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     

泼尼松对肾病综合征患儿骨合成分子标志物的影响

目的　探讨糖皮质激素对肾病综合征 (NS)征患儿骨合成分子标志物的影响。方法　以I型前胶原羧基端前肽 (PICP)、骨钙蛋白 (BGP)及碱性磷酸酶 (AKP)为参数 ,对单纯性NS患儿泼尼松治疗前及治疗 3～4周后骨合成状况进行探讨。结果　泼尼松治疗后PICP、BGP、AKP与治疗前相比有显著差异 ,治疗后低于治疗前水平 [PICP(P <0 .0 1)、BGP(P <0 .0 1)、AKP(P <0 .0 1) ]。结论　大剂量糖皮质激素治疗可完全抑制NS患儿的骨合成功能     

肾病综合征患儿免疫球蛋白检测与应用激素的探讨

目的检测原发性肾病患儿IgG、IgA、IgM、IgE、C3水平,探讨肾病患儿对肾上腺皮质激素(泼尼松)的敏感性,了解肾病的发病机制,指导临床治疗。方法分别采用散射比浊法检测血清IgG、IgA、IgM、C3及ELISA法检测血清IgE。结果原发性肾病患儿与正常对照组比较,血清IgA、C3无显著差异,肾病患儿血清IgG水平明显低于正常儿童,IgM、IgE明显高于正常儿童,单纯型与肾炎型肾病结果一致。单纯型与肾炎型肾病对激素治疗敏感性比较有非常显著性差异(x2=18.48 P< 0.005);原发性肾病患儿血清IgE升高组与IgE不升高组对激素治疗的敏感性比较有非常显著性差异(x2=12.46 P< 0.005)。结论原发性肾病综合征患儿存在免疫球蛋白合成异常,可能与患儿体液免疫紊乱有关。而激素治疗敏感性与肾病综合征临床分型及IgE水平高低有关。     

肾病综合征患儿的血清载脂蛋白的变化及其意义

为探讨原发性肾病综合征（简称肾病）病程中的脂代谢紊乱的特征及其意义，对３７例肾病患儿在疾病不同时期的血清载脂蛋白（ａｐｏ）浓度进行了检测。发现：活动性肾病患儿血清ａｐｏＡⅠ（１０８６±１７８ｍｇ／Ｌ）、ａｐｏＡⅡ（２５９±６７ｍｇ／Ｌ）浓度较正常对照组无差别，ａｐｏＢ１００（１６３６±４２２ｍｇ／Ｌ）、ａｐｏＣⅡ（８９±３１ｍｇ／Ｌ）、ａｐｏＣⅢ（２３１±８０ｍｇ／Ｌ）浓度升高（Ｐ＜０．０５）；在近期缓解患儿，血清ａｐｏＡⅠ（１３９２±１８５ｍｇ／Ｌ）、ａｐｏＡⅡ（３６５±７４ｍｇ／Ｌ）浓度上升，ａｐｏＢ１００（１４５０±３６５ｍｇ／Ｌ）、ａｐｏＣⅡ（６９±２５ｍｇ／Ｌ）、ａｐｏＣⅢ（２０２±６８ｍｇ／Ｌ）浓度仍高；而在长期缓解患儿，各项ａｐｏ水平与对照组比较差异无显著意义。结果表明，肾病患儿在活动期及近期缓解时存在显著的血清ａｐｏ异常，病情长期缓解后，ａｐｏ紊乱情况逐渐改善，以至正常。     

肾病综合征患儿的钙、维生素D及骨代谢变化

由于肾病综合征（ＮＳ）的病理生理改变，可致体内钙、维生素Ｄ（ＶＤ）、甲状旁腺功能及骨代谢等一系列内分泌代谢的异常。超生理剂量的糖皮质激素的长期应用又可加重以上变化，严重者可致病理性骨折。近几年来，随着维生素Ｄ代谢产物、甲状旁腺激素及骨代谢指标等新的检测技术的建立，为ＮＳ患者的ＶＤ、甲状旁腺功能及骨代谢的研究及骨病的早期诊断开辟了新的途径。由于活性ＶＤ及新型ＶＤ同系物（ＯＣＴ）的问世，为肾性骨病的防治提供了一定的应用前景。     

小儿激素抵抗型肾病综合征诊治中的一些问题

肾上腺皮质激素(下简称激素)用于治疗小儿原发性肾病综合征(NS)治疗已约半个世纪,在大多数患儿中激素可迅速诱导NS缓解,极大地改善了NS的预后;但部分患儿呈激素抵抗(steroid resistant),这一问题是目前小儿NS治疗中的难点之,是改善NS预后的关键,受到临床工作者的极大关注.今仅就其诊治中近年的一些进展概述如下.     

CD+4T淋巴细胞在激素敏感型肾病综合征发病中的作用研究

目的　明确激素敏感型肾病综合征T淋巴细胞致病因子的亚群来源 ,探讨CD 4T淋巴细胞在本病发病中的作用。方法　采用间接免疫荧光染色法测定肾病综合征患儿外周血CD 4、CD 8T细胞百分率 ,以及CD 4/CD 8比值 ;磺柳酸比浊法测定经大鼠尾静脉注射抗体结合 补体介导溶解法分离的患儿CD 4和CD 8T淋巴细胞培养上清液后的大鼠尿蛋白含量 ,电镜观察其肾小球超微结构与基底膜上阴离子位点改变。结果　 (1)肾病综合征活动期患儿外周血CD 4T淋巴细胞百分率为(33 4± 2 4) % ,与正常儿童比较差异无显著性 (P >0 0 5 ) ;CD 8T淋巴细胞百分率为 (15 5±4 5 ) % ,比正常儿显著降低 (P <0 0 1) ;CD 4/CD 8比值为 (2 2± 0 3) % ,比正常儿显著升高(P <0 0 1)。(2 )注射患儿CD 4T淋巴细胞培养上清液后 ,大鼠尿蛋白排泌量在 0～ 8h时间段由注射前的 (3 9± 0 6 )mg/ 2 4h显著增加到 (13 1± 4 3)mg/ 2 4h(P <0 0 0 1)。注射患儿CD 8T淋巴细胞培养上清液后 ,大鼠尿蛋白排泌量无明显变化 (P >0 0 5 )。蛋白尿大鼠出现肾小球上皮细胞足突融合、基底膜上阴离子位点显著减少。结论　注射患儿CD 4T淋巴细胞培养上清液可使大鼠出现一过性蛋白尿 ;CD 4T淋巴细胞功能紊乱 ,分泌致病因子是激素敏感型肾病综合     

生长和骨代谢的生化标志

成骨细胞分泌的骨钙素是特异的骨蛋白，血清骨钙素水平为骨转播儿骨形成的特殊标志。血清总碱性磷酸酶，骨特异性碱性磷酸酶活性测定亦代表骨的形成。吡哆酚和脱氧吡哆酚是主要存在软骨和骨骼中的交联氨基酸。     

原发性肾病综合征患儿血浆脂质及脂蛋白的变化

为了解小儿原发性肾病综合征（ＮＳ）时脂代谢的特点，观察了２０例原发性单纯型ＮＳ患儿的血脂、脂蛋白及载脂蛋白的变化。结果：（１）全部ＮＳ患儿血浆总胆固醇（ＴＣ）、低密度脂蛋白胆固醇（ＬＤＬ－Ｃ）和脂蛋白（ａ）［Ｌｐ（ａ）］均明显升高；兼有ＴＣ、甘油三酯（ＴＧ）升高者，其极低密度脂蛋白胆固醇明显升高，高密度脂蛋白胆固醇明显降低。（２）ＮＳ患儿２４小时尿蛋白定量与血浆ＴＣ、ＴＧ和ＬＤＬ－Ｃ呈正相关（Ｐ＜０．０１）。提示：（１）ＮＳ患儿存在脂代谢异常，兼有ＴＣ、ＴＧ同时升高者，伴有更严重的脂蛋白及载脂蛋白的紊乱，存在着多种致动脉硬化及肾损伤的因素。（２）ＮＳ患儿脂代谢异常的程度与蛋白尿严重程度高度正相关。     

原发肾病综合征患儿的血清脂质水平

为了解原发性肾病综合征（简称肾病）患儿的血清脂质变化情况，采用酶比色等方法，对２３例肾病患儿的血脂水平进行了测定，结果表明，原发性肾病患儿血清总胆固醇（ＴＣ）、甘油三酯（ＴＧ）、低密度脂蛋白胆固醇（ＬＤＬ－Ｃ）、载脂蛋白（ａｐｏ）Ｂ１００以及ＬＤＬ－Ｃ与高密度脂蛋白胆固醇（ＨＤＬ－Ｃ）的比值升高，血清ＨＤＬ－Ｃ及ａｐｏＡＩ与对照组差异不明显，单纯性肾病者的血ＴＣ、ＬＤＬ－Ｃ及ａｐｏＢ１００均较肾炎性肾病者高，且复发者的血脂异常较初发者更为严重，患儿的血脂异常与血清白蛋白浓度负相关（Ｐ＜０．０５），但与２４小时尿蛋白无明显关系。建议应加强对肾病高脂血症的随访及防治，尤单纯性肾病及频复发者更应被重视。     

IgE in patients with glomerulonephritis and minimal-change nephrotic syndrome

Serum levels of IgE were studied in 30 children with minimal-change nephrotic syndrome and 32 children with mesangioproliferative glomerulonephritis during different stages of the disease and treatment. In addition, tissue obtained by renal biopsy was investigated by immunofluorescence histology; no deposits of IgE could be found. The serum IgE levels, however, were increased, particularly in patients with minimal-change nephrotic syndrome. It is concluded that IgE does not play a pathogenic role in the development of the renal disease, but that increased IgE levels are an indication of a disturbance of the immune system.     

儿童原发性肾病综合征载脂蛋白E 基因检测及临床意义

目的　研究原发性肾病综合征 (INS)患儿血浆载脂蛋白E(ApoE)基因多态性分布及对血脂代谢的影响。方法　选择 2 0 0 0年 1月至 2 0 0 3年 9月潍坊市人民医院收治的 5 2例INS患儿 ,并选择 6 9名健康儿童作为对照组 ,测定其血清胆固醇 (TC)、甘油三酯 (TG)、高密度脂蛋白 (HDL)、低密度脂蛋白 (LDL)、载脂蛋白AI及载脂蛋白B10 0 (ApoAI,ApoB10 0 )水平 ,用聚合酶链反应 限制性片段长度多态性 (PCR RFLP)检测ApoE基因型。结果　与对照组相比 ,INS患儿血清TC、TG、LDL、HDL、ApoAI和ApoB10 0水平增高 (P <0 0 1) ;其Apoε等位基因频率分布不均 ,ε2、ε3、ε4等位基因携带者的TC、TG、ApoB10 0水平差异有显著性意义 (P <0 0 5 )。与ε3等位基因携带者相比 ,ε2携带者TG水平较高 ,LDL水平较低 (P <0 0 5 ) ;ε4携带者TC水平较高 ,TG水平较低 (P <0 0 5 )。ε2、ε3、ε4等位基因与TC、LDL浓度呈正相关 (P <0 0 5 )。难治性肾病综合征 (RNS)患儿Apoε2等位基因频率较对照组显著增高 (P <0 0 5 )。结论　INS患儿Apoε等位基因频率分布与对照组无差异 ,RNS患儿Apoε2等位基因频率显著增高 ,脂质代谢紊乱持续存在 ,易发生肾脏损害 ,应及早加用降血脂药物治疗。     

Final height in children with steroid-sensitive nephrotic syndrome

BACKGROUND: Growth retardation following steroid treatment in children is a major problem. Reduction of steroid dose has been tried using immunosuppressive agents such as cyclosporine A or mizoribine in children with frequently relapsing nephrotic syndrome. Few reports concerning final height in steroid-sensitive nephrotic syndrome (SSNS) are available. METHOD: Patients who developed SSNS before 15 years of age and reached their final height were retrospectively studied by standard deviation score (SDS) of height and target height calculated by their parental height. RESULTS: A total of 34 patients were evaluated for their final height. The mean age at onset of SSNS was 8.0 years and the mean age at last follow up was 21.6 years. In total, 22 patients had frequent relapses and were treated with cyclophosphamide, mizoribine or cyclosporin A. All patients had normal renal function at the last evaluation. The mean final height was 168 cm in males and 155 cm in females. The mean height SDS was 0.37 at the time of onset and was -0.43 when they reached their final height (P = 0.0001). The final height was a mean of 2.5 cm below target height and was significantly lower than their siblings (P = 0.007). Final height of two boys who continued to have frequent relapses during puberty and were not treated with cyclosporin A was 146 and 150 cm. CONCLUSION: Final height in children with SSNS was slightly affected by steroid treatment and two patients had severe growth retardation.     

The validity of biochemical markers in metabolic bone disease in preterm infants: a systematic review

To establish the validity of biochemical markers of metabolic bone disease (MBD) in preterm infants. CONCLUSION: There is insufficient evidence that any of the frequently used serum measurements are valid biochemical markers of MBD in preterm infants. Increased urinary calcium concentration may be a valid biochemical marker, but more research is necessary to confirm this.     

Cortisone cataract in children with nephrotic syndrome

In a group of 16 children with idiopathic nephrotic syndrome treated with corticosteroids for longer than 12 months, 9 developed a posterior subcapsular cataract (PSC). No correlation between the frequency of PSC and the duration of treatment and the total dose of treatment with steroids was demonstrable. However, the patients with PSC had received considerably higher average daily doses than those without PSC. Two patients with normal ophthalmologic findings at the end of treatment showed PSC 6 and 9 months later respectively. Only one patient acquired a signficant impairement of visus.     

Collagen-derived markers of bone metabolism in osteogenesis imperfecta

Markers of bone formation [C-terminal and N-terminal propeptides of procollagen I (PICP, PINP), osteocalcin and alkaline phosphatase] and bone resorption [C-terminal cross-linked telopeptide of collagen I (ICTP) and hydroxypyridinium cross-links, pyridinoline (Pyr) and deoxypyridinoline (Dpyr)] were measured in 78 osteogenesis imperfecta (OI) patients to investigate bone metabolism in vivo and relate marker concentrations to phenotype and in vitro collagen I defects, as shown by sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE). PICP and PINP were generally low, and the serum levels were lower in all children and adults with mild OI and a quantitative collagen defect than in patients with severe OI and a qualitative collagen I defect. ICTP, Pyr and Dpyr were generally normal or reduced, but elevated in severely affected adults with a qualitative collagen I defect. The in vivo findings correlated with in vitro results of collagen I SDS-PAGE. Bone turnover is reduced in OI children and mildly affected OI adults, whereas bone resorption is elevated in severely affected adults. These findings may prove helpful for diagnosis and decision-making regarding therapy in OI.     

原发性肾病综合征患儿淋巴细胞免疫功能的变化

为探讨原发性肾病综合征（ＩＮＳ）患儿淋巴细胞免疫功能的动态变化，分别采用噻唑蓝（ＭＴＴ）比色等方法，对２７例ＩＮＳ活动期患儿和其中１５例缓解期患儿的外周血淋巴细胞（ＰＢＬ）的增殖应答效应、白细胞介素２（ＩＬ－２）及其相应受体的表达能力和白细胞介素６（ＩＬ－６）的产生水平进行了检测。结果表明，ＩＮＳ活动期患儿ＰＢＬ的增殖应答能力比对照组和缓解组减低，并与Ｔ淋巴细胞亚群ＣＤ４／ＣＤ８比值呈正相关；活化的淋巴细胞ＩＬ－２受体表达和ＩＬ－２产生能力低下；而血浆和细胞培养上清液中可溶性ＩＬ－２受体水平升高。同时淋巴细胞培养上清液中ＩＬ－６含量增高。结果提示，ＩＮＳ患儿细胞免疫功能减低，影响体液免疫，激发其他细胞因子的产生，参与ＩＮＳ免疫病理过程的发生、发展。