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- W4200340895 abstract "One of the ongoing challenges with regard to the outcome and treatment of juvenile sclerosing cholangitis (JSC) is the limited number of published series, with the majority coming from small, single-center studies. Whereas the multicenter study of Deneau et al addressed the sample size, its limitations are the lack of a consensus on diagnostic tests including liver histology, radiology, and treatment in the various centres. 1 Deneau M.R. El-Matary W. Valentino P.L. Abdou R. Alqoaer K. Amin M. et al. The natural history of primary sclerosing cholangitis in 781 children: a multicenter, international collaboration. Hepatology. 2017; 66: 518-527 Crossref PubMed Scopus (78) Google Scholar We feel that one of the strengths of our study is that patients with JSC were managed following an identical diagnostic and therapeutic protocol, thereby addressing these limitations. Our diagnostic workup does include immunoglobulins and comprehensive autoantibody screen, liver biopsy, and cholangiography in all patients, increasing the diagnostic yield for autoimmune sclerosing cholangitis overlap syndrome (ASC), reflected in the greater prevalence of 72% in our present study compared with 28% in the multicenter cohort. The patients with ASC were more likely to have evidence of moderate-to-severe fibrosis on histology compared with the primary sclerosing cholangitis cohort (89% vs 61%), but this did not reach statistical significance." @default.
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- W4200340895 date "2021-09-01" @default.
- W4200340895 modified "2023-10-18" @default.
- W4200340895 title "Reply" @default.
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- W4200340895 doi "https://doi.org/10.1016/j.jpeds.2021.09.011" @default.
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