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作 者:Angelo Di Giorgio Anna Tulone Emanuele Nicastro Lorenzo Norsa Aurelio Sonzogni Lorenzo D'Antiga
机构地区:[1]Pediatric Hepatology,Gastroenterology and Transplantation,Hospital Papa Giovanni XXIII,Bergamo 24127,Italy [2]Liver Pathology,Hospital Papa Giovanni XXIII,Bergamo 24127,Italy
出 处:《World Journal of Hepatology》2021年第12期2113-2127,共15页世界肝病学杂志(英文版)(电子版)
摘 要:BACKGROUND Previous reports showed some beneficial effect of oral vancomycin treatment(OVT)in children with primary sclerosing cholangitis;conversely,the experience in patients with other autoimmune liver diseases(AILD),including autoimmune hepatitis(AIH)and autoimmune sclerosing cholangitis(ASC),is scant.AIM To assess the response to immunosuppressive treatment(IS)and to OVT in children diagnosed with AILD.METHODS Retrospective study of children diagnosed with AIH(normal biliary tree at cholangiography)and ASC(abnormal biliary tree at cholangiography)in the last 10 years.All underwent standard immunosuppressive therapy(IS),but nonresponders received also OVT.Biochemical remission[normal aspartate aminotransferase(AST)]and immunological remission(normal IgG and negative autoantibodies)rates and Sclerosing Cholangitis Outcomes in Pediatrics(SCOPE)index were assessed and compared during the follow up.RESULTS 75 children were included[69%female,median age 10.5 years(5.6-13.4 years),AIH=54,ASC=21].Sixty-three patients(84%,AIH=52,ASC=11)were treated with standard IS and 61 achieved biochemical remission,whereas 12 not responding to IS[16%,F=75%,median age 13.5 years,(12.2-15.7),10 with ASC]required OVT and 8 achieved biochemical remission.Overall OVT increased the biochemical remission rate of the whole group of AILD patients from 81%(61/75)to 92%(69/75).Median values of AST,alanine aminotransferase(ALT)and gamma-glutamyl transferase(GGT)decreased significantly after OVT start(P<0.05).Complete normalization of livers enzymes(AST,ALT and GGT)was observed in 6/12 patients(50%).Decrease in SCOPE index score was reported in 5/12 patients(42%).At last follow up(median of 4.4 years,range 0.6-13.8 years)all 75 patients are alive,6(8%,1 with ASC)successfully discontinued medications,1(with ASC)required liver transplantation.CONCLUSION Children with AIH and ASC respond well to IS treatment.OVT may represent a valuable treatment option to achieve biochemical remission in patients not responding to standard IS.These promising pr
关 键 词:Autoimmune hepatitis Autoimmune sclerosing cholangitis Autoimmune liver disease VANCOMYCIN CHILDREN Liver transplantation
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