AIM To summarize the experience in the clinical treatment of the biliary ductal strictures complicating localized left hepatolithiasis in recent two decades.
Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis(AIH),autoimmune sclerosing cholangitis(ASC),and de novo AIH after liver transplantation.AIH is divided into two subtypes ...Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis(AIH),autoimmune sclerosing cholangitis(ASC),and de novo AIH after liver transplantation.AIH is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody(SMA/ANA,type 1) or liver kidney microsomal antibody(LKM1,type 2).There is a female predominance in both.LKM1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity,presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both groups.The most common type of paediatric sclerosing cholangitis is ASC.The clinical,biochemical,immunological,and histological presentation of ASC is often indistinguishable from that of AIH type 1.In both,there are high IgG,non-organ specific autoantibodies,and interface hepatitis.Diagnosis is made by cholangiography.Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters,and decreased inflammatory activity on follow up liver biopsies.However,the cholangiopathy can progress.There may be evolution from AIH to ASC over the years,despite treatment.De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies,hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and collapse.Like classical AIH,it responds to treatment with prednisolone and azathioprine.De novo AIH postliver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection.Whether this condition is a distinct entity or a form of atypical rejection in individuals susceptible to the development of autoimmune phenomena is unclear.Whatever its etiology,the recognition of this potentially life-threatening syndrome is important since its management differs from that of standard anti-rejection therapy.展开更多
Objective: To evaluate the outcome of radiotherapy (RT) in locally advanced hepatic hilar cholangiocarcinoma. Methods: During March 1992 to December 1997, 47 patients who had positive microscopic surgical margins afte...Objective: To evaluate the outcome of radiotherapy (RT) in locally advanced hepatic hilar cholangiocarcinoma. Methods: During March 1992 to December 1997, 47 patients who had positive microscopic surgical margins after surgery were treated by postoperative radiotherapy (S+RT, 28 patients, with a median dose of 50 Gy, 45–62 Gy) and surgery alone (S, 19 patients). The median follow-up was 30 months (4–113 months). Results: The overall 5-year survival rate was 28%, with a median survival length of 19.6 months while 5-year survival rates of S+RT group and S group were 34% and 14%, with median survival lengths of 29 and 10 months respectively (P=0.015). The occurrence rate of complications was 11% in either group. Conclusion: Postoperative radiotherapy significantly prolongs survival lengths in patients with hepatic hilar cholangio- carcinoma who had positive microscopic surgical margins. Radiation toxicities are tolerable.展开更多
文摘AIM To summarize the experience in the clinical treatment of the biliary ductal strictures complicating localized left hepatolithiasis in recent two decades.
文摘Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis(AIH),autoimmune sclerosing cholangitis(ASC),and de novo AIH after liver transplantation.AIH is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody(SMA/ANA,type 1) or liver kidney microsomal antibody(LKM1,type 2).There is a female predominance in both.LKM1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity,presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both groups.The most common type of paediatric sclerosing cholangitis is ASC.The clinical,biochemical,immunological,and histological presentation of ASC is often indistinguishable from that of AIH type 1.In both,there are high IgG,non-organ specific autoantibodies,and interface hepatitis.Diagnosis is made by cholangiography.Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters,and decreased inflammatory activity on follow up liver biopsies.However,the cholangiopathy can progress.There may be evolution from AIH to ASC over the years,despite treatment.De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies,hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and collapse.Like classical AIH,it responds to treatment with prednisolone and azathioprine.De novo AIH postliver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection.Whether this condition is a distinct entity or a form of atypical rejection in individuals susceptible to the development of autoimmune phenomena is unclear.Whatever its etiology,the recognition of this potentially life-threatening syndrome is important since its management differs from that of standard anti-rejection therapy.
文摘Objective: To evaluate the outcome of radiotherapy (RT) in locally advanced hepatic hilar cholangiocarcinoma. Methods: During March 1992 to December 1997, 47 patients who had positive microscopic surgical margins after surgery were treated by postoperative radiotherapy (S+RT, 28 patients, with a median dose of 50 Gy, 45–62 Gy) and surgery alone (S, 19 patients). The median follow-up was 30 months (4–113 months). Results: The overall 5-year survival rate was 28%, with a median survival length of 19.6 months while 5-year survival rates of S+RT group and S group were 34% and 14%, with median survival lengths of 29 and 10 months respectively (P=0.015). The occurrence rate of complications was 11% in either group. Conclusion: Postoperative radiotherapy significantly prolongs survival lengths in patients with hepatic hilar cholangio- carcinoma who had positive microscopic surgical margins. Radiation toxicities are tolerable.
