Objetives: To investigate the incidence and clinical features of autoimmune hepatitis (AIH) in children from the province of Santa Fe, Argentina, over ten years. Methods: From the records of all of the pediatric hepatologists in the province of Santa Fe, Argentina, we reviewed the clinical charts of patients 6 points) and followed between January 2003 and December 2013. Population data were extracted from the 2010 national census. Values were expressed as percentages and median +/- interquartile range. Mann-Whitney test was used for comparison between groups. Results: 67 patients fulfilled inclusion criteria, from which 11 (16%) were later reclassified as "autoimmune sclerosing cholangitis" (ASC) according to biochemical, histological and radiological findings. A final sample of 56 patients (39 F) with AIH was analyzed, giving an annual incidence of 0.56/100,000. Median age at presentation was 8 (5.7 -11) years, and the median follow-up was 4 (2–7) years. Type 1 AIH was diagnosed in 89%. An acute presentation was observed in 53%, while 13 (23%) showed cirrhosis on initial biopsy. Prednisone (87%), and azathioprine (60%) were the most common drugs prescribed. At the end of follow-up, 53/56 (95%) were alive, including four patients (7%) who underwent liver transplantation. Conclusion: AIH has an estimated incidence of 0.56/100000/year in children from the province of Santa Fe (Argentina). Overall survival was 95%. A subgroup of patients diagnosed as AIH develops predominant biliary disease and should be better classified as ASC. Address correspondence and reprint requests to Alejandro Carlos Costaguta, MD, Division of Hepatology. Sanatorio de Niños. Alvear, 863 (2000) Rosario (SF), Argentina (e-mail: alejandro.costaguta@gmail.com). Received 9 May, 2018 Accepted 19 July, 2018 Conflict of interest, and source of founding: None to declare. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,
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