Incidence and Characteristics of XXXX .

@entity1 @entity1087 @entity500 @entity409 @entity205 @entity490 @entity775
BACKGROUND AND OBJECTIVES: @entity775 ( @entity775 ) is a progressive @entity409 of unknown etiology, with limited population-based estimates of pediatric incidence. We reported the incidence of pediatric @entity775 in Canada and described its clinical characteristics. METHODS: We conducted a retrospective cohort study of @entity1 aged <18 years diagnosed with @entity775 between 2000-2009 at all pediatric centers in Canada. RESULTS: A total of 159 @entity1 with @entity775 (60.3% female, 13.2% type 2 @entity775 ) were identified. Annual incidence was 0.23 per 100000 @entity1 . 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%), @entity1087 (54%), and @entity490 (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 @entity1 . Initial treatment included corticosteroids (80%), @entity500 (32%), and/or @entity205 (13%). Response to treatment at 1 year was complete in 90%, and partial in 3%. 3% of @entity1 had no response, and 3% responded and later relapsed. Nine @entity1 underwent liver transplantation, and 4 @entity1 died at a mean follow-up of 4 years. CONCLUSIONS: @entity775 is uncommon in @entity1 and adolescents in Canada. Type 1 @entity775 was diagnosed 5.5 times more frequently than type 2 @entity775 . Most @entity1 respond well to conventional therapy, diminishing the need for liver transplantation.
