IgA肾病(immunoglobulin A nephropathy,IgAN)属于常见的原发性肾小球肾炎之一,也是终末期肾病(end-stage renal disease,ESRD)的主要病因之一。IgAN的治疗仍然存在挑战,干预相关的危险因素,可延缓疾病的进展。近年发现,高尿酸血症是IgA...IgA肾病(immunoglobulin A nephropathy,IgAN)属于常见的原发性肾小球肾炎之一,也是终末期肾病(end-stage renal disease,ESRD)的主要病因之一。IgAN的治疗仍然存在挑战,干预相关的危险因素,可延缓疾病的进展。近年发现,高尿酸血症是IgAN患者常见的临床特点,也是疾病进展的危险因素之一,但高尿酸血症与IgAN病理改变及疾病进展的关系仍不明确。因此,明确高尿酸血症与IgAN的关系具有重要的临床意义。本文从高尿酸血症在IgAN中的患病率、相关的发病机制与IgAN进展的关系及治疗靶点等方面进行了较为详尽的综述。展开更多
目的探讨尿IgG/尿肌酐(UGCR)、24 h尿蛋白定量(24 h UP)、尿蛋白定量/尿肌酐(UPCR)在预测IgA血管炎肾炎(IgAVN)患儿肾脏病理进展中的价值。方法选取2018年1月—2022年10月收治的IgAVN患儿234例,均采集24 h尿液标本检测24 h UP。根据肾...目的探讨尿IgG/尿肌酐(UGCR)、24 h尿蛋白定量(24 h UP)、尿蛋白定量/尿肌酐(UPCR)在预测IgA血管炎肾炎(IgAVN)患儿肾脏病理进展中的价值。方法选取2018年1月—2022年10月收治的IgAVN患儿234例,均采集24 h尿液标本检测24 h UP。根据肾小球损伤程度不同将IgAVN患儿分为Ⅱ级47例、Ⅲ级175例、Ⅳ级12例,分析UGCR、24 h UP、UPCR与肾脏病理分级的相关性,通过受试者工作特征曲线分析上述指标预测肾脏IgAVN病理分级进展为Ⅲ级及以上的价值。结果肾脏病理分级Ⅲ级、Ⅳ级患儿UGCR、24 h UP、UPCR水平高于Ⅱ级患儿(P<0.01)。UGCR、24 h UP、UPCR与病理分级呈正相关,但相关性较弱(r=0.500、0.451、0.356,P<0.01)。UGCR、24 h UP和UPCR对IgAVN患儿肾脏病理分级进展为Ⅲ级及以上均具有预测价值,且三者联合检测预测价值更高(曲线下面积>0.9,P<0.01)。结论UGCR联合24 h UP、UPCR对IgAVN肾脏病理分级进展为Ⅲ级及以上的预测价值更高。展开更多
Fabry disease (FD) clinical manifestations often start in childhood. Among the FD complications, renal failure causes significant morbidity and mortality. Early diagnosis and treatment of FD nephropathy in children ma...Fabry disease (FD) clinical manifestations often start in childhood. Among the FD complications, renal failure causes significant morbidity and mortality. Early diagnosis and treatment of FD nephropathy in children may be critical to preserve renal function. In proteinuric progressive nephropathies it has been described that pro-fibrotic miR-21, miR-192, and miR-433 families are activated and that anti-fibrotic miR-29 and miR-200 families are inhibited. Objective: Analyze urinary excretion of microRNAs related to renal fibrosis in FD patients with mild or absent nephropathy. Patients with confirmed diagnosis of FD under 18 years of age were compared with healthy subjects. Patients were classified into two groups: 1) Patients with urinary excretion profile of microRNAs indicative of renal fibrosis;and 2) Patients with urinary excretion profile of microRNAs not indicative of renal fibrosis. Results: 9 healthy subjects were enrolled in the study (18.66 ± 13.43 years), 4 males and 5 females. All of them presented normal eFGR without pathological albuminuria. FD population: 12 patients (10.33 ± 3.93 years) were studied, 5 males and 7 females. Patients presented 2 different genotypes: L415P (6 patients) and E398X (6 patients). The urinary excretion profile of microRNAs indicative of renal fibrosis was present in 4 patients (2 with L415P genotype and 2 with E398X genotype), all of them with a decreased of miR-29 and/or miR-200. No patient presented increased miR-21, miR-192 and/or miR-433. Decreased α-gal-A activity was the only variable associated with statistical significance (p ≤ 0.01) to urinary excretion profile of microRNA indicative of renal fibrosis. Conclusions: Young FD patients with classical mutations of GLA gene and mild or absent nephropathy could present a profile of urinary excretion of microRNAs indicative of renal fibrosis associated with decreased α-gal-A activity independently of the other variables. Our findings could suggest a regulation of microRNAs not mediated by TGF-β in FD nephropathy.展开更多
Background Very limited data are available on factors predictive of corticosteroids plus cyclophosphamide treatment efficacy on IgA nephropathy （IgAN）.The aim of the study was to research the clinical factors predic...Background Very limited data are available on factors predictive of corticosteroids plus cyclophosphamide treatment efficacy on IgA nephropathy （IgAN）.The aim of the study was to research the clinical factors predictive of treatment efficacy in IgAN.Methods One hundred and fifty-nine patients with IgAN （proteinuria ≥2 g/d and estimated glomerular filtration rate 30-89 ml·min-1·1.73 m-2） were treated with corticosteroids/cyclophosphamide followed by a 12-month follow-up.According to their response,these patients were divided into remission group （proteinuria ＜0.5 g/d） and non-remission group （proteinuria ≥0.5 g/d）,and their clinical data collected.Results In the present study,72.96％ of the individuals underwent a complete remission,and their response was related to baseline proteinuria,urinary osmotic pressure,and renal function （P ＜0.05）.Patients with baseline proteinuria more than 3 g/d,urinary osmotic pressure greater than 600 mOsm/L,and eGFR 60-89 ml·min-1·1.73 m-2 responded well to the combination of corticosteroids and cyclophosphamide （86.90％ vs.57.33％,P=0.000; 81.48％ vs.64.10％,P=0.014; 83.17％ vs.55.17％,P=0.000）.Conclusion The response to the combination of corticosteroids and cyclophosphamide might be well associated with baseline proteinuria,urinary osmotic pressure,and renal function in patients with IgAN.展开更多
文摘IgA肾病(immunoglobulin A nephropathy,IgAN)属于常见的原发性肾小球肾炎之一,也是终末期肾病(end-stage renal disease,ESRD)的主要病因之一。IgAN的治疗仍然存在挑战,干预相关的危险因素,可延缓疾病的进展。近年发现,高尿酸血症是IgAN患者常见的临床特点,也是疾病进展的危险因素之一,但高尿酸血症与IgAN病理改变及疾病进展的关系仍不明确。因此,明确高尿酸血症与IgAN的关系具有重要的临床意义。本文从高尿酸血症在IgAN中的患病率、相关的发病机制与IgAN进展的关系及治疗靶点等方面进行了较为详尽的综述。
文摘目的探讨尿IgG/尿肌酐(UGCR)、24 h尿蛋白定量(24 h UP)、尿蛋白定量/尿肌酐(UPCR)在预测IgA血管炎肾炎(IgAVN)患儿肾脏病理进展中的价值。方法选取2018年1月—2022年10月收治的IgAVN患儿234例,均采集24 h尿液标本检测24 h UP。根据肾小球损伤程度不同将IgAVN患儿分为Ⅱ级47例、Ⅲ级175例、Ⅳ级12例,分析UGCR、24 h UP、UPCR与肾脏病理分级的相关性,通过受试者工作特征曲线分析上述指标预测肾脏IgAVN病理分级进展为Ⅲ级及以上的价值。结果肾脏病理分级Ⅲ级、Ⅳ级患儿UGCR、24 h UP、UPCR水平高于Ⅱ级患儿(P<0.01)。UGCR、24 h UP、UPCR与病理分级呈正相关,但相关性较弱(r=0.500、0.451、0.356,P<0.01)。UGCR、24 h UP和UPCR对IgAVN患儿肾脏病理分级进展为Ⅲ级及以上均具有预测价值,且三者联合检测预测价值更高(曲线下面积>0.9,P<0.01)。结论UGCR联合24 h UP、UPCR对IgAVN肾脏病理分级进展为Ⅲ级及以上的预测价值更高。
文摘Fabry disease (FD) clinical manifestations often start in childhood. Among the FD complications, renal failure causes significant morbidity and mortality. Early diagnosis and treatment of FD nephropathy in children may be critical to preserve renal function. In proteinuric progressive nephropathies it has been described that pro-fibrotic miR-21, miR-192, and miR-433 families are activated and that anti-fibrotic miR-29 and miR-200 families are inhibited. Objective: Analyze urinary excretion of microRNAs related to renal fibrosis in FD patients with mild or absent nephropathy. Patients with confirmed diagnosis of FD under 18 years of age were compared with healthy subjects. Patients were classified into two groups: 1) Patients with urinary excretion profile of microRNAs indicative of renal fibrosis;and 2) Patients with urinary excretion profile of microRNAs not indicative of renal fibrosis. Results: 9 healthy subjects were enrolled in the study (18.66 ± 13.43 years), 4 males and 5 females. All of them presented normal eFGR without pathological albuminuria. FD population: 12 patients (10.33 ± 3.93 years) were studied, 5 males and 7 females. Patients presented 2 different genotypes: L415P (6 patients) and E398X (6 patients). The urinary excretion profile of microRNAs indicative of renal fibrosis was present in 4 patients (2 with L415P genotype and 2 with E398X genotype), all of them with a decreased of miR-29 and/or miR-200. No patient presented increased miR-21, miR-192 and/or miR-433. Decreased α-gal-A activity was the only variable associated with statistical significance (p ≤ 0.01) to urinary excretion profile of microRNA indicative of renal fibrosis. Conclusions: Young FD patients with classical mutations of GLA gene and mild or absent nephropathy could present a profile of urinary excretion of microRNAs indicative of renal fibrosis associated with decreased α-gal-A activity independently of the other variables. Our findings could suggest a regulation of microRNAs not mediated by TGF-β in FD nephropathy.
文摘Background Very limited data are available on factors predictive of corticosteroids plus cyclophosphamide treatment efficacy on IgA nephropathy （IgAN）.The aim of the study was to research the clinical factors predictive of treatment efficacy in IgAN.Methods One hundred and fifty-nine patients with IgAN （proteinuria ≥2 g/d and estimated glomerular filtration rate 30-89 ml·min-1·1.73 m-2） were treated with corticosteroids/cyclophosphamide followed by a 12-month follow-up.According to their response,these patients were divided into remission group （proteinuria ＜0.5 g/d） and non-remission group （proteinuria ≥0.5 g/d）,and their clinical data collected.Results In the present study,72.96％ of the individuals underwent a complete remission,and their response was related to baseline proteinuria,urinary osmotic pressure,and renal function （P ＜0.05）.Patients with baseline proteinuria more than 3 g/d,urinary osmotic pressure greater than 600 mOsm/L,and eGFR 60-89 ml·min-1·1.73 m-2 responded well to the combination of corticosteroids and cyclophosphamide （86.90％ vs.57.33％,P=0.000; 81.48％ vs.64.10％,P=0.014; 83.17％ vs.55.17％,P=0.000）.Conclusion The response to the combination of corticosteroids and cyclophosphamide might be well associated with baseline proteinuria,urinary osmotic pressure,and renal function in patients with IgAN.
