Autoimmune liver diseases in children. Part II
Authors:
P. Dědek
Authors place of work:
Dětská klinika Fakultní nemocnice, Hradec Králové, přednosta prof. MUDr. M. Bayer, CSc.
Published in the journal:
Čes-slov Pediat 2011; 66 (1): 26-33.
Category:
Review
Summary
Autoimmune liver diseases form a group of chronic inflammatory illnesses associated with serious prognosis. Autoimmune hepatitis, autoimmune sclerosing cholangitis, and primary sclerosing cholangitis are the most common entities seen during childhood. Clinical findings of liver disorder are not typical, even the disease course may be asymptomatic. We can frequently found other illnesses with autoimmune pathogenesis in patients or their first-degree relatives. Laboratory diagnostics is based on detection of different types of autoantibodies, hypergammaglobulinemia, and elevated IgG. The definitive diagnosis is provided by liver biopsy and imaging of biliary tree.
Early and proper diagnosis followed by prompt therapy affects the patient’s prognosis significantly. Corticosteroids alone or paired with azathioprine have proven efficacy, administration of ursodeoxycholic acid is still a matter of debate. Chronic liver failure requiring liver transplant occurs despite treatment in many patients, especially those with sclerosing cholangitis.
Key words:
autoimmune liver disease, autoimmune hepatitis, primary sclerosing cholangitis, autoimmune sclerosing cholangitis, primary biliary cirrhosis, childhood, overlap syndrome
Zdroje
1. Kaplan GG, Laupland KB, Butzner D, et al. The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis. Am J Gastroenterol 2007; 102: 1042–1049.
2. Feldstein AE, Perrault J, El-Youssif, et al. Primary sclerosing cholangitis in children: a long-term follow-up study. Hepatology 2003; 38: 210–217.
3. Kerkar N, Miloh T. Sclerosing cholangitis: pediatric perspective. Curr Gastroenterol Rep 2010; 12: 195–202.
4. Mieli-Vergani G, Vergani D. Unique features of primary sclerosing cholangitis in children. Curr Opin Gastroenterol 2010; 26: 265–268.
5. Mieli-Vergani G, Vergani D. Sclerosing cholangitis in the paediatric patient. Best Pract Res Clin Gastroenterol 2001; 15: 681–690.
6. Mendes FD, Jorgensen R, Keach J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101: 2070–2005.
7. Miglani RK, Murthy D, Bhat R, et al. Immunoglobulin G4-associated cholangitis mimicking cholangiocarcinoma in a young boy. J Postgrad Med 2010; 56: 140–142.
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: 544–553.
9. Miloh T, Arnon R, Shneider B, et al. A retrospective single-center review of primary sclerosing cholangitis in children. Clin Gastroenterol Hepatol 2009; 7: 239–245.
10. Alvarez F. Autoimmune hepatitis and primary sclerosing cholangitis. Clin Liver Dis 2006; 10: 89–107.
11. Wilschanski M, Chait P, Wade JA, et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology 1995; 22: 1415–1422.
12. Vergani D, Alvarez F, Bianchi FB, et al. Liver autoimmune serology: a consensus statement from the committee for autoimmune serology of the International Autoimmune Hepatitis Group. J Hepatol 2004; 41: 677–683.
13. Tipnis NA, Dua KS, Werlin SL. A retrospective assessment of magnetic resonance cholangiopancreatography in children. J Pediatr Gastroenterol Nutr 2008; 46: 59–64.
14. Smolka V, Kolek A, Procházka V, et al. Primární sklerózující cholangitida u dětí a mladistvých. Čes-slov Pediat 2000; 9: 552–557.
15. Smolka V, Konečný M, Ehrmann J, et al. Společné autoimunní fenomény primární sklerozující cholangitidy a autoimunní hepatitidy v dětském věku. Čes slov Gastroent a Hepatol 2003; 57: 61–66.
16. Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology 2009; 50: 808–814.
17. Kitiyakara T, Chapman RW. Chemoprevention and screening in primary sclerosing cholangitis. Postgrad Med J 2008; 84: 228–237.
18. Davies YK, Cox KM, Abdullah BA, et al. Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic. J Pediatr Gastroenterol Nutr 2008; 47: 61–67.
19. Vergani D, Mieli-Vergani G. Autoimmune hepatitis and PSC connection. Clin Liver Dis 2008; 12: 187–202.
20. Invernizzi P, Crosignani A, Battezzati PM, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody -positive and -negative primary biliary cirrhosis. Hepatology 1997; 25: 1090–1095.
21. Nožička Z. Patologicko-anatomický příspěvek k protilátkové diagnostice imunopatologických onemocnění. Suppl. Sbor věd Prací LF UK Hradec Králové 1991; 33(1).
22. Chazouilleres O, Wendum D, Serfaty L, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998; 28: 296–301.
23. Lohse AW, Meyer zum Büschenfelde K-H, Franz B, et al. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. Hepatology 1999; 29: 1078–1084.
24. Czaja AJ, Carpenter HA, Santrach PJ, et al. Autoimmune cholangitis within the spectrum of autoimmune liver disease. Hepatology 2000; 31: 1231–1238.
25. Rust C, Beuers U. Overlap syndromes among autoimmune liver diseases. World J Gastroenterol 2008; 14: 3368–3373.
26. Horák J, Vaňásek T. Autoimunitní postižení jater. In: Ehrmann J, Hůlek P. Hepatologie. 1. vyd. Praha: Grada, 2010.
27. McFarlane IG. The relationship between autoimmune markers and different clinical syndromes in autoimmune hepatitis. Gut 1998; 4: 599–602.
Štítky
Neonatology Paediatrics Pneumology and ftiseology General practitioner for children and adolescents General practitioner for adultsČlánok vyšiel v časopise
Czech-Slovak Pediatrics
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