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
BACKGROUND Giant paraesophageal hiatal hernias (HH) are very infrequent,and their spectrum of clinical manifestations is large.Giant HH mainly occurs in elderly patients,and its relationship with anemia has been repor...BACKGROUND Giant paraesophageal hiatal hernias (HH) are very infrequent,and their spectrum of clinical manifestations is large.Giant HH mainly occurs in elderly patients,and its relationship with anemia has been reported.For the surgical treatment of large HH,Nissen fundoplication is the most common antireflux procedure,and the reinforcement of HH repair with a patch (either synthetic or biologic) is still debatable.CASE SUMMARY We report on a case of giant paraesophageal HH in a middle-aged male patient with reflux symptoms and severe anemia.After performing a series of tests and diagnostic approaches,results showed a complete intrathoradc stomach associated with severe iron deficiency anemia.The patient underwent successful laparoscopic hernia repair with mesh reinforcement and Nissen fundoplication.Postoperatively,reflux symptoms were markedly relieved,and the imaging study showed complete reduction of the hernia sac.More importantly,anemia was resolved,and hemoglobin,serum iron and ferritin level were returned to the normal range.The patient kept regular follow-up appointments and remained in a satisfactory condition.CONCLUSION This case report highlights the relationship between large HH and iron deficiency anemia.For the surgical treatment of large HH,laparoscopic repair of large HH combined with antireflux procedure and mesh reinforcement is recommended.展开更多
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BACKGROUND Giant paraesophageal hiatal hernias (HH) are very infrequent,and their spectrum of clinical manifestations is large.Giant HH mainly occurs in elderly patients,and its relationship with anemia has been reported.For the surgical treatment of large HH,Nissen fundoplication is the most common antireflux procedure,and the reinforcement of HH repair with a patch (either synthetic or biologic) is still debatable.CASE SUMMARY We report on a case of giant paraesophageal HH in a middle-aged male patient with reflux symptoms and severe anemia.After performing a series of tests and diagnostic approaches,results showed a complete intrathoradc stomach associated with severe iron deficiency anemia.The patient underwent successful laparoscopic hernia repair with mesh reinforcement and Nissen fundoplication.Postoperatively,reflux symptoms were markedly relieved,and the imaging study showed complete reduction of the hernia sac.More importantly,anemia was resolved,and hemoglobin,serum iron and ferritin level were returned to the normal range.The patient kept regular follow-up appointments and remained in a satisfactory condition.CONCLUSION This case report highlights the relationship between large HH and iron deficiency anemia.For the surgical treatment of large HH,laparoscopic repair of large HH combined with antireflux procedure and mesh reinforcement is recommended.
