以肾病综合征为表现的非IgA系膜增殖性肾炎HLA－DR基因频率分布

以肾病综合征为表现的非ＩｇＡ系膜增殖性肾炎ＨＬＡ一ＤＲ基因频率分布陈香美，柏立群，周柱亮，汪建国ＨＬＡ－ＤＲＧＥＮＥＦＲＥＱＵＥＮＣＩＥＳＩＮＡＤＵＬＴＮＥＰＨＲＯＴＩＣＳＹＮＤＲＯＭＥＰＡＴＩＥＮＴＳＷＩＴＨＮＯＮ－ＩＧＡＭＥＳＡＮＧＩＡＬＰＲＯＬ...     

IgA肾病的远期预后

ＩｇＡ肾病的远期预后［日］小山哲夫…／／医学　－１９９３．１６７（３）：－１６６１９６８年Ｂｅｒｇｅｒ首先报导ＩｇＡ肾病，患者肾小球系膜有弥漫性颗粒状ＩｇＡ沉积，临床呈轻度蛋白尿和持续性血尿，病理学以肾小球系膜增殖性肾炎为主。认为其原因是由于某种免疫...     

增殖性肾炎患者肾小球细胞胰岛素样生长因子—1的表达及其意义

目的　探讨人类增殖性肾炎（ＰＧＮ） 肾小球中胰岛素样生长因子１（ＩＧＦ１） 的表达及其意义。方法　采用原位杂交、免疫组织化学方法检测肾活检组织中ＩＧＦ１ ｍＲＮＡ及其蛋白质表达，并分析其与患者肾小球内细胞外基质（ＥＣＭ） 增生的关系。结果　增殖性肾炎组中ＩＧＦ１ 的表达水平显著高于非增殖性肾炎组和正常组（ Ｐ＜０－０１），ＩＧＦ１ 表达水平与ＥＣＭ 增生程度呈正相关。结论　肾小球局部ＩＧＦ１ 基因异常表达参与了ＰＧＮ的发病机制。     

系膜增生性肾炎患者尿微量白蛋白、IgG和视黄醇结合蛋白的变化

系膜增生性肾炎患者尿微量白蛋白、ＩｇＧ和视黄醇结合蛋白的变化周红卫孙安远杨桢华系膜增生性肾炎（ＭｓＰＧＮ）的病因目前尚未完全阐明，故人们对其预后便格外关注。我们应用ＥＬＩＳＡ检测７４例ＭｓＰＧＮ患者尿微量白蛋白（ＭＡＬｂ）、ＩｇＧ、黄醇结合蛋白（ＲＢ...     

IgA肾病患者尿白介素6检测的意义

我们用尿白介素６（ＩＬ－６）依赖细胞株７ＴＤ１细胞，测定了２１例原发性ＩｇＡ肾病患者尿和血清ＩＬ－６活性水平，并分析了ＩＬ－６活性水平与ＩｇＡＮ临床及病理改变的联系。结果：ＩｇＡＮ患者尿及血清ＩＬ－６与活性均增高，（活性增高者分别占４６．７％及２５％），尿ＩＬ－６与血清ＩＬ－６水平无平行关系，尿ＩＬ－６活性水平与ＩｇＡＮ严重蛋白尿的发生以及肾小球病变＊节段肾小球硬化、新月体、系膜扩张等）和小管一间     

大鼠阳离子化牛血清白蛋白肾炎肾脏前列腺素来源的探讨

目的 探讨大鼠阳离子化牛血清白蛋白（Ｃ－ＢＳＡ）肾炎中肾脏前列腺素来源。方法 应用羊抗大鼠血小板血清ＩｇＧ，对大鼠Ｃ－ＢＳＡ肾炎模型进行干扰。结果 实验组大鼠肾皮质ＰＧＥ２、ＰＧＩ２、ＴＸＡ２均减少，肾小球病理损害减轻，尿蛋白减少。结论 血小板参与Ｃ－ＢＳＡ肾炎病理过程以及肾脏前列腺素部分来源于血小板。     

系膜增殖性肾炎患者肾小球细胞IL-6、GP130和JunB mRNA表达及其意义

目的探讨白细胞介素６（ＩＬ－６）信号传递过程中某些成份（ＧＰ１３０、ＪｕｎＢ）在ＭｅｓＰＧＮ分子发病机制的作用。方法用原位杂交技术观察了ＭｅｓＰＧＮ患者肾活检组织肾小球细胞中ＩＬ－６、ＧＰ１３０、ＪｕｎＢｍＲＮＡ表达，并分析了其与患者肾小球系膜扩张、系膜细胞增殖程度、尿蛋白排泄量之间的关系。结果ＩＬ－６、ＧＰ１３０、ＪｕｎＢｍＲＮＡ表达分别是７．１±０．８、８．６±０．９、６．９±０．８，明显高于正常对照组（Ｐ＜０．０１），且相互间呈正相关；ＩＬ－６、ＧＰ１３０基因表达量与系膜扩张、尿蛋白排泄量呈正相关，中度系膜增殖性肾炎组、尿蛋白＞３．５克／日组，ＩＬ－６，ＧＰ１３０基因表达量明显高于轻度系膜增殖性肾炎组与尿蛋白＜３．５克／日组。结论ＩＬ－６及其信息传递中的ＧＰ１３０、ＪｕｎＢ基因异常表达在ＭｅｓＰＧＮ分子发病机制起一定作用。     

非胰岛素依赖型糖尿病肾病的临床研究

本文测定了２７例正常白蛋白尿、１２例早期糖尿病肾病和１２例临床糖尿病肾病的ＮＩＤＤＭ患者的肾血流动力学及肾脏体积，结果表明在ＮＩＤＤＭ正常白蛋白尿组病程ＧＦＲ及肾脏体积均明显高于对照组，临床糖尿病肾组ＧＦＲ、ＥＲＰＦ明显下降，早期糖尿病肾病组ＧＦＲ、ＥＲＰＦ与对照组无养成差异，但肾脏体积明显高于对照组。同时本文亦测定了尿白蛋白、ＩｇＧ、ＮＡＧ、ＴＨＰ排泄率及ＣＣｒ在糖尿病肾病各期的变化并探讨其各指     

体外免疫吸附对狼疮肾炎的治疗和保护作用

目的　观察体外免疫吸附（ＥＩＡ） 对狼疮肾炎（ＬＮ） 的治疗和肾脏保护作用。方法　比较树脂型ＥＩＡ、大剂量激素和ＣＴＸ联合治疗（ 简称ＥＩＡ组）与大剂量激素和ＣＴＸ治疗（ 简称对照组） 血ＩｇＧ、ＡＮＡ、ｄｓＤＮＡ、Ｃ３、２４ 小时尿蛋白、ＢＵＮ、Ｓｃｒ 及其他临床指标的变化。结果　治疗２ 周时,ＥＩＡ组血ＩｇＧ、ＡＮＡ、ｄｓＤＮＡ显著低于对照组［ 两组分别为ＩｇＧ（１０－０３ ±２－９１） 、（１３－５４ ±３－０５）ｇ／Ｌ,ＡＮＡ（１４－００ ±１３－１４）、（３１－８２ ±３０－０２）ｔｉｔｅｒ－ １,ｄｓＤＮＡ（８－３０ ±４－８３） 、（２７－００ ±１８－５１） ％］ 。Ｃ３ 显著回升［ 两组分别为（５８７－３±９７－０３） 、（４８１－５±１３２－０４）ｍｇ／Ｌ］,２４ 小时尿蛋白、ＢＵＮ、Ｓｃｒ 显著低于对照组［ 两组分别为２４ 小时尿蛋白（２－２８±２－０７） 、（５－９８ ±３－３４）ｇ／２４ｈ,ＢＵＮ（９－１７ ±２－８３）、（１５－２９ ±５－３８）ｍｍｏｌ／Ｌ、Ｓｃｒ（１５６－７５ ±８９－３３）、（２４７－１４±７４－６９）μｍｏｌ／Ｌ） 。治疗４ 周时,ＥＩＡ 组血ＩｇＧ、ｄｓＤＮＡ 仍低于对照组［ 两组分别为Ｉｇ     

乙型肝炎病毒感染与肾小球肾炎

目的研究乙型肝炎病毒（ＨＢＶ）感染与肾小球肾炎，尤其是与ＩｇＡ肾病（ＩｇＡＮ）、狼疮肾炎（ＬＮ）间的关系，并初步观察α干扰素（αＩＦＮ）对成人乙肝相关性肾炎（ＨＢＶＧＮ）的疗效。方法对１５７例肾活检标本采用免疫组织化学的方法检测肾组织中ＨＢＡｇ，配对比较３２例ＩｇＡＮ，３０例ＬＮ中ＨＢＡｇ阳性组与阴性组肾组织病理改变，和４２例ＧＮ中ＨＢｃＡｇ阳性组与阴性组对激素治疗的反应，并随访３例ＨＢＶＧＮ经αＩＦＮ治疗的临床演变。结果ＩｇＡＮ中ＨＢｃＡｇ阳性组肾小管及间质病变程度均较阴性组严重（Ｐ＜００５）；ＬＮ中ＨＢｃＡｇ阳性组肾小球硬化及间质炎症程度较阴性组显著（Ｐ＜００５）；ＨＢｃＡｇ阳性组对激素治疗的反应较阴性组差（Ｐ＜００５）；３例ＨＢＶＧＮ经αＩＦＮ治疗后临床症状缓解。结论肾小球肾炎部分病例起病与ＨＢＶ感染有关，ＩｇＡＮ、ＬＮ与ＨＢＶ感染有相关性，αＩＦＮ对成人ＨＢＶＧＮ可能有良好的治疗作用。     

Contribution of macromolecular IgA1 to IgA abnormality in IgA nephropathy

To evaluate the contribution of macromolecular IgA₁ to IgA abnormality in childhood IgA nephropathy, serum samples from 29 healthy children and 26 patients with IgA nephropathy in different age-groups (7–9, 10–12, and 13–15 years) were each separated by sucrose density gradient ultracentrifugation and assayed for IgA₁ using an enzyme-linked immunosorbent assay. IgA₁ in fraction I (sedimentation coefficient >11.4s) was significantly greater in patients 7–15 years of age (median 36.3–57.0 mg/dl) than in the age-matched controls (median 8.8–10.4 mg/dl). IgA₁ in fraction II (11.4–9.3s) was significantly greater in patients 10–15 years of age (median 46.7–52.6 mg/dl) than in the controls (median 27.8–35.5 mg/dl), and IgA₁ in fraction III (<9.3s) was significantly greater in patients 13–15 years of age (median 156.9 mg/dl) than in the controls (median 120.7 mg/dl). The ratio of IgA₁ in fractions I–III was higher in the patients of each age-group (median 0.233–0.314) than in the controls (median 0.067–0.082), while the ratio of IgA₁ in fractions II–III was not significantly high in patients 7–12 years old (median 0.268 to 0.318) compared with the controls (median 0.182–0.264). Thus, IgA abnormality in childhood IgA nephropathy would be better represented by an increase in macromolecular IgA₁ of >11.4s than by an increase in IgA₁ in fractions of 11.4–9.3s or <9.3s. Received: 8 December 1999 / Revised: 2 March 2000 / Accepted: 2 May 2000     

IgA glycosylation and IgA immune complexes in the pathogenesis of IgA nephropathy

Circulating immune complexes containing aberrantly glycosylated IgA1 play a pivotal role in the pathogenesis of IgA nephropathy (IgAN). A portion of IgA1 secreted by IgA1-producing cells in patients with IgAN is galactose-deficient and consequently recognized by anti-glycan IgG or IgA1 antibodies. Some of the resultant immune complexes in the circulation escape normal clearance mechanisms, deposit in the renal mesangium, and induce glomerular injury. Recent studies of the origin of these aberrant molecules, their glycosylation profiles, and mechanisms of biosynthesis have provided new insight into the autoimmune nature of the pathogenesis of this common renal disease. An imbalance in the activities of the pertinent glycosyltransferases in the IgA1-producing cells favors production of molecules with galactose-deficient O-linked glycans at specific sites in the hinge region of the alpha heavy chains. By using sophisticated analytic methods, it may be possible to define biomarkers for diagnostic purposes and identify new therapeutic targets for a future disease-specific therapy.     

Role of macromolecular IgA in IgA nephropathy

Primary IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis, leading to progressive renal failure in almost one third of the patients. The disease is characterized by mesangial deposits of IgA. The pathogenesis of IgAN remains incompletely understood. The basic abnormality of this disorder lies within the IgA immune system rather than in the kidney. Elevated levels of IgA and IgA-containing complexes are found in sera of most patients with IgAN, but increased levels alone are not sufficient to develop IgAN. Therefore abnormal physicochemical properties of circulating IgA, such as size, charge, and glycosylation may play a role. This is supported by the presence of altered glycosylation of serum and mesangial IgA in patients with IgAN. Although the precise origin and nature of the mesangial IgA deposits are still uncertain, they contain at least in part macromolecular IgA, which may be derived from circulating IgA-containing complexes. Recently, novel insights have been obtained in the molecular composition of circulating high-molecular-weight IgA, which might include complexes with underglycosylated IgA1 and IgA-CD89 complexes. In this review various aspects of macromolecular IgA in relation to IgAN will be discussed.     

IgA glomerulonephritis

Renal biopsy specimens from 204 patients with glomerulonephritis or nephrotic syndrome have been studied. In ten of the patients not suffering from acute poststreptococcal glomerulonephritis, systemic lupus erythematosus or Schönlein-Henoch syndrome, diffuse, selective mesangial IgA deposition was observed. Clinically, persistent microscopic haematuria, mild proteinuria and, except in one patient, normal renal function were found. Light microscopically the histological picture was dominated by a diffuse or focal increase in volume of the mesangial matrix, and mild mesangial cell proliferation. Exceptionally, there was also crescent formation. Immunofluorescence revealed large IgA, IgG and C3 deposits, as well as small IgM and fibrinogen deposits in the mesangial glomeruli. The authors' assumption that immunocomplexes containing a secretory component might be implicated in the pathomechanism of Berger's disease, could not be proved.On the basis of a lecture given at the XXIIIrd Itinerary Congress of the Transdanubian Section of the Association of Hungarian Internists, held at Gyr on 18th June, 1976. Supported by the Scientific Research Council, Ministry of Health, Hungary (3-18-0304-03-2/H).