肾病综合征患儿细胞免疫和细胞因子的变化及其意义

目的探讨肾病综合征(NS)患儿细胞免疫功能和IL-1、IL-6、IL-8及TNFα与NS发病机制的关系.方法应用流式细胞术和酶联免疫吸附(ELISA)方法,对23例NS患儿在活动期和缓解期(21例)以及20例正常儿童外周血T细胞亚群以及血清和外周血单个核细胞(PBMC)培养上清诱生IL-1、IL-6、IL-8、TNFα水平进行测定.结果NS活动期血清和PBMC上清液IL-1、IL-6、IL-8和TNFα水平明显高于缓解期和对照组,IL-6和TNFα增高显著,缓解期上述细胞因子仍高于对照组.外周血总CD3+、CD4+、CD4+/CD8+细胞比值、CD(16+56)+细胞均降低,CD8+细胞增高.相关分析发现NS活动期IL-1、IL-6、IL-8和TNFα与CD3+、CD4+、CD4+/CD8+和CD(16+56)+细胞呈负相关,与CD8+细胞呈正相关.结论NS患儿在活动期多种细胞因子失调,T细胞亚群的比例结构发生变化,细胞免疫功能异常及细胞因子网络失调在NS发病中起一定作用.     

肾病综合征甲状腺功能的研究

目的　探讨肾病综合征 (NS)患儿发作期和缓解期的甲状腺功能改变。方法　用放免法对 1 6例原发性NS在发作期和缓解期行血、尿T3、T4 、FT3、FT4 及血TSH水平测定。结果　发作期血清T3、T4 、FT3、FT4均低于正常 ,较缓解期及对照组显著下降 (P <0 .0 0 1 ) ,尿T3、T4 、FT3、FT4 较缓解期和对照组显著增多 (P <0 .0 0 0 1 )。结论　NS患儿发作期甲状腺功能低下 ,缓解后恢复正常 ,其主要原因可能是尿中大量蛋白丢失的结果     

原发性肾病综合征患儿血甲状腺激素水平与血、尿蛋白关系的观察

目的观察原发性肾病综合征(INS)患儿血甲状腺激素(TH)水平与血白蛋白、尿蛋白的关系.方法以放射免疫法检测18例INS患儿和18例正常儿(对照组)血总T3、T4(TT3、TT4)和游离T3、T4(FT3、FT4)水平,并测定24h尿蛋白定量(UPQ)及血白蛋白(Salb)水平.结果INS组血TT3、TT4、FT3、FT4水平分别为(0.76±0.22)nmol/L、(47.52±18.28)nmol/L、(2.28±1.01)pmol/L、(10.47±3.92)pmol/L,与正常对照组比较差异显著;INS组血TT4、FT3、FT4与Salb水平呈正相关,与UPQ水平无相关性;血TT3与Salb、UPQ水平无相关性.结论INS患儿存在甲状腺功能低下,这一状态与血Salb水平降低有一定的关系.     

单纯性肾病患儿甲状腺功能测定的临床意义

目的：　探讨单纯性肾病血清甲状腺素水平的变化。方法：　用双抗体放免法检测肾病患儿组与健康对照组各 20例血清甲状腺素,比较两组变化的差异。结果：　对照组血清甲状腺素水平正常,肾病组血清T3 ,T4 ,TSH有不同程度的变化 ,分别为 (1.0± 0 .5)nmol/L ,(15.5±32.4)nmol/L,(20.2±13.2)mU/L,两组差异有显著性意义(P<0.01)。结论：　检测单纯性肾病患儿血清甲状腺素水平,对增进治疗效果,估计和改善预后有比较重要的意义。     

甲状腺功能异常患儿血清瘦素水平与甲状腺激素的相关性研究

目的　研究甲状腺功能异常 [原发性甲状腺功能减退 (甲减 )和原发性甲状腺功能亢进 (甲亢 ) ]患儿血清瘦素 (leptin)水平变化 ,探讨血清瘦素与甲状腺功能的关系。方法　采用放射免疫法分别检测 2 0例甲减患儿、17例甲亢患儿和 2 5例健康儿童血清瘦素水平 ,同时采用微粒子化学发光免疫分析法检测血清游离三碘甲状腺原氨酸 (FT3 )、游离甲状腺素 (FT4)、促甲状腺素 (TSH)等指标。结果　甲低组治疗前血清瘦素水平显著低于正常对照组 (P <0 .0 0 1) ,经药物治疗甲状腺功能恢复至正常后 ,其血清瘦素浓度上升至正常水平 ;甲亢组治疗前后血清瘦素水平与正常对照组相比 ,差异无显著性 (P >0 .0 5 )。结论　甲状腺激素对血清瘦素的分泌具有促进作用     

肾病综合征患儿尿视黄醇结合蛋白测定及分析

临床资料 :1999年 8月至 2 0 0 0年5月本院收治的 5 2例肾病综合征 (NS)患儿 ,诊断符合 1981年全国小儿肾脏病科研协作组制定的标准。单纯性肾病组 2 8例 ,肾炎性肾病组 2 4例。其中 ,极期组 17例 ,初次发病 ,未用激素治疗 ;未缓解组 13例 ,应用激素 4～ 8周 ,尿蛋白仍 (+)～ (+++) ;缓解组2 2例 ,经治疗尿蛋白转阴 2周以上。正常对照组 2 3例 ,系健康查体儿童。方法 :取晨尿 10mL ,采用双抗体夹心ELESA法测定尿视黄醇结合蛋白(RBP) ,试剂购自上海德波生物技术公司 ,同时测定尿 β2 微球蛋白 (β2 MG)、尿白蛋白 (Alb)和尿IgG。统…     

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目的探讨心脏疾患及合并充血性心力衰竭(CHF)患儿心衰时,血清甲状腺激素(TH)水平的变化规律及临床意义。方法对2003-09—2006-09山西省阳泉市第一人民医院收治的116例心脏疾病患儿分为2组,心脏病合并CHF组82例,心脏病未合并CHF组34例,以28例健康体检儿童为对照组,均做如下检测及分析:(1)检测CHF患儿血清TH水平,包括三碘甲状腺原氨酸(T3)、游离T3(FT3)、甲状腺素(T4)、游离T4(FT4)、反T3(rT3)及促甲状腺激素(TSH),三组间进行比较。(2)以血清TH水平与心功能进行相关分析。(3)动态观察CHF患儿的血清TH水平,探索其变化规律。结果(1)CHF组与N-CHF组及正常对照组比,血清T3、FT3及FT4均显著降低(P<0.01),rT3显著升高(P<0.01)。(2)随心功能的下降,T3、FT3、T4、FT4渐降低,rT3渐升高。TSH与心功能变化无相关性。(3)CHF组经治疗后血清TH多恢复(P<0.01),顽固性心衰组则无恢复(P>0.05)。结论(1)小儿CHF伴有血清TH的改变,以T3、FT3及FT4的降低,rT3的升高为主,其改变程度与心功能状态具有相关性。(2)随着心功能的改善,TH的改变多渐恢复,顽固性心衰患儿则无恢复,提示TH水平持续不恢复者预后较差。     

激素敏感型肾病综合征患儿血管内皮细胞生长因子的观察及分析

目的　研究激素敏感型肾病综合征 (SSNS)患儿活动期和缓解期血清血管内皮细胞生长因子 (VEGF)水平及临床意义。方法　应用酶联免疫吸附测定 (ELISA)方法检测 2 2例SSNS活动期和缓解期的VEGF水平 ,以 18例年龄、性别匹配的正常儿童作对照 ;同时测定血尿素氮 (BUN)、肌酐 (Cr)和 2 4h尿蛋白定量 ,并分析与VEGF的关系。结果　SSNS患儿活动期血VEGF水平 [(64 3 5± 16 5 9)ng/L]高于缓解期 [(3 5 17± 13 67)ng/L]和正常对照 [(3 3 4± 5 9)ng/L] ,P <0 0 5 ;缓解期与正常对照组相比 ,无明显差异 ,P >0 0 5 ;活动期VEGF水平与BUN、Cr无相关 ,而与 2 4h尿蛋白定量呈正相关。结论　SSNS患儿活动期VEGF水平升高 ,并与蛋白尿呈正相关 ;激素治疗可降低VEGF水平 ,提示VEGF参与SSNS的发病     

单纯性肾病综合征患儿T淋巴细胞亚群及体液免疫变化

目的　探讨单纯性肾病综合征 (SNS)患儿T淋巴细胞及体液免疫变化。方法　应用流式细胞仪和特定蛋白分析仪对SNS患儿 1 4例活动期和缓解期 (1 2例 )及 1 5例正常儿童外周血T淋巴细胞亚群及血清免疫球蛋白 (Ig)进行检测。结果　SNS患儿活动期组外周血总CD3 、CD4、CD8细胞低于对照组 (t=2 .1 4 ,2 .86　P均 <0 .0 5)。活动期组CD4 、CD8 细胞明显低于缓解期组 (t=2 .49,2 .2 9　P均 <0 .0 5)。活动期组CD4 /CD8 明显低于缓解期组和对照组 (t =2 .75 ,4.1 0　P均<0 .0 5)活动期组和缓解期组血清IgG与对照组比较均有明显降低 (t=2 .75 ,7.0 1　P <0 .0 5 ,0 .0 1 )。结论　SNS患儿在活动期周围血T淋巴细胞数量异常 ,且T淋巴细胞亚群间比例失调 ,且伴体液免疫异常。     

尿足细胞标志蛋白检测对肾脏疾病的诊断价值

目的 探讨尿液中足细胞检测在肾病综合征(NS)和紫癜性肾炎(HSPN)中的临床诊断价值.方法 采用足细胞特定标志蛋白podocalyxin(PCX) ELISA检测试剂盒对89例儿童清晨首次尿标本进行检测.其中75例采自本院儿科住院患儿,包括NS活动期患儿21例、NS缓解期患儿14例、HSPN缓解期患儿14例、HSPN活动期患儿16例、轻型过敏性紫癜(HSP)患儿10例;另选14例体检健康儿童作为健康对照组.采用SPSS 13.0软件进行统计分析.结果 1.NS和HSPN疾病活动期及缓解期患儿尿PCX水平明显高于健康对照组(P≤0.009);轻型HSP患儿与健康对照组比较无显著性差异(P=0.754).2.NS活动期患儿尿PCX水平与缓解期患儿比较差异显著[(0.593±0.271) μg/L vs (0.162±0.093) μg/L P=0];HSPN活动期患儿尿PCX水平与缓解期比较具有显著性差异[(1.822±1.342) μg/L vs (0.236±0.141) μg/L P=0.004];轻型HSP患儿尿PCX水平为(0.089±0.061) μg/L,与HSPN活动期及缓解期患儿比较均有显著性差异(Pa＜0.05).3.NS活动期患儿尿PCX水平与24 h尿蛋白排泄量呈正相关(r=0.785 P=0.01).结论 NS与HSPN患儿发病过程中存在足细胞损伤与脱落,且疾病活动期足细胞脱落明显增加.尿足细胞检测对预测急性期肾小球损伤程度具有重要的临床意义.     

特发性肾病综合征患儿血清游离甲状腺激素检测的临床意义

探索特发性肾病综合症（ＩＮＳ）患儿血清游离甲状腺激素（ＴＨ）、甲状腺激素结合球蛋白（ＴＢＧ）的变化情况及其变化的机理和临床意义。对１６例ＩＮＳ患儿及１５例正常儿童对照,用放免法测Ｔ３、Ｔ４、ＦＴ３、ＦＴ４,化学发光酶免疫分析法测ＴＳＨ、ＴＢＧ,放射配基单点饱和饱和结合分析法测糖皮质激素受体（ＧＣＲ）。结果：①ＩＮＳ患儿Ｔ３、Ｔ４、ＦＴ３、ＦＴ４均低于对照组,ＴＳＨ升高,差异非常显著。②ＴＢＧ降低,ＴＢＧ与ＴＨ呈正相关。③ＩＮＳ患儿ＧＣＲ升高,ＴＨ与ＧＣＲ未见相关性。提示对糖皮质激素（ＧＣ）治疗敏感的ＩＮＳ患儿,虽然其ＴＨ是降低的,但其ＧＣＲ是升高的。     

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??Objective To observe the effect of mizoribine??MZR?? in the treatment of children with frequently relapsing primary nephrotic syndrome. Methods Totally 21 children with frequently relapsing nephrotic syndrome were treated with mizoribine?? and another 24 children were included as the control group. Observe the changes of relapse number??dosage of prednisone?? glomerular filtration rate?? serum albumin?? urine protein?? uric acid and other indicators. Results After the treatment with mizoribine??the recurrence rate and the effective dosage of prednisone decreased?? serum albumin level rose?? and primary urinary protein decreased?? but there were no significant changes in glomerular filtration rate and uric acid level. Conclusion The mizoribine is effective in treatment of children with frequently relapsing primery nephrotic syndrome .     

Clinical profile of children with nephrotic syndrome not on glucorticoid therapy, but presenting with infection

OBJECTIVE: This is a prospective, hospital based study over a period of one year, to examine the clinical profile of nephrotic children not on glucocorticoid therapy, but presenting with infection. METHODS: Sixty-eight children with 76 episodes of nephrotic syndrome were enrolled during the study year. They were examined for evidence of infection using clinical and investigative criteria. The children were monitored daily for proteinuria and improvement of infection. After the infection was controlled, prednisolone therapy was started in those who were not already in remission. RESULTS: Of the 76 episodes, eight were excluded from analysis as they had developed infection while they were on glucocorticoid therapy. Of the remaining 68 nephrotic episodes in 60 children, there was evidence of infection in 57 episodes (83.8%). Upper respiratory infection was the most common (28.0%) followed by urinary tract infection (22.8%), peritonitis (15.8%), pneumonia (14.0%), acute invasive diarrhoea (10.5%) and empyema (5.3%). Children with infection had significantly lower serum albumin and higher serum cholesterol compared to non-infected children. Of the 57 episodes with infection, remission occurred with control of infection alone in nine episodes (15.8%) and glucocorticoid therapy was not required. CONCLUSION: Infection is widely prevalent among Indian children presenting with episodes of nephrotic syndrome, even when they are not on glucocorticoid therapy. Some children may go into remission by control of infection alone. Among the infections, upper respiratory and urinary tract infections are the most common.     

ALBUMIN METABOLISM AND GASTROINTESTINAL PROTEIN LOSS IN CHILDREN WITH NEPHROTIC SYNDROME

In order to assess the significance of gastrointestinal protein loss as a cause of plasma protein hypercatabolism in patients with nephrotic syndrome, seven nephrotic children were studied simultaneously with radioiodine labelled albumin and a test substance, ^51,Fe-labelled iron dextran, for evaluation of gastrointestinal protein loss. All the patients displayed a pronounced hypercatabolism of albumin, which could not be accounted for by urinary protein loss. A slightly increased gastrointestinal protein loss was demonstrated in 4 patients, whereas, in 3 patients, the faecal loss of macromolecules was within normal limits. The relation between protein catabolism and faecal loss of macromolecules was compared in the nephrotic children and in 20 patients with protein-losing enteropathies. It is concluded that gastrointestinal protein loss is insignificant in the nephrotic syndrome, and unable to account for the marked endogenous hypercatabolism of albumin. Albumin synthesis was normal or somewhat increased. An abnormally high fraction of the total albumin mass was present in the intravascular space in 4 of the 7 children.     

儿童肾病综合征合并嗜酸性粒细胞增多的临床分析

目的 探讨儿童肾病综合征（NS）合并嗜酸性粒细胞增多的临床特点。方法 回顾性分析合并嗜酸性粒细胞增多的18例次NS患儿（15例,其中3例于第2次复发时也有嗜酸性粒细胞增多）的临床表现、实验室检查和治疗转归。结果 嗜酸性粒细胞轻度增多16例次（89%）、中度增多1例次（6%）、重度增多1例次（6%）。12例次（67%）的嗜酸性粒细胞增多发生在冬春季。9例次（50%）合并感染性疾病：4例肺炎（其中2例为支原体肺炎）、3例EB病毒感染、1例疑似蛲虫感染、1例链球菌感染;5例次（28%）合并过敏性疾病,其中2例表现为荨麻疹,2例表现为过敏性鼻炎,1例表现为湿疹。嗜酸性粒细胞计数与尿蛋白定量、血白蛋白、胆固醇水平均无明显相关性（P > 0.05）。8例次初发NS患儿,激素治疗后尿蛋白4周内转阴;10例次复发NS患儿,9例次患儿调整激素治疗后尿蛋白亦转阴。1例次（嗜酸性粒细胞中度增多）合并过敏性鼻炎的NS患儿予以抗过敏等对症治疗后症状缓解、尿蛋白转阴,未恢复激素治疗,嗜酸性粒细胞计数降至轻度增多;余17例次患儿嗜酸性粒细胞均恢复正常。结论 NS合并嗜酸性粒细胞增多多发生于冬春季节,常合并感染或过敏性疾病,嗜酸性粒细胞计数与尿蛋白定量、血白蛋白、胆固醇水平均无明显相关性。     

Anion gap in nephrotic syndrome

Low anion gap occurs in a variety of disorders including hypoproteinemia. We evaluated anion gap in 28 children - 18 boys and 10 girls with minimal change nephrotic syndrome during 55 separate episodes of exacerbations. To assess the contribution of serum total proteins, albumin, cholesterol and immunoglobulin levels to low anion gap, a coefficient of correlation was calculated. Anion gap was significantly low (p less than 0.01) during exacerbations in nephrotic. The low anion gap correlated with low serum total proteins (less than 4 g/dl) and serum albumin levels (less than 1.5 g/dl) but not to serum cholesterol or immunoglobulin levels. Low total serum protein at patients blood pH, contributed fewer negative charges to anion gap thereby lowering the anion gap. The major cause of low anion gap in nephrotic syndrome is hypoproteinemia especially hypoalbuminemia.     

Toll样受体4在原发性肾病综合征患儿肾组织及外周血中表达的意义

目的探讨Toll样受体4(TLR4)在原发性肾病综合征(INS)患儿肾组织及外周血中的表达及意义。方法采集2015年10月至2018年6月在新乡医学院第一附属医院确诊为INS的78例患儿肾活检组织及21例儿童正常肾组织(对照组1),应用免疫组织化学方法检测标本中TLR4表达水平,比较INS不同肾脏病理类型、不同临床分型中TLR4表达水平的差异,分析其与24 h尿蛋白、血清清蛋白的相关性;应用酶联免疫吸附试验(ELISA)检测INS患儿治疗前(活动期)及治疗后(缓解期)和23例健康儿童(对照组2)外周血中TLR4表达水平,比较INS不同肾脏病理类型、不同临床分型患儿血清中TLR4表达水平的差异,分析其与24 h尿蛋白、血清清蛋白的相关性;对INS患儿肾小管中TLR4的表达水平与血清TLR4的表达水平进行相关性分析。结果1.与正常肾组织TLR4水平[(0.93±0.26)%]比较,TLR4在各型INS患儿肾小球及肾间质的表达[系膜增生性肾小球肾炎(MsPNG)型:(0.93±0.21)%、局灶节段性肾小球硬化(FSGS)型:(1.02±0.25)%、膜性肾病(MN)型(1.03±0.09)%、微小病变(MCD)型(1.02±0.27)%]差异无统计学意义(F=0.741,P=0.562);而肾小管TLR4的表达水平明显增高[MCD型:(82.94±4.62)%、MN型:(63.54±1.98)%、MsPGN型:(42.32±2.97)%、FSGS型:(22.60±2.07)%],差异有统计学意义(F=1929.842,P<0.01),其中MCD型INS患儿肾小管TLR4表达水平较MN型、MsPGN型、FSGS型明显增高,差异均有统计学意义(均P<0.01);肾小管TLR4在临床分型为激素敏感型肾病综合征(SSNS型)肾脏组织中表达最高,在激素耐药型肾病综合征(SRNS)型表达最低,差异有统计学意义(F=220.951,P<0.01)。2.活动期INS患儿血清TLR4表达[MsPNG型:(143.36±12.99)ng/L、FSGS型(75.94±7.29)ng/L、MN型(210.22±14.66)ng/L、MCD型(283.93±21.58)ng/L]显著高于缓解期患儿[MsPNG型:(29.51±4.93)ng/L、FSGS型(15.66±3.78)ng/L、MN型(45.40±5.73)ng/L、MCD型(62.29±7.90)ng/L]及对照组2儿童[(0.69±0.33)ng/L],差异均有统计学意义(均P<0.01);且缓解期INS患儿血清TLR4的表达水平高于对照组2儿童,差异有统计学意义(F=286.287,P<0.01)。活动期及缓解期INS患儿血清TLR4的表达水平均以MCD型最高,其次为MN型,而FSGS型最低;血清TLR4在临床分型为SSNS型表达最高,SRNS型表达最低,差异有统计学意义(F=147.438,P<0.01)。3.INS患儿肾小管中TLR4的表达水平[(62.82±20.94)%]与其活动期血清中TLR4的表达水平[(213.26±73.33)ng/L]呈正相关(r=0.852,P<0.05);INS患儿肾小管及活动期血清TLR4的表达水平均与24 h尿蛋白水平[(123.05±33.55)mg/kg]均呈正相关(r=0.401、0.427,均P<0.05),与血清清蛋白水平[(19.54±3.55)g/L]均呈负相关(r=-0.602、-0.617,均P<0.05)。结论TLR4在INS患儿肾小管及血清中表达增高,且表达水平可能与不同肾脏病理类型及临床分型有关,与疾病活动具有相关性。     

儿童原发性肾病综合征血清酰化刺激蛋白及补体C3水平的变化

目的 观察原发性肾病综合征(PNS)患儿血清酰化刺激蛋白(ASP)、补体C3水平的变化以及与血脂的关系.方法 分别检测35例未经治疗的PNS患儿(蛋白尿组)和25例缓解期PNS患儿(缓解组)空腹血清ASP、C3、白蛋白及血脂水平,并与35例体重指数相匹配的正常儿童(对照组)进行比较.结果 蛋白尿组血清ASP水平(101...     

Enalapril and hydroxyurea therapy for children with sickle nephropathy

Proteinuria in children with sickle cell anemia (SCA) is an early sign of sickle nephropathy, and portends the development of nephrotic syndrome and chronic renal failure. Enalapril has been shown to reduce proteinuria in adult patients with SCA, but the potential benefits of hydroxyurea in this clinical setting have not been reported. A single institution retrospective analysis was performed. Children with sickle nephropathy were identified, and the laboratory effects of enalapril and hydroxyurea therapy were evaluated in children with substantial proteinuria. Three children developed proteinuria at 8 +/- 1 years of age. Pre-treatment laboratory studies included a low serum albumin (2.8 +/- 0.8 g/dl) and a highly elevated urine protein/creatinine ratio (6.9 +/- 3.7, normal <0.2). Enalapril treatment for 3.0 +/- 1.3 years normalized serum albumin (3.9 +/- 0.3 g/dl) without significant changes in serum potassium, serum creatinine, or systolic blood pressure. However, urine protein/creatinine remained elevated in the nephrotic range (1.6 +/- 0.7). The addition of hydroxyurea therapy for 3.5 +/- 1.2 years increased fetal hemoglobin levels (7.0 +/- 3.6% to 21.0 +/- 3.2%) and was associated with a near-normal urine protein/creatinine ratio (0.5 +/- 0.1). Enalapril therapy for children with sickle nephropathy reduces urinary protein excretion and normalizes serum albumin. Hydroxyurea therapy may further normalize the urine protein/creatinine ratio. Combination therapy should be tested prospectively in children with sickle nephropathy.