AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS betwe...AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS between June 1988 and May 2008.The diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography,computed tomography,magnetic resonance imaging,or venography.The median follow-up period was 103 ± 156 [interquartile range(IQR)] mo.RESULTS:The median age of the patients was 47 ± 16(IQR) years.At diagnosis,54 patients had cirrhosis,25(37.3%) Child-Pugh class A,23(34.3%) Child-Pugh class B,and six(9.0%) patients Child-Pugh class C.During the follow-up period,HCC was developed in 17 patients,and the annual incidence of HCC in patients with BCS was 2.8%.Patients in HCC group(n = 17) had higher hepatic venous pressure gradient(HVPG) than those in non-HCC group(n = 50)(21 ± 12 mmHg vs 14 ± 7 mmHg,P = 0.019).The survival rate of BCS patients was 86.2% for 5 years,73.8% for 10 years,and 61.2% for 15 years.In patients with BCS and HCC,survival was 79% for 5 years,43.1% for 10 years,and 21.5% for 15 years.CONCLUSION:The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South Korea.The HVPG is expected to provide additional information for predicting HCC development in BCS patients.展开更多
AIM:To evaluate the clinical outcomes and prognostic factors after intravenous corticosteroids following oral corticosteroid failure in active ulcerative colitis patients.METHODS:Consecutive patients with moderate to ...AIM:To evaluate the clinical outcomes and prognostic factors after intravenous corticosteroids following oral corticosteroid failure in active ulcerative colitis patients.METHODS:Consecutive patients with moderate to severe ulcerative colitis who had been treated with a course of intravenous corticosteroids after oral corticosteroid therapy failure between January 1996 and July 2010 were recruited at Severance Hospital,Seoul,South Korea.The disease activity was measured by the Mayo score,which consists of stool frequency,rectal bleeding,mucosal appearance at flexible sigmoidoscopy,and Physician Global Assessment.We retrospectively evaluated clinical outcomes at two weeks,one month,three months,and one year after the initiation of intravenous corticosteroid therapy.Two weeks outcomes were classified as responders or non-responders.One month,three month and one year outcomes were classified into prolonged response,steroid dependency,and refractoriness.RESULTS:Our study included a total of 67 eligible patients.At two weeks,56(83.6%) patients responded to intravenous corticosteroids.At one month,complete remission was documented in 18(32.1%) patients and partial remission in 26(46.4%).Eleven patients(19.7%) were refractory to the treatment.At three months and one year,we found 37(67.3%) and 25(46.3%) patients in prolonged response,ten(18.2%) and 23(42.6%) patients in corticosteroid dependency,8(14.5%) and 6(11.1%) patients with no response,respectively.Total 9 patients were underwent elective proctocolectomy within 1 year.The duration of oral corticosteroid therapy(> 14 d vs ≤ 14 d,P = 0.049) and lower hemoglobin level(≤ 11.0 mg/dL vs >11.0 mg/dL,P = 0.02) were found to be poor prognostic factors for response at two weeks.For one year outcome,univariate analysis revealed that only a partial Mayo score(≥ 6 vs <6,P = 0.057) was found to be associated with a poor response.CONCLUSION:The duration of oral corticosteroid therapy and lower hemoglobin level were strongly associated with poor outcome.展开更多
基金Supported by A grant of the South Korea Healthcare Technology R and D Project,Ministry of Health and Welfare,South Korea,No.A102065
文摘AIM:To evaluate long-term clinical course of BuddChiari syndrome(BCS) and predictive factors associated with the development of hepatocellular carcinoma(HCC) and survival.METHODS:We analyzed 67 patients with BCS between June 1988 and May 2008.The diagnosis of BCS was confirmed by hepatic venous outflow obstruction shown on abdominal ultrasound sonography,computed tomography,magnetic resonance imaging,or venography.The median follow-up period was 103 ± 156 [interquartile range(IQR)] mo.RESULTS:The median age of the patients was 47 ± 16(IQR) years.At diagnosis,54 patients had cirrhosis,25(37.3%) Child-Pugh class A,23(34.3%) Child-Pugh class B,and six(9.0%) patients Child-Pugh class C.During the follow-up period,HCC was developed in 17 patients,and the annual incidence of HCC in patients with BCS was 2.8%.Patients in HCC group(n = 17) had higher hepatic venous pressure gradient(HVPG) than those in non-HCC group(n = 50)(21 ± 12 mmHg vs 14 ± 7 mmHg,P = 0.019).The survival rate of BCS patients was 86.2% for 5 years,73.8% for 10 years,and 61.2% for 15 years.In patients with BCS and HCC,survival was 79% for 5 years,43.1% for 10 years,and 21.5% for 15 years.CONCLUSION:The incidence of HCC in patients with BCS was similar to that in patients with other etiologic cirrhosis in South Korea.The HVPG is expected to provide additional information for predicting HCC development in BCS patients.
文摘AIM:To evaluate the clinical outcomes and prognostic factors after intravenous corticosteroids following oral corticosteroid failure in active ulcerative colitis patients.METHODS:Consecutive patients with moderate to severe ulcerative colitis who had been treated with a course of intravenous corticosteroids after oral corticosteroid therapy failure between January 1996 and July 2010 were recruited at Severance Hospital,Seoul,South Korea.The disease activity was measured by the Mayo score,which consists of stool frequency,rectal bleeding,mucosal appearance at flexible sigmoidoscopy,and Physician Global Assessment.We retrospectively evaluated clinical outcomes at two weeks,one month,three months,and one year after the initiation of intravenous corticosteroid therapy.Two weeks outcomes were classified as responders or non-responders.One month,three month and one year outcomes were classified into prolonged response,steroid dependency,and refractoriness.RESULTS:Our study included a total of 67 eligible patients.At two weeks,56(83.6%) patients responded to intravenous corticosteroids.At one month,complete remission was documented in 18(32.1%) patients and partial remission in 26(46.4%).Eleven patients(19.7%) were refractory to the treatment.At three months and one year,we found 37(67.3%) and 25(46.3%) patients in prolonged response,ten(18.2%) and 23(42.6%) patients in corticosteroid dependency,8(14.5%) and 6(11.1%) patients with no response,respectively.Total 9 patients were underwent elective proctocolectomy within 1 year.The duration of oral corticosteroid therapy(> 14 d vs ≤ 14 d,P = 0.049) and lower hemoglobin level(≤ 11.0 mg/dL vs >11.0 mg/dL,P = 0.02) were found to be poor prognostic factors for response at two weeks.For one year outcome,univariate analysis revealed that only a partial Mayo score(≥ 6 vs <6,P = 0.057) was found to be associated with a poor response.CONCLUSION:The duration of oral corticosteroid therapy and lower hemoglobin level were strongly associated with poor outcome.