• Features of the autoimmune hepatitis manifestation in children

Features of the autoimmune hepatitis manifestation in children

SOVREMENNAYA PEDIATRIYA.2017.6(86):80-86; doi 10.15574/SP.2017.86.80

Berezenko V. S., Mykhayluk K. Z.
SI «Institute of Pediatrics, Obstetrics and Gynecology of NAMS of Ukraine», Kyiv

Objective. To study clinical and morphological features of autoimmune hepatitis manifestation in children.
Materials and methods. The studying included anamnesis of disease and life, hereditary anamnesis, clinical presentation, estimated stage of fibrosis (measured on the scale METAVIR) and histological index of activity (Knodell), which based on the results of morphological study of liver biopsy, cytolysis markers (alanine aminotransferase, aspartate aminotransferase, gamma-glutamyltransferase), cholestasis (common bilirubin, alkaline phosphatase), mesenchymal-inflammatory syndrome (total protein, thymol test, ESR, γ-globulin, IgG, CIC), violation of protein-synthetic liver function (albumin, prothrombin index) hypocoagulation syndrome (INR, APTT) in 26 children with debut of autoimmune hepatitis before the prescription of immunosuppressive treatment.
Results: Autoimmune hepatitis in children is characterized by a nonspecific clinical picture, an aggressive and high-level inflammatory process in the liver, with a rapid progression of the disease (the stage of fibrosis >F2). The diagnosis was established at the stage of liver cirrhosis in 50% of the examined children. The duration of the disease and high activity of process are predictors of the progression of liver fibrosis.
Conclusions. The clinical picture of debut of autoimmune hepatitis determined by the severity of functional state violation of the liver and the activity of process in children. The manifestation of autoimmune hepatitis most often occurs in girls of pre-pubertal age. The presence of autoimmune diseases in the family in conditions of increased functional liver tests requires a detailed examination of these children for the purpose of early diagnosis of autoimmune hepatitis.
Key words: autoimmune hepatitis, manifestation, children, fibrosis, clinic, diagnostics.


1. Babak OIa, Kolesnykova EV. (2011). Tsyroz pechinky. Kyiv: 363—373.

2. Berezenko VS. (2007). Kliniko-patohenetychni osoblyvosti fibrohenezu pechinky pry khronichnykh hepatytakh u ditei ta shliakhy yoho medykamentoznoi korektsii. Dys. … d-ra med. nauk. Kyiv.

3. Czaja AJ. (2016). Diagnosis and Management of Autoimmune Hepatitis: Current Status and Future Directions. Gutand Liver. 10; 2: 177—203. https://doi.org/10.5009/gnl15352; PMid:26934884 PMCid:PMC4780448

4. Ansgar W Lohse, Giorgina Mieli-Vergani (2011). Autoimmune hepatitis. Journal of Hepatology. 55: 171—182. https://doi.org/10.1016/j.jhep.2010.12.012; PMid:21167232

5. Priscila Menezes Ferri Liu, Debora Marques de Miranda et al. (2013). Autoimmune hepatitis in childhood: The role of genetic and immune factors. World J Gastroenterol. 19(28): 4455—4463. https://doi.org/10.3748/wjg.v19.i28.4455; PMid:23901220 PMCid:PMC3725369

6. Dancygier H. (2010). Clinical Hepatology: Principles and Practice of Hepatobiliary Diseases. 1. https://doi.org/10.1007/978-3-540-93842-2; https://doi.org/10.1007/978-3-642-04519-6

7. Czaja AJ. (2006). Autoimmune liver disease. Curr Opin Gastroenterol. 22(3): 234—40. https://doi.org/10.1097/01.mog.0000218959.48064.7f; PMid:16550037

8. Manns MP, Czaja AJ et al. (2010). Diagnosis and Management of Autoimmune Hepatitis. Hepatology. 51; 6: 1—21. https://doi.org/10.1002/hep.23584; PMid:20513004

9. Priscila Menezes Ferri, Alexandre Rodrigues Ferreira et al. (2012). Diagnostic criteria for autoimmune hepatitis in children: A challenge for pediatric hepatologists. World J Gastroenterol. 18(33): 4470—4473. https://doi.org/10.3748/wjg.v18.i33.4470; PMid:22969217 PMCid:PMC3435769

10. Liberal R, Grant CR, Longhi MS et al. (2014). Diagnostic criteria of autoimmune hepatitis. Autoimmun Rev. https://doi.org/10.1016/j.autrev.2013.11.009

11. EASL Clinical Practice Guidelines: Autoimmune hepatitis European Association for the Study of the Liver. (2015). Journal of Hepatology. 63: 971—1004. https://doi.org/10.1016/j.jhep.2015.06.030; PMid:26341719

12. Czaja AJ. (2014). Hepatic inflammation and progressive liver fibrosis in chronic liver disease WJG 20th Anniversary Special Issues (11): Cirrhosis. World J Gastroenterol. 20(10): 2515—2532. https://doi.org/10.3748/wjg.v20.i10.2515; PMid:24627588 PMCid:PMC3949261

13. Krawitt EL. (2006). Autoimmune hepatitis. Engl J Med. 354(1): 54—66. https://doi.org/10.1056/NEJMra050408; PMid:16394302

14. Gerosa C, Fanos V, et al. (2013). Liver biopsy interpretation in the differential diagnosis of autoimmune liver disease in children. Journal of Pediatric and Neonatal Individualized Medicine. 2(2).

15. Manns MP, Taubert R. (2014). Treatment of Autoimmune Hepatitis. Clinical Liver Disease. 3; 1. https://doi.org/10.1002/cld.306

16. Mieli-Vergani G, Vergani D. (2014). Autoimmune Hepatitis in Childhood. Clinical Liver Disease. 3; 1. https://doi.org/10.1007/978-1-4614-9005-0_16

17. Mileti E, Rosenthal P, Peters MG. (2012). Validation and Modification of Simplifed Diagnostic Criteria for Autoimmune Hepatitis in Children. Clinical Gastroenterology and Hepatology. 1: 417—421. https://doi.org/10.1016/j.cgh.2011.11.030; PMid:22179022 PMCid:PMC3321372

18. Liberal R, Longhi MS, Mieli-Vergani G et al. (2011). Pathogenesis of autoimmune hepatitis. Best Pract Res Clin Gastroenterol. 25: 653—664. https://doi.org/10.1016/j.bpg.2011.09.009; PMid:22117632

19. Liberal R, Vergani D, Mieli-Vergani G. (2015). Update on Autoimmune Hepatitis. Journal of Clinical and Translational Hepatology. 3: 42—52. https://doi.org/10.14218/JCTH.2014.00032; PMid:26357634 PMCid:PMC4542083

20. Hennes EM, Zeniya M, Czaja AJ et al. (2008). Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 48: 169—176. https://doi.org/10.1002/hep.22322; PMid:18537184

21. Vergani D, Mieli-Vergani G. (2014). Autoimmune Hepatitis: Diagnostic Criteria and Serological Testing. Clinical Liver Disease. 3; 2: 38—41. https://doi.org/10.1002/cld.321