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Pediatrics. 2015 Nov;136(5):e1237-48. doi: 10.1542/peds.2015-0578. Epub 2015 Oct 19.

Incidence and Characteristics of Autoimmune Hepatitis.

Author information

1
University of Ottawa and Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada; cajimenez@cheo.on.ca.
2
University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada;
3
McGill University and Montreal Children's Hospital, Montreal, Quebec, Canada;
4
University of Alberta and Stollery Children's Hospital, Edmonton, Alberta, Canada;
5
University of Ottawa and Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada;
6
Memorial University of Newfoundland and Janeway Children's Hospital, St. John's, Newfoundland, Canada;
7
Dalhousie University and IWK Health Center, Halifax, Nova Scotia, Canada;
8
McMaster University and McMaster Children's Hospital, Hamilton, Ontario, Canada;
9
University of Saskatchewan and Children's Hospital of Saskatchewan, Saskatoon, Saskatchewan, Canada;
10
University of Western Ontario and London Health Science Center, London, Ontario, Canada;
11
University of Manitoba and The Children's Hospital of Winnipeg, Winnipeg, Manitoba, Canada;
12
University of British Columbia and BC Children's Hospital, Vancouver, British Columbia, Canada;
13
University of Calgary and Alberta Children's Hospital Calgary, Alberta, Canada; and.
14
University of Montreal and Centre Hospitalier Universitaire Sainte-Justine, Montreal, Quebec, Canada.

Abstract

BACKGROUND AND OBJECTIVES:

Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease of unknown etiology, with limited population-based estimates of pediatric incidence. We reported the incidence of pediatric AIH in Canada and described its clinical characteristics.

METHODS:

We conducted a retrospective cohort study of patients aged <18 years diagnosed with AIH between 2000-2009 at all pediatric centers in Canada.

RESULTS:

A total of 159 children with AIH (60.3% female, 13.2% type 2 AIH) were identified. Annual incidence was 0.23 per 100000 children. Median age at presentation for type 1 was 12 years (interquartile range: 11-14) versus 10 years for type 2 (interquartile range: 4.5-13) (P = .03). Fatigue (58%), jaundice (54%), and abdominal pain (49%) were the most common presenting symptoms. Serum albumin (33 vs 38 g/L; P = .03) and platelet count (187 000 vs 249 000; P <.001) were significantly lower and the international normalized ratio (1.4 vs 1.2; P <.001) was higher in cirrhotic versus noncirrhotic patients. Initial treatment included corticosteroids (80%), azathioprine (32%), and/or cyclosporine (13%). Response to treatment at 1 year was complete in 90%, and partial in 3%. 3% of patients had no response, and 3% responded and later relapsed. Nine patients underwent liver transplantation, and 4 patients died at a mean follow-up of 4 years.

CONCLUSIONS:

AIH is uncommon in children and adolescents in Canada. Type 1 AIH was diagnosed 5.5 times more frequently than type 2 AIH. Most patients respond well to conventional therapy, diminishing the need for liver transplantation.

PMID:
26482664
DOI:
10.1542/peds.2015-0578
[Indexed for MEDLINE]
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