Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conduct...Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic distribution of different subtypes’ glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone therapy (60 mg/m<sup>2</sup>/day) referred to nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients separately. This study investigated prognostic effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59 (29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ± 4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of 102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children with iSRNS secondary to MCD are more likely to achieve remission and have better long term prognostic value (P 0.00). Focal segmental glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive therapy (P 0.04). Conclusions: This study defines the true spectrum of clinicohistopathology patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the underlying histopathology patterns which have been earned by adequate renal biopsy.展开更多
Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevert...Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevertheless,the correlation between TLR genes and the progression of INS has not yet been clarified.The present study aimed to investigate the association of single-nucleotide polymorphisms(SNPs)in TLR2,TLR4,and TLR9 with susceptibility to INS as well as the clinical phenotyping of steroid responsiveness in Chinese children with INS.Methods A total of 183 pediatric inpatients with INS were included and given standard steroid therapy.Based on their clinical response to steroids,the patients were classified into three groups:steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).A total of 100 healthy children were employed as controls.The blood genome DNA was extracted from each participant.Six SNPs(rs11536889,rs1927914,rs7869402,rs11536891,rs352140,and rs3804099)in TLR2,TLR4,and TLR9 were selected and detected by multiplex polymerase chain reaction with next-generation sequencing to assess TLR gene polymorphisms.Results Among the 183 patients with INS,89(48.6%)had SSNS,73(39.9%)had SDNS,and 21(11.5%)had SRNS.No significant difference was found in the genotype distribution between healthy children and patients with INS.However,the genotype and allele frequencies of TLR4 rs7869402 were significantly different between SRNS and SSNS.Compared with patients with the C allele and CC genotype,patients with the T allele and CT genotype had an increased risk of SRNS.Conclusion TLR4 rs7869402 affected the steroid response in Chinese children with INS.It might be a predictor for the early detection of SRNS in this population.展开更多
目的观察比较环磷酰胺(CTX)、环孢素A(CsA)治疗儿童激素耐药型原发性肾病综合征(SRNS)诱导缓解及维持缓解的疗效。方法回顾性研究应用CTX或CsA治疗的51例SRNS患者的临床资料。结果治疗3个月时,CsA组完全缓解率(18/25 vs 9/26)及有效率(2...目的观察比较环磷酰胺(CTX)、环孢素A(CsA)治疗儿童激素耐药型原发性肾病综合征(SRNS)诱导缓解及维持缓解的疗效。方法回顾性研究应用CTX或CsA治疗的51例SRNS患者的临床资料。结果治疗3个月时,CsA组完全缓解率(18/25 vs 9/26)及有效率(21/25 vs 14/26)均高于CTX组。CTX组与CsA组最终的完全缓解率(16/26 vs 19/25)及有效率(17/26 vs 21/25)差异无显著性。CsA组尿蛋白阴转时间(中位数表示)短于CTX组(21天vs 75天)。病理为局灶节段性肾小球硬化(FSGS)治疗8周时,CsA组尿蛋白阴转比例(8/10 vs 1/8)、有效率(8/10 vs 2/8)均高于CTX组。CTX组维持缓解时间(以中位数表示)长于CsA组(16.0个月vs 10.0个月)。随访1年时CTX组维持缓解者所占比例高于CsA组(12/16 vs7/17)。结论 CTX与CsA治疗SRNS的总有效率相当,但CsA起效更快,尤其对FSGS效果显著;而CTX维持缓解的作用优于前者。展开更多
文摘Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic distribution of different subtypes’ glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone therapy (60 mg/m<sup>2</sup>/day) referred to nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients separately. This study investigated prognostic effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59 (29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ± 4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of 102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children with iSRNS secondary to MCD are more likely to achieve remission and have better long term prognostic value (P 0.00). Focal segmental glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive therapy (P 0.04). Conclusions: This study defines the true spectrum of clinicohistopathology patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the underlying histopathology patterns which have been earned by adequate renal biopsy.
基金This study was funded by the Science and Technology Projects of Zhejiang Province(No.LGC21H200004)the Key Research and Development Plan of Zhejiang Province(No.2019C03028)the Medical Scientific Projects from Health Department of Zhejiang Province(No.2018KY455)。
文摘Objective Idiopathic nephrotic syndrome(INS)is the most common glomerular disease in children.Toll-like receptors(TLRs)have been reported to be associated with response to steroid treatment in children with INS.Nevertheless,the correlation between TLR genes and the progression of INS has not yet been clarified.The present study aimed to investigate the association of single-nucleotide polymorphisms(SNPs)in TLR2,TLR4,and TLR9 with susceptibility to INS as well as the clinical phenotyping of steroid responsiveness in Chinese children with INS.Methods A total of 183 pediatric inpatients with INS were included and given standard steroid therapy.Based on their clinical response to steroids,the patients were classified into three groups:steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).A total of 100 healthy children were employed as controls.The blood genome DNA was extracted from each participant.Six SNPs(rs11536889,rs1927914,rs7869402,rs11536891,rs352140,and rs3804099)in TLR2,TLR4,and TLR9 were selected and detected by multiplex polymerase chain reaction with next-generation sequencing to assess TLR gene polymorphisms.Results Among the 183 patients with INS,89(48.6%)had SSNS,73(39.9%)had SDNS,and 21(11.5%)had SRNS.No significant difference was found in the genotype distribution between healthy children and patients with INS.However,the genotype and allele frequencies of TLR4 rs7869402 were significantly different between SRNS and SSNS.Compared with patients with the C allele and CC genotype,patients with the T allele and CT genotype had an increased risk of SRNS.Conclusion TLR4 rs7869402 affected the steroid response in Chinese children with INS.It might be a predictor for the early detection of SRNS in this population.
文摘目的观察比较环磷酰胺(CTX)、环孢素A(CsA)治疗儿童激素耐药型原发性肾病综合征(SRNS)诱导缓解及维持缓解的疗效。方法回顾性研究应用CTX或CsA治疗的51例SRNS患者的临床资料。结果治疗3个月时,CsA组完全缓解率(18/25 vs 9/26)及有效率(21/25 vs 14/26)均高于CTX组。CTX组与CsA组最终的完全缓解率(16/26 vs 19/25)及有效率(17/26 vs 21/25)差异无显著性。CsA组尿蛋白阴转时间(中位数表示)短于CTX组(21天vs 75天)。病理为局灶节段性肾小球硬化(FSGS)治疗8周时,CsA组尿蛋白阴转比例(8/10 vs 1/8)、有效率(8/10 vs 2/8)均高于CTX组。CTX组维持缓解时间(以中位数表示)长于CsA组(16.0个月vs 10.0个月)。随访1年时CTX组维持缓解者所占比例高于CsA组(12/16 vs7/17)。结论 CTX与CsA治疗SRNS的总有效率相当,但CsA起效更快,尤其对FSGS效果显著;而CTX维持缓解的作用优于前者。
