Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating Syst...Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating System (MARS) has been reported to reverse severe cholestasis-linked pruritus. Here, we report the first use of MARS during a spontaneous pregnancy and its successful outcome in a patient with PFIC3 and intractable pruritus. Albumin dialysis could be considered as a pregnancy-saving procedure in pregnant women with severe cholestasis and refractory pruritus.展开更多
Massive ascites and hepatorenal syndrome (HRS) are frequent complications of liver cirrhosis. Thus, effective therapy is of great clinical importance. This concise review provides an update of recent advances and ne...Massive ascites and hepatorenal syndrome (HRS) are frequent complications of liver cirrhosis. Thus, effective therapy is of great clinical importance. This concise review provides an update of recent advances and new developments. Therapeutic paracentesis can be safely performed even in patients with severe coagulopathy. Selected patients with a refractory or recurrent ascites are good candidates for non-surgical portosysternic shunts (TIPS) and may have a survival benefit and improvement of quality of life. Novel pharmaceutical agents mobilizing free water (aquaretics) are currently under test for the therapeutic potential in patients with ascites. Prophylaxis of hepatorenal syndrome in patients with spontaneous bacterial peritonitis is recommended and should be considered in patients with alcoholic hepatitis. Liver transplantation is the best therapeutic option with long-term survival benefit for patients with HRS. To bridge the time until transplantation, TIPS or Terlipressin and albumin are good options. Albumin dialysis can not be recommended outside prospective trials.展开更多
AIM: To evaluate whether treatment with the Prometheus system significantly affects cytokines, coagulation factors and other plasma proteins. METHODS: We studied nine patients with acute-onchronic liver failure and ...AIM: To evaluate whether treatment with the Prometheus system significantly affects cytokines, coagulation factors and other plasma proteins. METHODS: We studied nine patients with acute-onchronic liver failure and accompanying renal failure. Prometheus therapy was performed on 2 consecutive days for up to 6 h in all patients. Several biochemical parameters and blood counts were assessed at regular time points during Prometheus treatment. RESULTS: We observed a significant decrease of both protein-bound (e.g. bile acids) and water-soluble (e.g. ammonia) substances after Prometheus therapy. Even though leukocytes increased during treatment (P〈 0.01), we found no significant changes of C-reactive protein, interleukin-6, and tumor necrosis factor-o plasma levels (all P 〉 0.5). Further, antithrombin 3, factor II and factor V plasma levels did not decrease during Prometheus therapy (all P 〉0.5), and the INR remained unchanged (P = 0.4). Plasma levels of total protein, albumin, and fibrinogen were also not altered during Prometheus treatment (all P 〉 0.5). Finally, platelet count did not change significantly during therapy (P= 0.6). CONCLUSION: Despite significant removal of protein- bound and water-soluble substances, Prometheus therapy did not affect the level of cytokines, coagulation factors or other plasma proteins. Thus, the filters and adsorbers used in the system are highly effective and specific for water-soluble substances and toxins bound to the albumin fraction.展开更多
AIM:To determine the short-term cost-utility of mo-lecular adsorbent recirculating system(MARS) treatment in acute liver failure(ALF).METHODS:A controlled retrospective study was conducted with 90 ALF patients treated...AIM:To determine the short-term cost-utility of mo-lecular adsorbent recirculating system(MARS) treatment in acute liver failure(ALF).METHODS:A controlled retrospective study was conducted with 90 ALF patients treated with MARS from 2001 to 2005.Comparisons were made with a historical control group of 17 ALF patients treated from 2000 to 2001 in the same intensive care unit(ICU) specializing in liver diseases.The 3-year outcomes and number of liver transplantations were recorded.All direct liver disease-related medical expenses from 6 mo before to 3 years after ICU treatment were determined for 31 MARS patients and 16 control patients.The health-related quality of life(HRQoL) before MARS treatment was estimated by a panel of ICU doctors and after MARS using a mailed 15D(15-dimensional generic healthrelated quality of life instrument) questionnaire.The HRQoL,cost,and survival data were combined and the incremental cost/quality-adjusted life years(QALYs) was calculated.RESULTS:In surviving ALF patients,the health-related quality of life after treatmeant was generally high and comparable to the age-and gender-matched general Finnish population.Compared to the controls,the average cost per QALY was considerably lower in the MARS group(64 732€ vs 133 858€) within a timeframe of 3.5 years.The incremental cost of standard medical treatment alone compared to MARS was 10 928€,and the incremental number of QALYs gained by MARS was 0.66.CONCLUSION:MARS treatment combined with standard medical treatment for ALF in an ICU setting is more cost-effective than standard medical treatment alone.展开更多
文摘Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating System (MARS) has been reported to reverse severe cholestasis-linked pruritus. Here, we report the first use of MARS during a spontaneous pregnancy and its successful outcome in a patient with PFIC3 and intractable pruritus. Albumin dialysis could be considered as a pregnancy-saving procedure in pregnant women with severe cholestasis and refractory pruritus.
文摘Massive ascites and hepatorenal syndrome (HRS) are frequent complications of liver cirrhosis. Thus, effective therapy is of great clinical importance. This concise review provides an update of recent advances and new developments. Therapeutic paracentesis can be safely performed even in patients with severe coagulopathy. Selected patients with a refractory or recurrent ascites are good candidates for non-surgical portosysternic shunts (TIPS) and may have a survival benefit and improvement of quality of life. Novel pharmaceutical agents mobilizing free water (aquaretics) are currently under test for the therapeutic potential in patients with ascites. Prophylaxis of hepatorenal syndrome in patients with spontaneous bacterial peritonitis is recommended and should be considered in patients with alcoholic hepatitis. Liver transplantation is the best therapeutic option with long-term survival benefit for patients with HRS. To bridge the time until transplantation, TIPS or Terlipressin and albumin are good options. Albumin dialysis can not be recommended outside prospective trials.
文摘AIM: To evaluate whether treatment with the Prometheus system significantly affects cytokines, coagulation factors and other plasma proteins. METHODS: We studied nine patients with acute-onchronic liver failure and accompanying renal failure. Prometheus therapy was performed on 2 consecutive days for up to 6 h in all patients. Several biochemical parameters and blood counts were assessed at regular time points during Prometheus treatment. RESULTS: We observed a significant decrease of both protein-bound (e.g. bile acids) and water-soluble (e.g. ammonia) substances after Prometheus therapy. Even though leukocytes increased during treatment (P〈 0.01), we found no significant changes of C-reactive protein, interleukin-6, and tumor necrosis factor-o plasma levels (all P 〉 0.5). Further, antithrombin 3, factor II and factor V plasma levels did not decrease during Prometheus therapy (all P 〉0.5), and the INR remained unchanged (P = 0.4). Plasma levels of total protein, albumin, and fibrinogen were also not altered during Prometheus treatment (all P 〉 0.5). Finally, platelet count did not change significantly during therapy (P= 0.6). CONCLUSION: Despite significant removal of protein- bound and water-soluble substances, Prometheus therapy did not affect the level of cytokines, coagulation factors or other plasma proteins. Thus, the filters and adsorbers used in the system are highly effective and specific for water-soluble substances and toxins bound to the albumin fraction.
基金Supported by Scientific grants from the Helsinki University Central Hospital Research Fund (EVO) and the Finnish Office for Health Technology Assessment
文摘AIM:To determine the short-term cost-utility of mo-lecular adsorbent recirculating system(MARS) treatment in acute liver failure(ALF).METHODS:A controlled retrospective study was conducted with 90 ALF patients treated with MARS from 2001 to 2005.Comparisons were made with a historical control group of 17 ALF patients treated from 2000 to 2001 in the same intensive care unit(ICU) specializing in liver diseases.The 3-year outcomes and number of liver transplantations were recorded.All direct liver disease-related medical expenses from 6 mo before to 3 years after ICU treatment were determined for 31 MARS patients and 16 control patients.The health-related quality of life(HRQoL) before MARS treatment was estimated by a panel of ICU doctors and after MARS using a mailed 15D(15-dimensional generic healthrelated quality of life instrument) questionnaire.The HRQoL,cost,and survival data were combined and the incremental cost/quality-adjusted life years(QALYs) was calculated.RESULTS:In surviving ALF patients,the health-related quality of life after treatmeant was generally high and comparable to the age-and gender-matched general Finnish population.Compared to the controls,the average cost per QALY was considerably lower in the MARS group(64 732€ vs 133 858€) within a timeframe of 3.5 years.The incremental cost of standard medical treatment alone compared to MARS was 10 928€,and the incremental number of QALYs gained by MARS was 0.66.CONCLUSION:MARS treatment combined with standard medical treatment for ALF in an ICU setting is more cost-effective than standard medical treatment alone.