Autoimmune hepatitis in children in Eastern Denmark.
OBJECTIVE
Autoimmune hepatitis (AIH) in childhood is a progressive chronic inflammatory liver disease. The aim of this study was to compare the clinical and biochemical characteristics of 33 paediatric patients diagnosed as having AIH with earlier described cohorts, and to examine the effect of early treatment strategies on the course of disease.


METHODS
A population-based cohort of patients from January 1993 to September 2009 was identified prospectively, and the patient data were collected by a retrospective examination of the files.


RESULTS
Twenty-nine patients had type 1 AIH, 2 had type 2, and 2 could not be categorised. Among the 33 children, 16 (48.5%) were girls and 17 (51.5%) were boys. Twenty-three (69.7%) of the patients had symptoms at presentation indistinguishable from acute viral hepatitis, but in 16 (69.6%) of those the liver biopsy showed cirrhosis. Twenty (60.6%) patients were treated with prednisolone and azathioprine at the time of remission, whereas 8 (24.2%) were treated with prednisolone. One (3%) patient did not experience remission during the observation period.


CONCLUSIONS
The patients in our study appeared similar to previously published cohorts, although a female predominance was not observed. Our data suggest that early treatment including both prednisolone and azathioprine could be more effective than prednisolone alone, even if randomised controlled paediatric studies comparing these 2 different treatment regimens are needed.
Sarcoma Unit, Royal Marsden Hospital, London, UK
Joanna
J
Vitfell-Pedersen
Marianne Hørby
MH
Jørgensen
Klaus
K
Müller
Carsten
C
Heilmann
D000293:Adolescent; D001379:Azathioprine / Q000627:therapeutic use*; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003718:Denmark / Q000453:epidemiology; D004359:Drug Therapy, Combination; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D006525:Hepatitis, Viral, Human; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use*; D015995:Prevalence; D011446:Prospective Studies; D012737:Sex Factors
D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine