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先天性肾病综合征
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  congenital nephrotic syndrome
     NPHS1 mutations in a Chinese family with congenital nephrotic syndrome
     中国人先天性肾病综合征NPHS1基因突变
短句来源
     Objective Congenital nephrotic syndrome(CNS) is defined as heavy proteinuria or nephrotic syndrome occurring before 3 months of age.
     目的分析并确定一个中国先天性肾病综合征(CNS)家系NPHS1基因突变及特征。
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  “先天性肾病综合征”译为未确定词的双语例句
     The relationship between NPHS1 gene mutation and protein in lupus nephritis
     先天性肾病综合征基因突变与狼疮肾炎蛋白尿的关系探讨
短句来源
     Heavy proteinuria, a main characteristic of naphrotic syndrome, is caused by increasing permeability of glomerular filtrated barrier to plasma albumin.
     目的 nephrin是一种细胞黏附蛋白,其缺乏导致芬兰型先天性肾病综合征
短句来源
     It was reported that congenital nephritic syndrome of the Finnish type (CNF) was caused by the mutation in the NPHS1 gene. The mutation of NPHS1 gene induced nephrin disappeared from the slit diaphragm, destroyed the integrality of the glomerular capillary wall, and then leaded the occurrence of proteinuria, which suggesting an important role of nephrin in the development of proteinuria.
     研究发现,在芬兰型先天性肾病综合征中,NPHS1基因发生突变,导致其基因产物nephrin蛋白不能表达在裂孔隔膜上,破坏肾小球滤过屏障的完整性,引起大量蛋白尿的发生,由此提示nephrin在肾小球蛋白尿发生中的重要作用。
短句来源
     Objective:To study the cellular expression and distribution of V822M and C265R, two novel mutants of NPHS1. Methodology:Two mutants and a wild type of NPHS1 clones were constructed and were transfected to COS7cells.
     目的:研究NPHS1两种新突变编码蛋白在细胞内的分布与先天性肾病综合征发病机制的关系。
短句来源
     Conclusion:A mutant protein of nephrin is detained in endoplasmic reticulum and cannot reach cell membrane due to its misfolding.
     结论:突变的Nephrin蛋白由于错误折叠,不能由内质网被输送至细胞表面,这可能是先天性肾病综合征发病的机制之一。
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  相似匹配句对
     Das nephrotische Syndrom
     肾病综合征
短句来源
     Management of Idiopathic Nephrotic Syndrome in Children
     肾病综合征的治疗
短句来源
     NPHS1 mutations in a Chinese family with congenital nephrotic syndrome
     中国人先天性肾病综合征NPHS1基因突变
短句来源
     Congenital syphilis presenting with neprotic syndrome and leucocytoclastic vasculitis
     表现为肾病综合征和白细胞碎裂性血管炎的先天性梅毒
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     Congenital eyelid imbrication syndrome
     先天性眼睑重叠综合征
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  congenital nephrotic syndrome
Late ultrasonographic pattern in congenital nephrotic syndrome of Finnish type
      
Diagnostic sonographic changes in the kidneys of 20 infants with congenital nephrotic syndrome of the Finnish type
      
A renal ultrasonographic study was performed on 20 infants with congenital nephrotic syndrome of the Finnish type.
      
Thus, the sonographic follow-up is one of the main diagnostic indices f congenital nephrotic syndrome.
      
Peanut and Lotus tetragonolobus binding sites in human kidney from congenital nephrotic syndrome of finnish type
      
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1 cases infant with

对61例婴幼儿肾小球疾病患儿进行肾穿刺检查,其中急性肾炎4例,迁延性肾炎1例,无症状性蛋白尿或血尿3例,单纯性肾病45例,肾炎性肾病7例,先天性肾病综合征1例。病理类型:毛细血管增生性肾炎7例,微小病变5例,系膜增生性肾炎26例,IgA肾病5例,IgM肾肩10例,膜性肾病7例,局灶节段性肾小球硬化1例。完全经解49例(88.5%),部分缓解6例(9.8%),无效1例(1.6%).结果表明,婴幼儿肾小球疾病的病理类型以系膜增生性肾炎为主,微小病变只占8.2%。婴幼儿肾小球疾病的疗效较好。

Objective From January 1990 to December 2000 in the 281st Hospital of People′s Liberation Army, 170 attempts of percutaneous renal biopsy were performed in infant patients In this report, the clinic presentations and pathological features of glomerular diseases in infants were analyzed retrospectively, and pathological classification was compared between infant patients and adult patients. Method Of 170 infant patients, 127 were boys and 43 were girls The mean age at the renal biopsy was 31 2 months...

Objective From January 1990 to December 2000 in the 281st Hospital of People′s Liberation Army, 170 attempts of percutaneous renal biopsy were performed in infant patients In this report, the clinic presentations and pathological features of glomerular diseases in infants were analyzed retrospectively, and pathological classification was compared between infant patients and adult patients. Method Of 170 infant patients, 127 were boys and 43 were girls The mean age at the renal biopsy was 31 2 months (30 days to 36 months) The pathological classification was made according to the WHO criteria of 1982 and to the criteria of Pathological Coordinating Group of the Chinese Society of Nephrology in 1985 The clinical diagnosis was made according to the criteria of Collaboration Group of the Chinese Society of Pediatric Nephrology One hundred and fifty patients with infantile nephritic syndrome were divided into the simple nephrosis group ( n =133) and the nephritic nephrosis group ( n =17) All patients were treated with the combination of prednisone [1 5-2 mg/ (kg·d)], mustargen (0 06-0 09 mg/kg, once every other day), persantime, heparin and and Chinese herbs according to the syndrome type Result The primary glomerulonephritis accounted for 96 5% of the total renal biopsy samples, secondary glomerulonephritis 1 8%, and hereditary nephritis 1 8% The mesangial proliferative glomerulonephritis accounted for 40 9% of the primary glomerulonephritis, minimal change disease 22 6%, immunoglobulin M nephropathy 17 7%, endocapillary proliferative glomerulinephritis 5 5%, membranous nephropathy 4 9%, minor lesion nephropathy 3 7%, immunoglobulin A nephropathy 3 0%, focal segmental glomerulosclerosis 1 2% and C1q nephropathy 0 6% Two 2 patients were diagnosed to have Henoch Schonlein purpura nephritis and 1 patient to have drug induced renal injury Congenital nephrotic syndrome was found in 3 patients Among 150 patients with nephrotic syndrome, 133 had simple nephrosis, the remaining 17 patients had nephritic nephrosis Acute glomerulonephritis was diagnosed in 8 patients, persistent glomerulonephritis in 2 patients, and isolated hematuria or proteinuria in 4 patients The complete remission rate of the simple nephrosis group was 100 %, which was significantly higher than that of the nephritic nephrosis (64 8%,χ 2=28 325, P <0 01) The therapeutic effects of minimal change disease and minor lesion nephropathy and mesangial proliferative glomerulonephritis patients were significantly superior to those of mesangial hypercellularity, crescent, tuft adhesion, mesangial sclerosis,global sclerosis, tubular atrophy, and interstitial fibrosis patients (χ 2=51 45, P <0 01) Conclusion Primary glomerulonephritis dominated infantile glomerular diseases Mesangial proliferative glomerulonephritis and minimal change disease were the most common pathological type of the primary glomerulonephritis Nephrotic syndrome was the most common clinical type of the primary glomerulonephritis, among whom simple nephrosis was predominant Henoch Schonlein nephritis was the most common type of the secondary glomerulonephritis The common cause of the hereditary nephritis was congenital nephrotic syndrome, which was found only in the infants and newborns younger than 6 months The percutaneous renal biopsy is a useful diagnostic aid and can be used as a therapeutic guidance even in infantile patients with renal diseases

目的 探讨婴幼儿肾小球疾病的病理与临床特点。方法 对经肾活检确诊的 170例婴幼儿肾小球疾病进行分析。结果 原发性肾小球肾炎 16 4例 (占 96 5 % ) ,继发性肾小球肾炎 3例(1 8% ) ,遗传性肾病 3例 (1 8% )。原发性肾小球肾炎中系膜增生性肾炎 6 7例 (40 9% ) ,其他依次为微小病变 37例 (2 2 6 % ) ,IgM肾病 2 9例 (17 7% ) ,毛细血管内增生性肾炎 9例 (5 5 % ) ,膜性肾病8例 (4 9% ) ,轻微病变 6例 (3 7% ) ,IgA肾病 5例 (3 0 % ) ,局灶节段性肾小球硬化 2例 (1 2 % )和C1q肾病 1例 (0 6 % )。继发性肾小球肾炎中紫癜性肾炎 2例 ,药物性肾损害 1例。遗传性肾病中先天性肾病综合征 3例。原发性肾小球肾炎的临床类型肾病综合征 15 0例 (占 88 2 % ) ,其中单纯性肾病 133例 ,肾炎性肾病 17例。结论 婴幼儿肾小球疾病中原发性肾小球肾炎占绝大多数。系膜增生性肾炎和微小病变为最常见的病理类型。原发性肾小球肾炎的临床类型以肾病...

目的 探讨婴幼儿肾小球疾病的病理与临床特点。方法 对经肾活检确诊的 170例婴幼儿肾小球疾病进行分析。结果 原发性肾小球肾炎 16 4例 (占 96 5 % ) ,继发性肾小球肾炎 3例(1 8% ) ,遗传性肾病 3例 (1 8% )。原发性肾小球肾炎中系膜增生性肾炎 6 7例 (40 9% ) ,其他依次为微小病变 37例 (2 2 6 % ) ,IgM肾病 2 9例 (17 7% ) ,毛细血管内增生性肾炎 9例 (5 5 % ) ,膜性肾病8例 (4 9% ) ,轻微病变 6例 (3 7% ) ,IgA肾病 5例 (3 0 % ) ,局灶节段性肾小球硬化 2例 (1 2 % )和C1q肾病 1例 (0 6 % )。继发性肾小球肾炎中紫癜性肾炎 2例 ,药物性肾损害 1例。遗传性肾病中先天性肾病综合征 3例。原发性肾小球肾炎的临床类型肾病综合征 15 0例 (占 88 2 % ) ,其中单纯性肾病 133例 ,肾炎性肾病 17例。结论 婴幼儿肾小球疾病中原发性肾小球肾炎占绝大多数。系膜增生性肾炎和微小病变为最常见的病理类型。原发性肾小球肾炎的临床类型以肾病综合征为主 ,其中单纯性肾病占绝大多数。继发性肾小球肾炎主要为紫癜性肾炎。遗传性肾病以先天性肾病综合征多见 ,仅见于小于 6月的婴幼儿和新生儿。婴幼儿肾脏疾病经皮肾活检对患者的临床诊断和指导治疗是有益的

Objective Congenital nephrotic syndrome(CNS) is defined as heavy proteinuria or nephrotic syndrome occurring before 3 months of age.It is characterized by early onset,resistance to steroid therapy and progressing to end-stage renal disease(ESRD).In recent years,several genes associated with CNS have been identified,such as NPHS1,NPHS2 and WT1.The mutations of these genes have been identified in the patients with CNS in Finland,other European countries,North Africa,North America,and Asia,respectively.However,the...

Objective Congenital nephrotic syndrome(CNS) is defined as heavy proteinuria or nephrotic syndrome occurring before 3 months of age.It is characterized by early onset,resistance to steroid therapy and progressing to end-stage renal disease(ESRD).In recent years,several genes associated with CNS have been identified,such as NPHS1,NPHS2 and WT1.The mutations of these genes have been identified in the patients with CNS in Finland,other European countries,North Africa,North America,and Asia,respectively.However,the investigation of the above genes has not been performed in Chinese CNS patients.In this study, NPHS1 mutations in a Chinese family with CNS were detected and analyzed.Methods There were two CNS patients in the investigated family.The proband,a 45-day-old boy,was born at fullterm and weighed 2700 g at birth.The placenta weighed 450 g.At the age of 10 days,generalized edema,proteinuria,hypoproteinemia,and hypoalbuminemia were found without renal insufficiency.The proband′s sister,with the same phenotype and normal renal function,underwent renal biopsy at 5 years of age.Their parents and elder half-sister all had normal phenotypes.Genomic DNA samples were extracted from peripheral bloods of the proband,his family members and 50 unrelated,normal individuals.All 29 exons and exon-intron boundaries of NPHS1 were detected in the proband by polymerase chain reaction(PCR),direct DNA sequencing,and restriction enzyme analysis.Results Three heterozygous mutations of NPHS1,namely,G928A(D310N),1893-1900del 8(CGAAACCG),and G2869C(V957L)were identified in the proband.These mutations involved exons 8,14,and 21.The same genotype was found in the proband′s sister who had the same phenotype,but was not detected in proband′s elder half-sister who had normal phenotype.Fifty normal individuals had no these mutations.The proband′s mother with normal urinalysis had G928A(D310N)heterozygous mutation,and the father with normal urinalysis had two heterozygous mutations of 1893-1900del 8(CGAAACCG) and G2869C(V957L).At the same time,three types of single nucleotide polymorphisms(SNPs),E117K(rs3814995),S1105S(rs2071327),and IVS27+45c>t,were confirmed in the proband.Another variant,IVS8+68 a>g had also been found.Conclusion This is the first report about NPHS1 mutations in Chinese CNS kindred.These three heterozygous mutations of NPHS1 are novel genetic defects of CNS,which have not been described before.

目的分析并确定一个中国先天性肾病综合征(CNS)家系NPHS1基因突变及特征。方法对先证者及其家系成员采用聚合酶链反应(PCR)和DNA直接测序方法进行NPHS1基因突变检测,确定基因突变的位点。同时应用限制性内切酶酶切分析的方法,分析先证者及其家系成员和对照人群的基因组DNA,确定突变特征。结果在本家系的先证者发现同时存在NPHS1的G928A(D310N)、1893~1900del 8(CGAAACCG)和G2869C(V957L)的3个杂合突变。具有相同表型的姐姐(Ⅲ:11)与先证者的测序结果完全一致,而在表型正常的先证者的同父异母的大姐及对照样本均未发现这些突变。患儿母亲尿检正常,基因检测显示仅有第8外显子G928A突变;患儿父亲尿检也正常,基因检测显示仅存在第14外显子的1893~1900del 8和第21外显子的G2869C突变,没有第8外显子G928A突变。同时还在先证者发现了4种碱基变异:E117K(rs3814995)、S1105S(rs2071327)、IVS27+45 c>t和IVS8+68 a>g,经比对前3种均为单核苷酸多态性,位于内含子内的变异(IVS8+68 a>g)...

目的分析并确定一个中国先天性肾病综合征(CNS)家系NPHS1基因突变及特征。方法对先证者及其家系成员采用聚合酶链反应(PCR)和DNA直接测序方法进行NPHS1基因突变检测,确定基因突变的位点。同时应用限制性内切酶酶切分析的方法,分析先证者及其家系成员和对照人群的基因组DNA,确定突变特征。结果在本家系的先证者发现同时存在NPHS1的G928A(D310N)、1893~1900del 8(CGAAACCG)和G2869C(V957L)的3个杂合突变。具有相同表型的姐姐(Ⅲ:11)与先证者的测序结果完全一致,而在表型正常的先证者的同父异母的大姐及对照样本均未发现这些突变。患儿母亲尿检正常,基因检测显示仅有第8外显子G928A突变;患儿父亲尿检也正常,基因检测显示仅存在第14外显子的1893~1900del 8和第21外显子的G2869C突变,没有第8外显子G928A突变。同时还在先证者发现了4种碱基变异:E117K(rs3814995)、S1105S(rs2071327)、IVS27+45 c>t和IVS8+68 a>g,经比对前3种均为单核苷酸多态性,位于内含子内的变异(IVS8+68 a>g)意义有待进一步研究。结论首次发现中国人CNS存在NPHS1基因突变,并证实致病突变为国际首次报道的3个杂合突变。

 
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