BACKGROUND Giant cell hepatitis in the adult population remains very poorly defined with only 100 case reports published in the literature over the last three decades.AIM To present our center’s experience in an atte...BACKGROUND Giant cell hepatitis in the adult population remains very poorly defined with only 100 case reports published in the literature over the last three decades.AIM To present our center’s experience in an attempt to learn about the predisposing factors,outcomes and efficacy of proposed therapeutic interventions for giant cell hepatitis.METHODS A retrospective chart review was conducted through the electronic records of the University of Pittsburgh Medical Center.We queried 36726 liver biopsy reports from January 1,1991 to December 6,2016.Our search yielded 50 patients who were identified as carrying a definite diagnosis of post-infantile giant cell hepatitis(PIGCH)by pathology.The data collected included demographic information,laboratory data(liver function tests,autoimmune markers)and transplant status.In order to better analyze patient characteristics and outcomes,subjects were separated into a non-transplant(native)liver group and a post-liver transplant(allograft)group.RESULTS The incidence of PIGCH was approximately 0.14%of all biopsies queried in the 25-year period.The mean age was 48 years with 66%females.Liver function tests were classified as 38.2%cholestatic,35.3%hepatocellular and 26.5%mixed.Autoimmune hepatitis was found to be the most prevalent predisposing factor leading to PIGCH constituting 32%of cases.Management consisted mainly of immunosuppression,viral targeted therapy,supportive care and in six cases liver transplantations.CONCLUSION The diagnosis of PIGCH remains clinically challenging and requires a high index of suspicion as well as a thorough history,physical examination,serological workup and liver biopsy.Treatment of the underlying cause can result in clinical stability in a large number of cases.展开更多
Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days...Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days before hospitalization.Physical examination found pallor,jaundice and hepatosplenomegaly.The laboratory workup showed serum total bilirubin at 101 μmol/L,conjugated bilirubin at 84 μmol/L,hemolytic anemia,thrombocytopenia and immunoglobulin G(IgG)and anti-C3d positive direct Coombs' test.The antinuclear,anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies,antiendomisium antibodies were negative.Serological assays for viral hepatitis B and C,cytomegalovirus,herpes simplex and Epstein Barr virus were negative.The association of acute liver failure,Evan's syndrome,positive direct Coomb's test of mixed type(IgG and C3)and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH.The diagnosis was confirmed by a needle liver biopsy.The patient was treated by corticosteroids,immunomodulatory therapy and azathioprine but died with septicemia.展开更多
Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of...Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity.The etiology is unknown;concomitant viral infections(as potential trigger factors)have been identified in a few patients.The pathogenesis reportedly relies upon immune-mediated/autoimmune mechanisms.This condition should be considered in any infant developing Coombs-positive anemia;indeed,anemia usually precedes the development of hepatitis.The clinical course is usually aggressive without the appropriate immunosuppressive therapy,which may include steroids,conventional immunosuppressors(e.g.,azathioprine and cyclophosphamide as first-line treatments),intravenous immunoglobulin,and biologics(rituximab).Improvements in medical management(including the availability of rituximab)have significantly reduced the mortality of this condition in the last decade.展开更多
文摘BACKGROUND Giant cell hepatitis in the adult population remains very poorly defined with only 100 case reports published in the literature over the last three decades.AIM To present our center’s experience in an attempt to learn about the predisposing factors,outcomes and efficacy of proposed therapeutic interventions for giant cell hepatitis.METHODS A retrospective chart review was conducted through the electronic records of the University of Pittsburgh Medical Center.We queried 36726 liver biopsy reports from January 1,1991 to December 6,2016.Our search yielded 50 patients who were identified as carrying a definite diagnosis of post-infantile giant cell hepatitis(PIGCH)by pathology.The data collected included demographic information,laboratory data(liver function tests,autoimmune markers)and transplant status.In order to better analyze patient characteristics and outcomes,subjects were separated into a non-transplant(native)liver group and a post-liver transplant(allograft)group.RESULTS The incidence of PIGCH was approximately 0.14%of all biopsies queried in the 25-year period.The mean age was 48 years with 66%females.Liver function tests were classified as 38.2%cholestatic,35.3%hepatocellular and 26.5%mixed.Autoimmune hepatitis was found to be the most prevalent predisposing factor leading to PIGCH constituting 32%of cases.Management consisted mainly of immunosuppression,viral targeted therapy,supportive care and in six cases liver transplantations.CONCLUSION The diagnosis of PIGCH remains clinically challenging and requires a high index of suspicion as well as a thorough history,physical examination,serological workup and liver biopsy.Treatment of the underlying cause can result in clinical stability in a large number of cases.
文摘Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days before hospitalization.Physical examination found pallor,jaundice and hepatosplenomegaly.The laboratory workup showed serum total bilirubin at 101 μmol/L,conjugated bilirubin at 84 μmol/L,hemolytic anemia,thrombocytopenia and immunoglobulin G(IgG)and anti-C3d positive direct Coombs' test.The antinuclear,anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies,antiendomisium antibodies were negative.Serological assays for viral hepatitis B and C,cytomegalovirus,herpes simplex and Epstein Barr virus were negative.The association of acute liver failure,Evan's syndrome,positive direct Coomb's test of mixed type(IgG and C3)and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH.The diagnosis was confirmed by a needle liver biopsy.The patient was treated by corticosteroids,immunomodulatory therapy and azathioprine but died with septicemia.
文摘Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity.The etiology is unknown;concomitant viral infections(as potential trigger factors)have been identified in a few patients.The pathogenesis reportedly relies upon immune-mediated/autoimmune mechanisms.This condition should be considered in any infant developing Coombs-positive anemia;indeed,anemia usually precedes the development of hepatitis.The clinical course is usually aggressive without the appropriate immunosuppressive therapy,which may include steroids,conventional immunosuppressors(e.g.,azathioprine and cyclophosphamide as first-line treatments),intravenous immunoglobulin,and biologics(rituximab).Improvements in medical management(including the availability of rituximab)have significantly reduced the mortality of this condition in the last decade.
