肾活检免疫病理呈“满堂亮”现象临床意义的探讨(附50例病例分析）

目的 探讨肾活检免疫病理“满堂亮”（指肾组织免疫荧光染色IgG、IgA、IgM、C3、C1q同时阳性）的现象与临床肾脏疾病关系。方法 对1984－1999年有完整临床资料及肾活检免疫荧光呈“满堂亮”的50例闰例进行分析。结果 50例“满堂亮”的诊断为：（1）狼疮性肾炎21例,其病理改变Ⅱ型（系膜增生型）4例,Ⅲ型（局灶节段型）5段,Ⅳ型（弥漫增殖型）9例,Ⅴ型（膜型）3例。（2）乙型肝炎病毒相关肾炎13例,其病理为轻系膜增生型4例;膜性肾病7例;膜增生型肾小球肾炎2例。（3）过敏性紫瘢性肾炎（均为肾病型）9例。（4）IgA肾病6例。（5）抗中性粒细胞胞浆抗体阳性的急进性肾小球肾炎1例。结论 （1）“满堂亮”现象可见于多种肾小球疾病,最多见于狼疮性肾炎,是狼疮性肾炎最有特片性的免疫病理改变。（2）“满堂亮”现象也可出现于乙型肝炎病毒相关肾炎。（3）对呈‘满堂亮‘而临床诊断为非狼疮性肾炎的病例应追踪观察,尤应注意其与狼疮性肾炎的密切关系。以便发现些延迟出现狼疮血清学阳性反应的和（或）狼疮临床表现的狼疮患者。     

IMMUNOFLUORESCENT AND MORPHOLOGICAL STUDIES IN CONGENITAL NEPHROTIC SYNDROME1

Two kidneys from patients with congenital nephrotic syndrome obtained at nephrectomy at the age of 3 and 23 months, were investigated for possible immunopathological mechanisms for the kidney injury. Immuno-fluorescent staining with anti IgG, IgM, IgA and /SIC-globulins did not show attachment of immunolglobulins or complement to the glomeruli. Staining was found in some tubular casts and occasionally in tubular epithelium, as well. Electron microscopic studies of the glomeruli corroborated the idea that immunocomplexes are not conglomerated in the glomeruli because of the absence of typical deposits, which are usually found in association with positive immunofluorescent staining in various glomerulonephritides. Kidneys were also eluted with acidic buffer in order to remove antibodies from immunocomplexes. The eluates were analyzed for their immunoglobulin content and used for indirect immunofluorescent staining. The content of IgG of the kidney eluates was low and no difference of eluted IgG between the nephrotic kidneys and the control was apparent. No binding of immunoglobulin with structures of normal kidney could be seen in the indirect immunofluorescent study. Our results corroborate the previous results (10) that immunopathogenesis is not essential in the congenital nephrotic syndrome.     

LOCALIZATION OF PLASMA PROTEINS IN KIDNEYS OF CHILDREN WITH DIFFUSE NEPHROPATHIES, STUDIED BY AN INDIRECT IMMUNOFLUORESCENT TECHNIQUE

Preliminary report of the results obtained with an indirect immunofluorescent technique applied to the study of renal biopsy specimens. Depositions of IgG, IgA, IgM, complement, fibrinogen and albumin were investigated in glomeruli of children with diffuse nephropathies and in adult controls. The localization of the protein precipitates was confirmed by comparing the immunofluorescent picture with the results of the P.A.S.M./ H.E. staining of the same sections.     

Immunofluorescence Studies of Lung Tissue in Cystic Fibrosis

Previous studies have suggested that immune mechanisms contribute to lung injury in cystic fibrosis (CF); however, there have been no comprehensive studies of immunofluorescent staining patterns in CF lung tissue. We performed immunofluorescence (IF) studies for immunoglobulins, C3, and fibrinogen on autopsy frozen lung tissue from 21 CF patients. Results were compared with lung findings in patients without CF. In CF-derived lung tissue fibrinogen was ubiquitous along the alveolar wall, alveolar space, and interstitium. Free immunoglobulin G (IgG) and IgA coated the alveolar surface segmentally in 14 and 6 cases, respectively. Unequivocal interstitial deposits were infrequent and IgM was present in blood vessels in one patient only. Intra-alveolar and interstitial inflammatory cells demonstrated cytoplasmic IgG, IgA, and IgM, respectively, in 18, 14, and 6 patients. C3 was seen only segmentally along the alveolar wall in two patients and in blood vessels in one. Antinuclear antibody (ANA) staining of interstitial cells for C3 and immunoglobulins was seen in five patients, four of whom had interstitial pneumonitis. Insignificant amounts of alveolar or interstitial fibrinogen and immunoglobulins in inflammatory cells were seen in controls in the absence of lung inflammation. The IF patterns were similar in the inflammatory lesions of CF and control specimens.

The IF patterns observed in CF lung tissue are consistent with nonspecific vascular leakage and chronic inflammation with little evidence of immune complex deposition in the interstitium or blood vessels. This study confirms previous reports of ANA activity in CF patients, although the significance of this finding is unknown.     

Selective enhancement of human IgE production in vitro by synergy of pokeweed mitogen and mercuric chloride

Selective stimulation of IgE production by pokeweed mitogen (PWM) plus mercuric chloride (HgCl₂) was further investigated in peripheral blood mononuclear cells (MNC) from non-atopic donors, who are high and low responders to PWM stimulation with respect to IgG, IgM and IgA production, and from various patients with elevated serum IgE levels. Non-atopic high responders showed a strong selective increase in IgE plaque forming cells (PFC) without changes in IgG, IgM and IgA PFC, whereas low responders showed a slight increase in IgE PFC only when B cells were co-cultured with mitomycin C (MMC)-treated T cells. In patients with elevated serum IgE levels, PWM plus HgCl₂ caused a variable selective increase in IgE PFC, however, there was no correlation between IgE PFC production and serum IgE levels.     

Serum immunoglobulins in children perinatally exposed to human immunodeficiency virus

OBJECTIVE: Hypergammaglobulinemia is an early manifestation of perinatal HIV infection. Our objective was to analyze the differences in serum immunoglobulin levels between infected and seroreverter children and their association with clinical outcome. METHODS: We carried out a historical prospective study with 107 infected and 90 seroreverter children. We compared the IgA, IgG, and IgM levels between infected and seroreverters in the first 18 months of life; IgA, IgG, and IgM as surrogate markers of infection; and IgA, IgG, and IgM levels in the first 5 years in infected children, according to clinical outcome. The Mann-Whitney test was used for comparison between groups. Surrogate markers were assessed according to sensitivity, specificity, positive and negative predictive values, and J index. RESULTS: Infected children, when compared to seroreverters, showed significantly higher levels of: IgM from the 1st to the 5th trimester; IgA and IgG from the 2nd to the 6th trimester (P /= 90 mg/dl in the 2nd trimester and IgG >/= 1,700 mg/ dl or 1,200 mg/dl in the 2nd and 3rd trimesters were associated with HIV infection with J indexes of 0.97, 0.92, and 0.93, respectively. Infected children in the B and C categories, compared to those in the N and A, showed higher levels of IgM from the 2nd to the 4th year, and IgA from the 3rd to the 5th year (P 相似文献   

Clinical impact of altered immunoglobulin levels in Henoch–Schönlein purpura

Background:  The aim of the present study was the identification of immunological features, present at the time of diagnosis, that would predict the severity of Henoch–Schönlein purpura and its outcome.
Methods:  A cohort study was carried out in a tertiary pediatric hospital of 69 children with Henoch–Schönlein purpura, in whom serum complement components C3, C4 and IgA, IgM, IgG were repeatedly determined.
Results:  During the acute phase of the disease in 54/69 patients (78.3%) immunological imbalances were observed. In 24/54 cases (44.4%) certain complications involving the kidneys and the gastrointestinal tract were noted as opposed to in 3/15 children (20%) without immunologic abnormalities. In 50/69 children (72.5%), elevated serum IgA was detected and 16 of them (32%) developed renal involvement while only 1/19 children (5.3%) with normal IgA concentration had renal involvement. Considering separately the group of 9/69 children (13%) with increased IgM and those with normal IgM levels (53/69; 76.8%), irrespective of IgA and IgG concentration, we found a comparable percentage of children who had both renal and intestinal involvement without, however, developing severe complications, which were exclusively seen in patients with increased IgA (5/7 children) and reduced IgM levels. Serum C3 fraction was elevated in 26 children (37.7%) and in 73% of cases it was associated with increased serum IgA values.
Conclusion:  Renal involvement was seen in 32% of children with increased IgA values. Most importantly, elevated IgA concentration along with reduced IgM levels was associated with higher prevalence of severe complications.     

Serum anti-streptococcal IgA,IgG and IgM antibodies in IgA-associated diseases

Serum anti-streptolysin-O antibody (ASO) and anti-streptococcal polysaccharide antibody (ASP) of IgA, IgG and IgM classes were measured using an enzyme-linked immunosorbent assay in 41 children with IgA nephropathy (Group A), 15 children with uncomplicated anaphylactoid purpura (Group B) and 13 children with purpura nephritis (Group C). The serum concentrations of the IgA, IgG and IgM classes were measured by single radial immunodiffusion. When compared with sex- and age-matched controls, the concentrations of serum IgA (but not of IgG or IgM) were significantly increased in the three groups studied. The titers of ASO of the IgA and IgM classes, and those of ASP of the IgA and IgG classes, were significantly increased in Group A. In Group B, only the ASP titers of the IgA class were significantly increased. No significant difference was noted in the titers of either ASO or ASP of any class in Group C. Thus, increased antibody response in IgA nephropathy is not restricted to IgA. Anaphylactoid purpura with or without renal disease appears to be different in its humoral anti-streptococcal response from IgA nephropathy.     

反复肺炎婴幼儿血清β-防御素-1和免疫球蛋白A、G、M水平的研究

目的：通过检测反复肺炎婴幼儿和健康婴幼儿血清中β-防御素-1（hBD-1）和免疫球蛋白A（IgA）、免疫球蛋白G（IgG）、免疫球蛋白M（IgM）浓度,探讨hBD-1和IgA、IgG、IgM在反复肺炎发病中的可能作用。方法：收集35例2~24月龄反复肺炎和35例健康婴幼儿的血清,处理后应用酶联免疫吸附法（ELISA）检测血清中hBD-1浓度,应用免疫比浊法测定血清IgA、IgG、IgM浓度,并分析血清hBD-1与IgA、IgG、IgM之间的相关性。结果：反复肺炎组血清hBD-1浓度为14±11 μg/mL,显著低于健康对照组（18±11 μg/mL）,差异有统计学意义（P0.05）。结论：反复肺炎婴幼儿存在血清hBD-1、IgA、IgG水平低下的现象,提示呼吸道免疫防御功能存在障碍,这可能是婴幼儿反复肺炎的免疫因素之一;对反复肺炎婴幼儿同时检测 hBD-1、IgA、IgG、IgM具有重要的临床意义。     

Celiac disease diagnosis in misdiagnosed children

Antiendomysial antibodies (EMA) are today considered the most sensitive and specific serological marker of celiac disease (CD). The aim of the present study was to assess the occurrence of EMA of IgG isotype in EMA IgA negative children with clinical suspicion of malabsorption and their relationship with CD. Serum EMA IgG1 determination was performed on 30 EMA IgA negative children with clinical suspicion of CD. Total serum IgA levels were further investigated. Sixty children with gastroenterological diseases other than CD were used as control disease patients and 63 healthy children were evaluated as the control group. Eighteen out of 30 children in the study showed EMA IgG1 positivity in sera and a villous height/crypt depth ratio <3:1 as index of intestinal atrophy. It is noticeable that a selective IgA deficiency was present in only 9 of 18 EMA IgG1 positive children. In addition, clinical symptoms, EMA IgG1, and mucosal atrophy disappeared after 8-10 mo on a gluten-free diet. Neither EMA IgA nor EMA IgG1 were detected in the children in the control groups. The other 12 children in study group showed no histologic abnormalities and were EMA IgG1 negative. In this study, we reveal a group of EMA IgG1 CD children without IgA deficiency. The diagnosis was based on the presence of gluten-dependent typical serological and histologic features of CD. Our data suggest that EMA IgG1 determination could be a new tool in the diagnostic workup of CD, useful in avoiding possible misdiagnosis.     

Serum immunoglobulins, IgG subclasses, isohemagglutinins and complement-3 levels in patients with thalassemia major

Serum IgG, IgM, IgA, IgG subclasses (IgG1, G2, G3, G4), isohemagglutinins and complement-3 concentrations were measured in 23 beta-thalassemic patients suffering from recurrent infections. No significant abnormalities were found in these humoral immunity investigations, both in splenectomized and non-splenectomized patients. On the other hand, iron overload or repeated blood transfusions were not found to down-regulate the humoral immune system of thalassemic patients.     

Immune complexes and Pseudomonas aeruginosa antibodies in cystic fibrosis.

Serum samples from 57 patients with cystic fibrosis were tested for the presence of IgG, IgA, IgM, IgE, and circulating immune complexes containing IgG, IgA, and IgM. Titres of class specific antibodies to Pseudomonas aeruginosa, and class specific antibodies to Ps aeruginosa in circulating immune complexes, were also measured. According to the Shwachman score the patients were divided into three clinical groups: group 1-moderate and severe disease, group 2-mild disease, and group 3-well. The results of the immunological investigations were correlated with the clinical state of the patients as assessed by the Shwachman score. Serum concentrations of IgG, IgA, and IgM were inversely correlated with the Shwachman score, but the differences between the groups were only significant for IgG and IgA. The same correlations were found for circulating immune complexes containing IgG and IgA. Antibodies to Ps aeruginosa could be detected in most of the patients'' serum samples. IgA antibody specific to Ps aeruginosa was the most often raised, even in patients in group 3. It is therefore suggested that IgA antibody specific to Ps aeruginosa could be an early marker of colonisation by Ps aeruginosa and a sensitive measurement of infection with Ps aeruginosa in young children with cystic fibrosis. Moreover, in the circulating immune complexes, class specific antibodies to Ps aeruginosa were found in nearly half the patients. The highest titres of IgG and IgA antibodies specific to Ps aeruginosa in the circulating immune complexes were detected in the patients with the worst clinical state (group 1).     

Prolonged exclusive breast-feeding results in low serum concentrations of immunoglobulin G, A and M

Serum levels of IgG, IgA and IgM were measured in 198 infants at ages 2, 4, 6, 9 and 12 months. By age 9 months 30 infants were still exclusively breast-fed; their IgG and IgM levels were significantly lower than those of infants weaned early to formula (before age 3.5 months). By 12 months 6 infants were still exclusively breast-fed; their IgA levels were by then also similarly lower. There was no significant difference in the number of infections experienced by these groups of infants. After 2 months on formula feeding, the IgG and IgM levels of the infants who were exclusively breast-fed for 9 months had caught up with the levels of the infants weaned early to formula. Only at 12 months of age prealbumin levels of the exclusively breast-fed infants showed a positive correlation to IgG and IgA levels; no correlation was found between immunoglobulin levels and levels of serum iron and zinc.     

Prolonged Exclusive Breast-Feeding Results in Low Serum Concentrations of Immunoglobulin G, A and M

ABSTRACT. Serum levels of IgG, IgA and IgM were measured in 198 infants at ages 2, 4, 6, 9 and 12 months. By age 9 months 30 infants were still exclusively breast-fed; their IgG and IgM levels were significantly lower than those of infants weaned early to formula (before age 3.5 months). By 12 months 6 infants were still exclusively breast-fed; their IgA levels were by then also similarly lower. There was no significant difference in the number of infections experienced by these groups of infants. After 2 months on formula feeding, the IgG and IgM levels of the infants who were exclusively breast-fed for 9 months had caught up with the levels of the infants weaned early to formula. Only at 12 months of age prealbumin levels of the exclusively breast-fed infants showed a positive correlation to IgG and IgA levels; no correlation was found between immunoglobulin levels and levels of serum iron and zinc.     

新生儿肺炎白细胞介素-10、-13与免疫球蛋白的关系

目的　探讨新生儿肺炎白细胞介素 (IL) 10、 13与免疫球蛋白 (Ig)的关系。 方法　采用免疫酶法(ELISA)和速率散射比浊法检测新生儿肺炎患儿血IL 10、IL 13、IgG、IgA、IgM。以C反应蛋白 (CRP)≥ 2 0mg/L作为诊断细菌感染的界限值 ,结合临床资料 ,将肺炎分为 4组进行结果分析。结果　 1.肺炎组 8型常见病毒及支原体特异性IgM阳性 4 0份 (36 .0 % ) ;对照组 30份血清检测均阴性。病毒及支原体感染 (病毒感染 ) 2 3例(2 0 .7% ) ,细菌感染 4 5例 (4 0 .5 % ) ,混合感染 17例 (15 .3% ) ,不明病原感染 (其他感染 ) 2 6例 (2 3.4 % )。 2 .肺炎组IgA、IgM明显高于对照组 (P <0 .0 5 )。其中病毒感染组IgA明显高于其他感染组和对照组 (P <0 .0 5 ) ;IgM含量为细菌感染组显著高于对照组 (P <0 .0 5 )。 3.病毒感染组IgG、IgA、IgM分别与IL 10有显著相关 (P<0 .0 5 )。混合感染组、细菌感染组和对照组IgM分别与其IL 13呈显著相关 (P <0 .0 5 )。结论　新生儿肺炎时 ,IgA是完成抗病毒体液免疫应答的重要成分 ,IL 10对IgA产生具有调节作用 ;IgM能在抗菌性体液免疫机制中发挥重要作用 ,IL 13有助于调节IgM产生     

THE SIGNIFICANCE OF ANTINUCLEAR ANTIBODIES IN JUVENILE RHEUMATOID ARTHRITIS ASSOCIATED WITH CHRONIC BILATERAL IRIDOCYCLITIS

ABSTRACT. Serum samples from 8 children with juvenile rheumatoid arthritis (JRA) and chronic bilateral iridocyclitis were significantly distinguished from 5 children with JRA and no eye symptoms by the presence of large immune complexes (IC)>22S, IgM antinuclear antibodies (ANA), IgG granulocyte-specific (GS-) ANA, C3 fixing ANA, and IgM anti-IgG. One serum with and two sera without IC>22S, all from patients with iridocyclitis, were fractionated by rate zonal ultracentrifugation. Each fraction relevant for the study was separately concentrated and reexamined. In one of the sera without IC>22S this technique exposed the presence of IgA GS-ANA not detectable in the corresponding whole serum. IgG ANA were precipitated in an area with higher molecular weight than the one for IgG indicating the presence of aggregated IgG ANA. Fractionation of the serum with IC>22S demonstrated IgM GS-ANA not present in whole serum. The results support previous suggestions that ANA may be involved in the pathogenesis of chronic iridocyclitis and may explain why ANA (In particular C3 fixing ANA) negative patients with JRA rarely develop chronic iridocyclitis.     

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。     

小儿包虫病免疫球蛋白及IgG亚类抗体检测的诊断价值探讨

目的　研究小儿包虫病的免疫诊断方法 ,探索其抗体应答阴性反应原因。方法　采用间接ELISA和单克隆双抗体夹心ELISA方法 ,对 1998年 5月至 2 0 0 2年 5月新疆自治区人民医院普外科 5 5例小儿包虫病患儿血清的IgG及亚类IgG1、IgG2 、IgG3 、IgG4和IgA、IgM、IgE类抗体及抗原和循环免疫复合物 (CIC)进行检测。 结果　 8种抗体检查方法中IgG1亚型抗体检测的敏感性和特异性最好 ;15例IgG抗体阴性患儿中 ,有 12例分别检测出IgG亚类和 (或 )IgM、IgA、IgE ,有 3例患儿血清各种抗体均呈阴性反应 ;患儿IgM抗体阳性率高于成人 ;IgG1分别与其它种抗体联合检测 ,以IgG1 IgA IgM检出率最高 ;IgG阴性小儿患者血清的循环抗原和CIC阳性率分别为 4 0 %及 2 6 6 7%。结论　IgG1 IgA IgM抗体联合检测方法可提高小儿包虫病免疫诊断的敏感性。抗包虫总IgG抗体表达水平低下、抗体表达种类不同及CIC的形成 ,是造成包虫病患儿IgG抗体反应阴性的主要原因     

Quantification of immunoglobulins after organ culture of human duodenal mucosa

Duodenal biopsies from 33 celiac and 16 nonceliac patients were kept in organ culture for 24 h. Quantities of immunoglobulins were measured by rocket immunoelectrophoresis in mucus removed from the biopsy surface after culture and in culture media. Increased amounts of immunoglobulins were recorded after culture of biopsies from celiac disease patients in the exacerbation state; but only in 11 of 33 celiac mucosae could an increment be detected after culture in the presence of gluten compared to culture on gluten-free medium. The amount of IgA showed a significant correlation with radioactivity of mucus and culture medium after [14C]leucine incorporation during culture. In such experiments autoradiograms of immunoprecipitates disclosed in vitro synthesis of IgA, whereas 47% of the IgG precipitates were radionegative. Amounts of IgA corresponded significantly to serum concentration of this immunoglobulin, whereas for IgM and IgG no such correlation existed. Quantification of immunoglobulins seems to be unsuitable as a method of evaluating in vitro gluten toxicity in celiac disease.     

Neutrophil function tests in children with repeated infections—a preliminary study

Nitroblue tetrazolium (NBT) reduction test and candida avidity test were done in 100 cases of children with repeated infections. Serum IgG, IgA, IgM and C3 levels were also determined to assess the status of humoral immunity. It was seen that in the predominent (85%) group of repeated respiratory tract infections, 87 percent and 70 percent had moderate and marked rise of serum IgG and IgM, respectively, though 59 percent had lower than normal IgA values. C3 levels were normal in 41 percent and decreased in 36 percent in the same group. NBT was decreased in 45 percent within normal range in 30 percent and increased in 25 percent. The Candida avidity index (CAI) was decreased in 90 percent of cases with respiratory tract infections. The observations in the other groups were comparable to those of group repeated respiratory infections. As many of the cases were malnourished the influence of malnutrition on phagocytic activity could not be ruled out. There was no correlation between NBT and CAI tests and the levels of serum immunoglobulins and C3. The CAI appears to be more sensitive than NBT, indicating the particle avidity of the neutrophils.