Autoimmune Hepatitis In Children:clinical Analysis Of Patients In Lyon Hospital And Children’s Hospital Of Chongqing Medical University

Background: Autoimmune hepatitis(AIH) is a progressive chronic liver disease caused by immune disorder.It is characterized by elevated transaminases, hypergammaglobulinemia, the presence of autoantibodies and interface hepatitis histologically.The morbidity is low and the clinical presentation is non-specific of children’s autoimmune hepatitis,so the diagnosis is difficult to make.Objective: This study aims to describe and analyze general conditions, features of onset, clinical presentations, biochemical features and the effects of treatment of children’s autoimmune hepatitis,summarize the clinical characteristics in order to improve the comprehension of autoimmune hepatitis in children.Material and Methods: The medical records including clinical data,liver pathology,treatment and follow-up of 31 children with AIH hospitalized in civil hospital in Lyon from 1989~2006 and 8 children with AIH hospitalized in Children’s Hospital of Chongqing Medical University from 2007~2015 were read and analyzed retrospectively.Results :1.Lyon Hospital:1)Of the 31 children with AIH,the median onset age is 8.90 years,the male female ratio is 1:1.8,7 cases had sudden onset,14cases had subacute onset,10 had chronic onset,the median duration before diagnose is 3 months;2)17(54.8%)patients were diagnosed as type-1AIH(AIH-1),8(25.8%)were diagnosed as type-2 AIH(AIH-2),and 6patients were diagnosed as non-type AIH;3)The major symptom was jaundice,with the rate of 48.4%;4)The average value of biochemical markers were as following:ALT(924.26±798.29)U/L,AST(897.48±809.98)U/L,γ-GT 87.50(55.25,189.75)U/L,TBIL45.00(13.25,94.25)μmol/L,PTA 72%±25%,γ球蛋白(27.80±13.90)g/L;5)The comparison between AIH-1 and AIH-2 reveals that the differences of ALT,AST,γ-GT,TBIL and PTA are not significative,while the gamma-globulin of type 1 is higher than that of type2;6)27 cases have undergone biopsy and 24 cases presented with portal inflammation,while 26 presented with fibrosis;7)10 patients combined with other autoimmune diseases;8)29 patients were treated with prednisone and azathioprine,alone or in combination after diagnosis.20 patients have encountered relapse on account of the diminution or the interruption of immunosuppressants.In the end of follow-up,3 patients have successfully stopped all the immunosuppressants,with normal biochemical markers more than 6 months.While 3 patients have sustained only with azathioprine,with transaminases and gamma-globulin stable and normal;9)The major complications were related to glucocorticoid and mental disorder.2.Children’s Hospital of Chongqing Medical University:1) Of the 8 children with AIH,the median onset age is 7.22 years,the male female ratio is 1:3, 1 cases had sudden onset,4cases had subacute onset,3 had chronic onset,the median duration before diagnose is 3.25 months;2)5(62.5%)patients were diagnosed as type-1 AIH(AIH-1),3 patients were diagnosed as non-type AIH;3)The major symptom was jaundice,with the rate of 75%;4) The average value of biochemical markers were as following: ALT(984.14±452.43)U/L, AST(1120.79±591.02)U/L, γ-GT 191.55(142.85,809.25)U/L, TBIL108.25(80.03,256.35)μmol/L,PT(27.00±13.22)s,Ig G(23.59±9.91)g/L;5)2 cases obtained liver biopsy,the infiltration of lymphocytes and plasmocytes and the fibrosis in portal area is obvious;6)1 case combined with other autoimmune disease;7)1 case treated with dexamethasone,2 treated with predison,3 treated with ordinary liver protection medicine,1 was transferred to another hospital and 1 was given up during the hospitalization.Conclusion:1)The range of onset age is wide for children with AIH,but the majority is around pre-puberty.For most of the patients,the onset is chronic and the duration is long before diagnosis. AIH-1 is more common than AIH-2.2)The symptoms are non-specific,with jaundice as the most common symptom.3)The treatment with prednisone and azathioprine is effective,but the relapse is universal during the drug withdrawal which is very difficult to reach.4)There is no significant difference of the onset age and the transaminases level between French and Chinese patients.