BACKGROUND Erythropoietic protoporphyria(EPP)is a rare genetic disorder stemming from ferrochelatase gene mutations,which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes,skin,bone marrow ...BACKGROUND Erythropoietic protoporphyria(EPP)is a rare genetic disorder stemming from ferrochelatase gene mutations,which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes,skin,bone marrow and liver.Although porphyria-related severe liver damage is rare,its consequences can be severe with limited treatment options.CASE SUMMARY This case study highlights a successful intervention for a 35-year-old male with EPP-related liver impairment,employing a combination of red blood cell(RBC)exchange and therapeutic plasma exchange(TPE).The patient experienced significant symptom relief and a decrease in bilirubin levels following multiple PE sessions and an RBC exchange.CONCLUSION The findings suggest that this combined approach holds promise for managing severe hepatic impairment in EPP.展开更多
BACKGROUND The neutrophil-to-lymphocyte ratio(NLR)is commonly utilized as a prognostic indicator in end-stage liver disease(ESLD),encompassing conditions like liver failure and decompensated cirrhosis.Nevertheless,som...BACKGROUND The neutrophil-to-lymphocyte ratio(NLR)is commonly utilized as a prognostic indicator in end-stage liver disease(ESLD),encompassing conditions like liver failure and decompensated cirrhosis.Nevertheless,some studies have contested the prognostic value of NLR in ESLD.AIM To investigate the ability of NLR to predict ESLD.METHODS Databases,such as Embase,PubMed,Web of Science,Cochrane Library,China National Knowledge Infrastructure,Weipu,and Wanfang,were comprehensively searched to identify studies published before October 2022 assessing the prognostic ability of NLR to predict mortality in patients with ESLD.Effect sizes were calculated using comprehensive meta-analysis software and SATAT 15.1.RESULTS A total of thirty studies involving patients with end-stage liver disease(ESLD)were included in the evaluation.Among the pooled results of eight studies,it was observed that the Neutrophil-to-Lymphocyte Ratio(NLR)was significantly higher in non-survivors compared to survivors(random-effects model:standardized mean difference=1.02,95%confidence interval=0.67-1.37).Additionally,twenty-seven studies examined the associations between NLR and mortality in ESLD patients,reporting either hazard ratios(HR)or odds ratios(OR).The combined findings indicated a link between NLR and ESLD mortality(randomeffects model;univariate HR=1.07,95%CI=1.05-1.09;multivariate HR=1.07,95%CI=1.07-1.09;univariate OR=1.29,95%CI=1.18-1.39;multivariate OR=1.29,95%CI=1.09-1.49).Furthermore,subgroup and meta-regression analyses revealed regional variations in the impact of NLR on ESLD mortality,with Asian studies demonstrating a more pronounced effect.CONCLUSION Increased NLR in patients with ESLD is associated with a higher risk of mortality,particularly in Asian patients.NLR is a useful prognostic biomarker in patients with ESLD.展开更多
文摘BACKGROUND Erythropoietic protoporphyria(EPP)is a rare genetic disorder stemming from ferrochelatase gene mutations,which leads to abnormal accumulation of protoporphyrin IX primarily in erythrocytes,skin,bone marrow and liver.Although porphyria-related severe liver damage is rare,its consequences can be severe with limited treatment options.CASE SUMMARY This case study highlights a successful intervention for a 35-year-old male with EPP-related liver impairment,employing a combination of red blood cell(RBC)exchange and therapeutic plasma exchange(TPE).The patient experienced significant symptom relief and a decrease in bilirubin levels following multiple PE sessions and an RBC exchange.CONCLUSION The findings suggest that this combined approach holds promise for managing severe hepatic impairment in EPP.
基金Supported by the Science and Technology Planning Project of Guangdong Province,China,No.2019B0202280015010 Project of Sun Yatsen University,No.2018024Science and Technology Program of Guangzhou,China,No.202103000060.
文摘BACKGROUND The neutrophil-to-lymphocyte ratio(NLR)is commonly utilized as a prognostic indicator in end-stage liver disease(ESLD),encompassing conditions like liver failure and decompensated cirrhosis.Nevertheless,some studies have contested the prognostic value of NLR in ESLD.AIM To investigate the ability of NLR to predict ESLD.METHODS Databases,such as Embase,PubMed,Web of Science,Cochrane Library,China National Knowledge Infrastructure,Weipu,and Wanfang,were comprehensively searched to identify studies published before October 2022 assessing the prognostic ability of NLR to predict mortality in patients with ESLD.Effect sizes were calculated using comprehensive meta-analysis software and SATAT 15.1.RESULTS A total of thirty studies involving patients with end-stage liver disease(ESLD)were included in the evaluation.Among the pooled results of eight studies,it was observed that the Neutrophil-to-Lymphocyte Ratio(NLR)was significantly higher in non-survivors compared to survivors(random-effects model:standardized mean difference=1.02,95%confidence interval=0.67-1.37).Additionally,twenty-seven studies examined the associations between NLR and mortality in ESLD patients,reporting either hazard ratios(HR)or odds ratios(OR).The combined findings indicated a link between NLR and ESLD mortality(randomeffects model;univariate HR=1.07,95%CI=1.05-1.09;multivariate HR=1.07,95%CI=1.07-1.09;univariate OR=1.29,95%CI=1.18-1.39;multivariate OR=1.29,95%CI=1.09-1.49).Furthermore,subgroup and meta-regression analyses revealed regional variations in the impact of NLR on ESLD mortality,with Asian studies demonstrating a more pronounced effect.CONCLUSION Increased NLR in patients with ESLD is associated with a higher risk of mortality,particularly in Asian patients.NLR is a useful prognostic biomarker in patients with ESLD.
