Presentation and Outcomes of Autoimmune Hepatitis Type 1 and Type 2 in Children: A Single-center Study.

OBJECTIVE: Autoimmune hepatitis (AIH) is designated as type 1 or 2 (AIH-1/2) on the basis of serum autoantibody (Ab) profiles. In children, AIH may present as acute or chronic liver failure or cirrhotic AIH (ALF/CLF/CAIH) with or without overlap sclerosing cholangitis (SC). The aim of this study was to compare demographics, presentation, and outcomes between groups in children. METHODS: A retrospective electronic chart review of children with AIH who met standard diagnostic criteria with histologic confirmation at Texas Children's Hospital was performed, with de novo AIH after liver transplant (LT) excluded. Patients were identified and divided into AIH-1, AIH-2, ALF, CAIH, AIH-SC, and LT and compared using chi-square analysis, Student t-test, and Mood median test. RESULTS: Among 91 children with AIH, 72 (79.1%) had AIH-1, 19 (20.9%) had AIH-2, 13 (14.3%) had ALF, 25 (27.5%) had CAIH, and 14 (15.4%) had AIH-SC. Both AIH-1/2 had female and Hispanic predominance (72.2/89.5%, 40.3/57.9%). AIH-2 presented at younger mean age in years than AIH-1 (6.8, 12.1, P < 0.05). Both AIH-1/2 had low rates of remission after 1 year of IS (25.4, 35.7%) and most recent (30.6, 54.5%) follow-up. Twenty-two (24.2) patients received LT: 16 had AIH-1 (72.7%), 6 had AIH-2 (27.3%), 9 (40.9%) had ALF, and 13 (59.1%) had CAIH. One-year patient and graft survivals were 100%. CONCLUSIONS: The epidemiology and clinical presentation of AIH-1 and -2 had a few subtle differences. AIH-1 was associated with more complications after LT. More data are needed to better characterize the 2 as separate disease entities.

Does the stabilization of the calcaneocuboid joint with Steinmann pin in Evans osteotomy affect its incidence of arthritis?

PURPOSE: This study aims to determine the effect of retaining a Steinmann pin in the calcaneocuboid joint on its rates of arthritis following Evans calcaneal osteotomy lengthening procedure. The study hypothesized that leaving the pin across the calcaneocuboid joint post-operatively would lead to higher rates of arthritis. METHODS: This is a retrospective review of patients under 18 years of age who underwent Evans osteotomy at our hospital from January 2015 to May 2020. The Patients were stratified into 2 groups: Group 1 (pin was left post-operatively across the joint and removed on follow-up) and Group 2 (pin was removed during surgery). Demographic data were recorded, including age, sex, and body mass index. Calcaneocuboid arthritis was determined by radiographic imaging at least one-year follow-up. Time to pin removal, size of the pin and graft, and subluxation were analyzed as possible risk factors for arthritis. Odds ratios (OR) and corresponding 95% confidence intervals (CI) were estimated. RESULTS: 39 patients were included in the study: 24 patients had the pin removed post-operatively, and 15 had the pin removed intra-operatively. There were no significant differences between the 2 groups regarding body mass index, age, sex, or laterality. Calcaneocuboid arthritis rates were significantly higher in the Group 1 than Group 2 (12(50%) vs. 2(13.3%), respectively; p = 0.02). On multivariate regression, both age (OR, 1.305 [95%CI: 1.009-1.696]; p = 0.044) and leaving the pin in the joint postoperatively (OR, 7.661 [95%CI: 1.208-48.570]; p = 0.031) were found as risk factors for arthritis at follow-up. The mean time to pin removal, the size of the pin, and the size of the graft were not found to be significant predictors of arthritis (p > 0.05). CONCLUSIONS: This study shows that leaving the Steinmann pin in the calcaneocuboid joint in children post-operatively is associated with an increased risk of developing early radiographic signs of calcaneocuboid joint arthritis.