showed no significant differences in patient or graft survival at both 1 and 5 years.read more
Citations
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Journal ArticleDOI
Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases.
Cara L. Mack,David H. Adams,David N. Assis,Nanda Kerkar,Michael P. Manns,M.J. Mayo,John M. Vierling,Mouaz Alsawas,Mohammad Hassan Murad,Albert J. Czaja +9 more
TL;DR: This practice guideline/guidance constitutes an update of the guidelines on AIH published in 2010 by the American Association for the Study of Liver Diseases (AASLD) and updates the epidemiology, diagnosis, management, and outcomes of AIH in adults and children.
Journal ArticleDOI
Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement.
Giorgina Mieli-Vergani,Diego Vergani,Ulrich Baumann,Piotr Czubkowski,Dominique Debray,Antal Dezsofi,Björn Fischler,Girish Gupte,Loreto Hierro,Giuseppe Indolfi,Jörg Jahnel,Françoise Smets,Henkjan J. Verkade,Nedim Hadžić +13 more
TL;DR: Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology.
Journal ArticleDOI
Juvenile autoimmune hepatitis: A comprehensive review
Christiane Sokollik,Valérie A. McLin,Diego Vergani,Benedetta Terziroli Beretta-Piccoli,Giorgina Mieli-Vergani +4 more
TL;DR: Immunosuppressive therapy, based on steroids and azathioprine, is required, and in the vast majority of patients leads to clinical and biochemical remission, defined as absence of symptoms, normal transaminase and IgG levels, and negative or low-titer autoantibodies.
Journal ArticleDOI
Treatment of primary sclerosing cholangitis in children.
TL;DR: The evidence for drug efficacy, dosing, duration of therapy, and treatment targets in PSC is reviewed, and a framework for endoscopic and medical management of this complex problem is provided.
Journal ArticleDOI
Immunosuppressive Treatment Regimens in Autoimmune Hepatitis: Systematic Reviews and Meta-Analyses Supporting American Association for the Study of Liver Diseases Guidelines.
John M. Vierling,Nanda Kerkar,Albert J. Czaja,Cara L. Mack,David H. Adams,David N. Assis,Michael P. Manns,Marlyn J. Mayo,Tarek Nayfeh,Abdul M. Majzoub,Muayad A Alzuabi,Jingyi Ding,Samir Haffar,M. Hassan Murad,Mouaz Alsawas +14 more
TL;DR: First-line treatment with budesonide and azathioprine normalizes serum aminotransferase levels more often than prednisone and azthioprine after six months in non-cirrhotic adults, and its effectiveness in treating other presentations or reducing either mortality or need for liver transplantation remains unproven.
References
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Diagnosis and management of autoimmune hepatitis.
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Journal ArticleDOI
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G V Gregorio,Bernard Portmann,John Karani,P.M. Harrison,Peter T. Donaldson,Diego Vergani,Giorgina Mieli-Vergani +6 more
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Journal ArticleDOI
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Journal ArticleDOI
A re‐evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts
Edward B. Alabraba,Edward B. Alabraba,Peter Nightingale,Bridget Gunson,Bridget Gunson,Stefan G. Hubscher,Stefan G. Hubscher,Simon Olliff,Darius F. Mirza,Darius F. Mirza,James Neuberger,James Neuberger +11 more
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