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The Approach to the Pediatric Patient

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Diagnosis and Management of Autoimmune Hepatitis

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Abstract

Pediatric autoimmune hepatitis is an inflammatory progressive liver disease characterized by inflammatory liver histology, elevated immunoglobulin G (IgG), and autoantibodies in the absence of a known etiology. Untreated, the condition often progresses rapidly to cirrhosis.

In children, autoimmune liver disorders can be divided into three different types: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo autoimmune hepatitis after liver transplant. Pediatric autoimmune liver disorders are diagnosed more frequently than in the past possibly due to increased awareness or possible increase in prevalence.

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References

  1. Mieli-Vergani G, Vergani D, Baumann U, et al. Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN hepatology committee position statement. J Pediatr Gastroenterol Nutr. 2018;66:345–60.

    Article  Google Scholar 

  2. Gregorio GV, Portmann B, Reid F, Donaldson PT, Doherty DG, McCartney M, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology. 1997;25(3):541–7.

    Article  CAS  Google Scholar 

  3. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical variation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med. 1990;322(26):1829–36.

    Article  CAS  Google Scholar 

  4. Wherman A, Waisbourd-Zinman O, Shah A, et al. Steroid free treatment of autoimmune hepatitis in selected children. J Pediatr. 2019;207:244–7.

    Article  Google Scholar 

  5. Manns MP, Czaja AJ, Gorham JD, et al. Diagnosis and management of autoimmune hepatitis. Hepatology. 2010;51:2193–213.

    Article  CAS  Google Scholar 

  6. Ferri PM, Ferreira AR, Miranda DM, et al. Diagnostic criteria for autoimmune hepatitis in children: a challenge for pediatric hepatologists. World J Gastroenterol. 2012;18:4470–3.

    Article  Google Scholar 

  7. Mileti E, Rosenthal P, Peters MG. Validation and modification of simplified diagnostic criteria for autoimmune hepatitis in children. Clin Gastroenterol Hepatol. 2012;10:417–421.e1–2.

    Article  Google Scholar 

  8. Gregorio GV, Portmann B, Karani J, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33:544–53.

    Article  CAS  Google Scholar 

  9. Kumari N, Kathuria R, Srivastav A, et al. Significance of histopathological features in differentiating autoimmune liver disease from nonautoimmune chronic liver disease in children. Eur J Gastroenterol Hepatol. 2013;25:333–7.

    Article  Google Scholar 

  10. Tucker SM, Jonas MM, Perez-Atayde AR. Hyaline droplets in Kupffer cells: a novel diagnostic clue for autoimmune hepatitis. Am J Surg Pathol. 2015;39:772–8.

    Article  Google Scholar 

  11. Tiniakos DG, Brain JG, Bury YA. Role of histopathology in autoimmune hepatitis. Dig Dis. 2015;33(suppl 2):53–64.

    Article  Google Scholar 

  12. Mieli-Vergani G, Vergani D. Budesonide for juvenile autoimmune hepatitis? Not yet. J Pediatr. 2013;163:1246–8.

    Article  Google Scholar 

  13. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology. 1997;25:541–7.

    Article  CAS  Google Scholar 

  14. Mieli-Vergani G, Heller S, Jara P, et al. Autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 2009;49:158–64.

    Article  CAS  Google Scholar 

  15. Chai PF, Lee WS, Brown RM, et al. Childhood autoimmune liver disease: indications and outcome of liver transplantation. J Pediatr Gastroenterol Nutr. 2010;50:295–302.

    Article  CAS  Google Scholar 

  16. Saadah OI, Smith AL, Hardikar W. Long-term outcome of autoimmune hepatitis in children. J Gastroenterol Hepatol. 2001;16:1297–302.

    Article  CAS  Google Scholar 

  17. Ferreira AR, Roquete ML, Toppa NH, et al. Effect of treatment of hepatic histopathology in children and adolescents with autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 2008;46:65–70.

    Article  Google Scholar 

  18. Samaroo B, Samyn M, Buchanan C, et al. Long-term daily oral treatment with prednisolone in children with autoimmune liver disease does not affect final adult height. Hepatology. 2006;44:438A.

    Google Scholar 

  19. Alvarez F. Autoimmune hepatitis and primary sclerosing cholangitis. Clin Liver Dis. 2006;10:89–107.

    Article  Google Scholar 

  20. Chang MH, Hadzic D, Rouassant SH, et al. Acute and chronic hepatitis: working group report of the second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2004;39(suppl 2):S584–8.

    Article  Google Scholar 

  21. Heneghan MA, Allan ML, Bornstein JD, et al. Utility of thiopurine methyltransferase genotyping and phenotyping, and measurement of azathioprine metabolites in the management of patients with autoimmune hepatitis. J Hepatol. 2006;45:584–91.

    Article  CAS  Google Scholar 

  22. Sheiko MA, Sundaram SS, Capocelli KE, et al. Outcomes in pediatric autoimmune hepatitis and significance of azathioprine metabolites. J Pediatr Gastroenterol Nutr. 2017;65:80–5.

    Article  Google Scholar 

  23. Franulovic OZ, Rajacic N, Lesar T, et al. Cyclosporine induced biochemical remission in childhood autoimmune hepatitis. Coll Antropol. 2012;36:973–9.

    PubMed  Google Scholar 

  24. Marlaka JR, Papadogiannakis N, Fischler B, et al. Tacrolimus without or with the addition of conventional immunosuppressive treatment in juvenile autoimmune hepatitis. Acta Paediatr. 2012;101:993–9.

    Article  CAS  Google Scholar 

  25. Aw MM, Dhawan A, Samyn M, et al. Mycophenolate mofetil as rescue treatment for autoimmune liver disease in children: a 5-year follow-up. J Hepatol. 2009;51:156–60.

    Article  CAS  Google Scholar 

  26. Kurowski J, Melin-Aldana H, Bass L, et al. Sirolimus as rescue therapy in pediatric autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 2014;58:e4–6.

    Article  Google Scholar 

  27. D’Agostino D, Costaguta A, Alvarez F. Successful treatment of refractory autoimmune hepatitis with rituximab. Pediatrics. 2013;132:e526–30.

    Article  Google Scholar 

  28. Weiler-Normann C, Schramm C, Quaas A, et al. Infliximab as a rescue treatment in difficult-to-treat autoimmune hepatitis. J Hepatol. 2013;58:529–34.

    Article  CAS  Google Scholar 

  29. Bjarnason I, Hayee B, Pavlidis P, et al. Contrasting pattern of chronic inflammatory bowel disease in primary and autoimmune sclerosing cholangitis. EBioMedicine. 2015;2:1523–7.

    Article  Google Scholar 

  30. Martin SR, Alvarez F, Anand R, et al. Outcomes in children who underwent transplantation for autoimmune hepatitis. Liver Transpl. 2011;17:393–401.

    Article  Google Scholar 

  31. Bahar RJ, Yanni GS, Martin MG, et al. Orthotopic liver transplantation for autoimmune hepatitis and cryptogenic chronic hepatitis in children. Transplantation. 2001;72:829–33.

    Article  CAS  Google Scholar 

  32. Edmunds C, Ekong UD. Autoimmune liver disease post-liver transplantation: a summary and proposed areas for future research. Transplantation. 2016;100:515–24.

    Article  CAS  Google Scholar 

  33. Kerkar N, Yanni G. ‘De novo’ and ‘recurrent’ autoimmune hepatitis after liver transplantation: a comprehensive review. J Autoimmun. 2016;66:17–24.

    Article  Google Scholar 

  34. Birnbaum AH, Benkov KJ, Pittman NS, et al. Recurrence of autoimmune hepatitis in children after liver transplantation. J Pediatr Gastroenterol Nutr. 1997;25:20–5.

    Article  CAS  Google Scholar 

  35. Banff Working Group, Demetris AJ, Adeyi O, et al. Liver biopsy interpretation for causes of late liver allograft dysfunction. Hepatology. 2006;44:489–501.

    Article  Google Scholar 

  36. Carbone M, Neuberger J. Liver transplantation in PBC and PSC: indications and disease recurrence. Clin Res Hepatol Gastroenterol. 2011;35:446–54.

    Article  Google Scholar 

  37. Kerkar N, Hadzic N, Davies ET, et al. De-novo autoimmune hepatitis after liver transplantation. Lancet. 1998;351:409–13.

    Article  CAS  Google Scholar 

  38. Venick RS, McDiarmid SV, Farmer DG, et al. Rejection and steroid dependence: unique risk factors in the development of pediatric posttransplant de novo autoimmune hepatitis. Am J Transplant. 2007;7:955–63.

    Article  CAS  Google Scholar 

  39. Salcedo M, Vaquero J, Banares R, et al. Response to steroids in de novo autoimmune hepatitis after liver transplantation. Hepatology. 2002;35:349–56.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Levine Children’s Hospital at Atrium Health, Charlotte, NC, USA

    Vani V. Gopalareddy

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  1. Vani V. Gopalareddy
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Correspondence to Vani V. Gopalareddy .

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Editors and Affiliations

  1. Carolinas Medical Center-Atrium Health, University of North Carolina School of Medicine, Charlotte, NC, USA

    Mark W. Russo

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Gopalareddy, V.V. (2020). The Approach to the Pediatric Patient. In: Russo, M. (eds) Diagnosis and Management of Autoimmune Hepatitis. Springer, Cham. https://doi.org/10.1007/978-3-030-33628-8_7

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  • DOI: https://doi.org/10.1007/978-3-030-33628-8_7

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-33627-1

  • Online ISBN: 978-3-030-33628-8

  • eBook Packages: MedicineMedicine (R0)

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