Non-classical manifestations of autoimmune hepatitis can delay diagnosis and treatment. Our aims were to describe the clinical phenotypes that can confound the diagnosis, detail scoring systems that can ensure their r...Non-classical manifestations of autoimmune hepatitis can delay diagnosis and treatment. Our aims were to describe the clinical phenotypes that can confound the diagnosis, detail scoring systems that can ensure their recognition, and outline advances in treatment that can improve their outcome. Prime source and review articles in English were selected throuqh Medline from 1970-2008 and assimilated into personal libraries spanning 32 years. Acute severe or asymptomatic presentations and atypical histological findings,including centrilobular zone 3 necrosis and concurrent bile duct changes, are compatible with the diagnosis. Cholangiographic abnormalities may be present in children and adults with the disease, and autoimmune hepatitis must be considered in patients without autoantibodies or with antimitochondrial antibodies and no other cholestatic features. Asymptomatic patients frequently become symptomatic; mild disease can progress; and there are no confident indices that justify withholding treatment. Two diagnostic scoring systems with complementary virtues have been developed to evaluate patients with confusing features. Normal liver tests and tissue constitute the optimal end point of treatment, and the first relapse is an indication for long- term azathioprine therapy. Cyclosporine, tacrolimus and mycophenolate mofetil are promising salvage therapies,and budesonide with azathioprine may be a superior frontline treatment. We conclude that the non-classical phenotypes of autoimmune hepatitis can be recognized promptly, diagnosed accurately, and treated effectively.展开更多
Objective:Ectopic tissue transplantation is not a new idea.Godina and his colleagues pioneered this method in the 1980s.This method is a last resort method of preserving an amputated body part,which consists of banki...Objective:Ectopic tissue transplantation is not a new idea.Godina and his colleagues pioneered this method in the 1980s.This method is a last resort method of preserving an amputated body part,which consists of banking the amputated segment in an ectopic area and returning it to its native place at a later date.In this article we present our experience with this demanding procedure.Methods:Debridement was the mainstay of this procedure.The stump and amputated part are carefully debrided and the stump was either closed primarily or covered by a flap.The amputated part was transplanted to one of several banking sites in the body and at a later date it will be transferred to its native site in an elective setting.Results:Seven patients meeting the set criteria for ectopic transplantation were enrolled in this study.The overall success rate was about 70%,lower than expected but these are cases of severe crush injury.Although the functional recovery of these patients are very low,all of the successful cases except one could find a job as a janitor or light manual worker.No patient could return to his previous job.Conclusion:Ectopic transplantation of body parts is an accepted method of treatment of severely crushed extremity or finger injuries.In our country an amputee has very little chance of finding a job instead a disabled person can.In addition in Iran cultures amputation is seen as punishment of either the God or the society,so it is not well accepted and many patients persist on saving the limb even with no functional recovery.None of our successful cases could return to his previous occupation but almost all of them could find a job as janitors or light manual workers.展开更多
文摘Non-classical manifestations of autoimmune hepatitis can delay diagnosis and treatment. Our aims were to describe the clinical phenotypes that can confound the diagnosis, detail scoring systems that can ensure their recognition, and outline advances in treatment that can improve their outcome. Prime source and review articles in English were selected throuqh Medline from 1970-2008 and assimilated into personal libraries spanning 32 years. Acute severe or asymptomatic presentations and atypical histological findings,including centrilobular zone 3 necrosis and concurrent bile duct changes, are compatible with the diagnosis. Cholangiographic abnormalities may be present in children and adults with the disease, and autoimmune hepatitis must be considered in patients without autoantibodies or with antimitochondrial antibodies and no other cholestatic features. Asymptomatic patients frequently become symptomatic; mild disease can progress; and there are no confident indices that justify withholding treatment. Two diagnostic scoring systems with complementary virtues have been developed to evaluate patients with confusing features. Normal liver tests and tissue constitute the optimal end point of treatment, and the first relapse is an indication for long- term azathioprine therapy. Cyclosporine, tacrolimus and mycophenolate mofetil are promising salvage therapies,and budesonide with azathioprine may be a superior frontline treatment. We conclude that the non-classical phenotypes of autoimmune hepatitis can be recognized promptly, diagnosed accurately, and treated effectively.
文摘Objective:Ectopic tissue transplantation is not a new idea.Godina and his colleagues pioneered this method in the 1980s.This method is a last resort method of preserving an amputated body part,which consists of banking the amputated segment in an ectopic area and returning it to its native place at a later date.In this article we present our experience with this demanding procedure.Methods:Debridement was the mainstay of this procedure.The stump and amputated part are carefully debrided and the stump was either closed primarily or covered by a flap.The amputated part was transplanted to one of several banking sites in the body and at a later date it will be transferred to its native site in an elective setting.Results:Seven patients meeting the set criteria for ectopic transplantation were enrolled in this study.The overall success rate was about 70%,lower than expected but these are cases of severe crush injury.Although the functional recovery of these patients are very low,all of the successful cases except one could find a job as a janitor or light manual worker.No patient could return to his previous job.Conclusion:Ectopic transplantation of body parts is an accepted method of treatment of severely crushed extremity or finger injuries.In our country an amputee has very little chance of finding a job instead a disabled person can.In addition in Iran cultures amputation is seen as punishment of either the God or the society,so it is not well accepted and many patients persist on saving the limb even with no functional recovery.None of our successful cases could return to his previous occupation but almost all of them could find a job as janitors or light manual workers.