Background Lipoprotein glomerulopathy (LPG) is a renal disease characterized by thrombus-like lipoproteins in the glomerular capillaries and its abnormal lipoprotein profiles with marked elevation of apolipoprotein E ...Background Lipoprotein glomerulopathy (LPG) is a renal disease characterized by thrombus-like lipoproteins in the glomerular capillaries and its abnormal lipoprotein profiles with marked elevation of apolipoprotein E (apoE). In this study, 15 Chinese patients with LPG were involed in exploring the association of the genetic variation and its plasma level in the pathogenesis of LPG.Methods A retrospective analysis of the clinical and pathological features was made in 15 patients with LPG. Plasma concentrations of apoE were measured with radial immunodiffusion assay. Genetic variations of apoE gene were detected using polymerase chain reaction and restriction fragment length polymorphism. Glomerular deposition of apoA, apoB and apoE in these patients were detected by immunofluorescence staining using monoclonal antibodies. Results Biochemical profiles of lipids and lipoproteins revealed markedly elevated levels of triglyceride, apoB and apoE, but approximately normal levels of total cholesterol, apoA1 and lipoprotein(a) [Lp(a)], which resembled familial hypertriglyceridemia. Genetic analysis demonstrated that the genotype distribution of apoE were 7 cases with (ε3/ε 4,)4 cases with ε3/ε 3 and 2 cases with ε2/ε 3. The other 2 cases (a mother and her son) showed a same distinct band. The band pattern of later 2 cases was quite similar to the apoE variant of Tokyo type. The calculated allele frequency of ε 4 was relatively high in cases with LPG in comparison with that in the normal controls. We further divided the 13 patients into three groups according to their genotypes of apoE. Patients with the genotype of apoE ε2/ε3 showed a lower level of plasma apoE as compared to those with apoE ε3/ε4 (P<0.05). The serum level of high-density lipoprotein (HDL) was the lowest in patients with the genotype of apoE ε3/ε4. No difference was found among the patients with different apoE genotype in the other clinical and pathological characteristics. Conclusions The genotype of apoE ε3/ε4 is the predominant one in Chinese patients with LPG. Patients with this genotype tend to have a higher plasma level of apoE and more severe lipid dysmetabolism. No correlation was found between the genotype of apoE and the clinical features in patients with LPG.展开更多
Plasma lipids are controlled by genes and play an important role in the development of atherosclerosis. Dysplipidemia is an important risk factor for coronary artery disease (CAD). Coronary artery disease is the leadi...Plasma lipids are controlled by genes and play an important role in the development of atherosclerosis. Dysplipidemia is an important risk factor for coronary artery disease (CAD). Coronary artery disease is the leading cause of mortality and morbidity in the developed world. More than 14 million Americans are afflicted with clinically significant CAD.~1 To illustrate the impact of CAD in developed countries, the medical and societal costs of CAD in the United States alone are in excess of $90 billion annually.~1 More than (600 000) Americans each year develop new cardiac events, more than 10% of which occur in Americans (<50) years of age.~1 Identifying genetic predisposition to early onset of CAD could help in understanding basic disease mechanism, guiding targeted preventive efforts, and planning appropriate therapeutic strategies.展开更多
目的:观察双重血浆滤过(DFPP)治疗脂蛋白肾病(LPG)患者的临床疗效及安全性。方法:回顾性分析2010年1月至2016年4月于东部战区总医院国家肾脏疾病临床医学研究中心经肾活检病理确诊为LPG,并行DFPP治疗的患者共17例,比较治疗前后的血清肌...目的:观察双重血浆滤过(DFPP)治疗脂蛋白肾病(LPG)患者的临床疗效及安全性。方法:回顾性分析2010年1月至2016年4月于东部战区总医院国家肾脏疾病临床医学研究中心经肾活检病理确诊为LPG,并行DFPP治疗的患者共17例,比较治疗前后的血清肌酐(SCr)、血浆载脂蛋白E(ApoE)及尿蛋白定量的变化及蛋白尿缓解率,DFPP治疗相关并发症,以及随访中主要临床指标变化。结果:15例肾活检证实的LPG患者接受了3~10次DFPP治疗。所有患者均接受降脂、降压药物等对症支持治疗。DFPP治疗后,SCr(155.58±76.91μmol/L vs 137.02±73.37μmol/L,P<0.01)、ApoE(7.94±1.87 mg/dl vs 3.58±1.32 mg/dl,P<0.01)及尿蛋白定量(4.68±2.50 g/24h vs 2.70±2.20 g/24h,P<0.01)均较显著下降。其中1例(6.7%)患者蛋白尿完全缓解,5例(33.3%)患者蛋白尿部分缓解;4例患者DFPP后行重复肾活检,2例DFPP治疗10次者肾组织脂蛋白栓子几乎完全消失,ApoE染色转阴,分别接受3次及4次DFPP治疗的2例患者肾组织栓子无明显减少。2例(13.3%)患者因变态反应无法耐受DFPP治疗,部分患者在治疗过程中出现血压下降(10~20 mmHg)及心率增快。中位随访时间为7月(IQR 1~32),随访中患者ApoE水平较DFPP后均有升高。尿蛋白达到部分缓解及完全缓解并随访>12月的4例患者,3例蛋白尿稳定。结论:DFPP治疗LPG短期疗效明确,总体安全性及患者耐受性较好。展开更多
文摘Background Lipoprotein glomerulopathy (LPG) is a renal disease characterized by thrombus-like lipoproteins in the glomerular capillaries and its abnormal lipoprotein profiles with marked elevation of apolipoprotein E (apoE). In this study, 15 Chinese patients with LPG were involed in exploring the association of the genetic variation and its plasma level in the pathogenesis of LPG.Methods A retrospective analysis of the clinical and pathological features was made in 15 patients with LPG. Plasma concentrations of apoE were measured with radial immunodiffusion assay. Genetic variations of apoE gene were detected using polymerase chain reaction and restriction fragment length polymorphism. Glomerular deposition of apoA, apoB and apoE in these patients were detected by immunofluorescence staining using monoclonal antibodies. Results Biochemical profiles of lipids and lipoproteins revealed markedly elevated levels of triglyceride, apoB and apoE, but approximately normal levels of total cholesterol, apoA1 and lipoprotein(a) [Lp(a)], which resembled familial hypertriglyceridemia. Genetic analysis demonstrated that the genotype distribution of apoE were 7 cases with (ε3/ε 4,)4 cases with ε3/ε 3 and 2 cases with ε2/ε 3. The other 2 cases (a mother and her son) showed a same distinct band. The band pattern of later 2 cases was quite similar to the apoE variant of Tokyo type. The calculated allele frequency of ε 4 was relatively high in cases with LPG in comparison with that in the normal controls. We further divided the 13 patients into three groups according to their genotypes of apoE. Patients with the genotype of apoE ε2/ε3 showed a lower level of plasma apoE as compared to those with apoE ε3/ε4 (P<0.05). The serum level of high-density lipoprotein (HDL) was the lowest in patients with the genotype of apoE ε3/ε4. No difference was found among the patients with different apoE genotype in the other clinical and pathological characteristics. Conclusions The genotype of apoE ε3/ε4 is the predominant one in Chinese patients with LPG. Patients with this genotype tend to have a higher plasma level of apoE and more severe lipid dysmetabolism. No correlation was found between the genotype of apoE and the clinical features in patients with LPG.
文摘Plasma lipids are controlled by genes and play an important role in the development of atherosclerosis. Dysplipidemia is an important risk factor for coronary artery disease (CAD). Coronary artery disease is the leading cause of mortality and morbidity in the developed world. More than 14 million Americans are afflicted with clinically significant CAD.~1 To illustrate the impact of CAD in developed countries, the medical and societal costs of CAD in the United States alone are in excess of $90 billion annually.~1 More than (600 000) Americans each year develop new cardiac events, more than 10% of which occur in Americans (<50) years of age.~1 Identifying genetic predisposition to early onset of CAD could help in understanding basic disease mechanism, guiding targeted preventive efforts, and planning appropriate therapeutic strategies.
文摘目的:观察双重血浆滤过(DFPP)治疗脂蛋白肾病(LPG)患者的临床疗效及安全性。方法:回顾性分析2010年1月至2016年4月于东部战区总医院国家肾脏疾病临床医学研究中心经肾活检病理确诊为LPG,并行DFPP治疗的患者共17例,比较治疗前后的血清肌酐(SCr)、血浆载脂蛋白E(ApoE)及尿蛋白定量的变化及蛋白尿缓解率,DFPP治疗相关并发症,以及随访中主要临床指标变化。结果:15例肾活检证实的LPG患者接受了3~10次DFPP治疗。所有患者均接受降脂、降压药物等对症支持治疗。DFPP治疗后,SCr(155.58±76.91μmol/L vs 137.02±73.37μmol/L,P<0.01)、ApoE(7.94±1.87 mg/dl vs 3.58±1.32 mg/dl,P<0.01)及尿蛋白定量(4.68±2.50 g/24h vs 2.70±2.20 g/24h,P<0.01)均较显著下降。其中1例(6.7%)患者蛋白尿完全缓解,5例(33.3%)患者蛋白尿部分缓解;4例患者DFPP后行重复肾活检,2例DFPP治疗10次者肾组织脂蛋白栓子几乎完全消失,ApoE染色转阴,分别接受3次及4次DFPP治疗的2例患者肾组织栓子无明显减少。2例(13.3%)患者因变态反应无法耐受DFPP治疗,部分患者在治疗过程中出现血压下降(10~20 mmHg)及心率增快。中位随访时间为7月(IQR 1~32),随访中患者ApoE水平较DFPP后均有升高。尿蛋白达到部分缓解及完全缓解并随访>12月的4例患者,3例蛋白尿稳定。结论:DFPP治疗LPG短期疗效明确,总体安全性及患者耐受性较好。
