幼年类风湿关节炎患者隐匿性IgM型类风湿因子的临床意义

目的检测隐匿性IgM型类风湿因子(HIgM-RF)并探讨其在幼年类风湿关节炎(JRA)中的临床意义.方法利用聚乙二醇(PEG)分离含类风湿因子(RF)的免疫复合物,在酸性条件下解离出RF,以酶联免疫吸附试验方法,测定HlgM-RF.68例JRA患儿中的16例在活动期和缓解期HIgM-RE,与正常对照组(35例)比较.结果(1)HIgM-RF在JRA的阳性率为71.4%,其中多关节炎型阳性率为80.0%,少关节炎型阳性率为71.4%,全身型阳性率58.8%.(2)多关节炎型、少关节炎型、全身型JRA活动期HIgM-RF均值高于缓解期,差异均具有非常显著意义(t=11.15,t=14.67,t=5.78;P＜0.01).全组活动期JRA均值同正常对照组比较,差异有显著性(F=16.34,P＜0.01).但缓解期均值同正常组比较差异未见显著性意义(P＞0.05).(3)HIgM-RF与血沉(ESR)、C-反应蛋白(CRP)正相关(r=0.69,P＜0.01;r=0.32,P＜0.01).同年龄、性别、病程无关.结论HIgM-RE在JRA中有较高的检出率,并与病情活动性有关,可作为JRA诊断、评估活动性的一个指标.     

腮腺炎脑膜脑炎患儿血清及脑脊液特异性IgM抗体检测及其临床意义

目的探讨腮腺炎脑膜脑炎患儿血清及脑脊液特异性IgM抗体检测的临床意义。方法对97例临床诊断为腮腺炎脑膜脑炎患儿，应用酶联免疫吸附试验(ELISA)方法检测血清及脑脊液特异性IgM抗体。结果血清IgM抗体阳性率为94.44％(85/90)，脑脊就座IgM抗体阳性率为86.90％(73/84)。结论采用ELISA方法检测脑脊液腮腺炎病毒特异性IgM抗体是早期诊断腮腺炎脑膜脑炎的有效方法之一。     

Vitamin K prophylaxis and vitamin K deficiency bleeding (VKDB) in early infancy

The efficacy of vitamin K prophylaxis (1 mg im or sc, or 1-2 mg orally both given as a single dose at birth) in the prevention of vitamin K deficiency bleeding in early infancy was estimated in Germany during a 15-month period between 1988 and 1989. Cases were identified by a survey of all paediatric hospitals and population denominators by a survey of all obstetric hospitals. Response rates were 85% and 68%, respectively. Thirteen cases of vitamin K deficiency bleeding in early infancy with confirmed prophylactic states were confirmed, seven of whom had intracranial haemorrhage. The estimated efficacy of single parenteral administration of vitamin K versus no prophylaxis was 96.7% (95% confidence interval: 74-99.6%) and for single oral administration versus no prophylaxis 80.4% (9.1-95.6%). Single parenteral vitamin K prophylaxis gave substantial protection against vitamin K deficiency bleeding in early infancy. Single oral prophylaxis appeared to be less effective, although the difference was not significant, as indicated by the wide overlap of the respective 95% confidence intervals.     

隐匿型类风湿因子-IgM、抗环瓜氨酸肽抗体、抗角蛋白抗体联合检测在幼年特发性关节炎诊断中的意义

目的探讨隐匿型类风湿因子（HRF）IgM、抗环瓜氨酸肽（CCP）抗体、抗角蛋白抗体（AKA）联合检测对幼年特发性关节炎（JIA）的诊断价值。方法用酶联免疫吸附试验（ELISA）对60例JIA、27例系统性红斑狼疮（SLE）、18例川崎病、50例健康对照组儿童血清行抗CCP抗体、类风湿因子（RF）检测;用间接免疫荧光法进行AKA检测;利用葡聚糖凝胶A-50为层析柱在酸性条件下分离出RF,再用ELISA测定HRF-IgM。结果抗CCP抗体阳性率21.7%（13/60）,特异性96.8%;RF在JIA与非JIA组中阳性率无差异（χ^2=0.17P=0.68）;AKA与抗CCP抗体结果相近,敏感性差,特异性高;HRF-IgM敏感性65%,特异性85.3%JIA与非JIA组有极显著性差异（χ^2=40.22P=0.0001）。结论抗CCP抗体及AKA敏感性低,特异性高,单独作为JIA的诊断指标有一定的局限性;HRF-IgM敏感性及特异性高,作为JIA诊断指标较好;三者联合对提高JIA的诊断率更有价值。     

Large granular lymphocyte leukemia (LGL) in a child with hyper IgM syndrome and autoimmune hemolytic anemia

We describe a female with a history of autosomal recessive hyper-IgM (HIGM) syndrome along with a history of autoimmune hemolytic anemia and intermittent lymphadenopathy. She subsequently developed neutropenia, lymphocyostosis and mild thrombocytopenia. Flow cytometry of the peripheral blood revealed the presence of a marked predominance of cytotoxic T lymphocytes, shown to be clonal, with concomitant natural killer (NK) antigen expression. She responded to weekly methotrexate therapy.     

19S IgM rheumatoid factor-7S IgG rheumatoid factor immune complexes isolated in sera of patients with juvenile rheumatoid arthritis

19S IgM rheumatoid factors (RF) and hidden 19S IgM RF have been associated with increased disease activity in juvenile rheumatoid arthritis (JRA). Recently, immune complexes (IC) were isolated from JRA sera by several methods which demonstrated the presence of 19S IgM RF. The present study evaluates 25 JRA patients' sera by separation on a Sepharose 4B column to which were bound F(ab')2 fragments of goat IgG antihuman IgM antibody to separate IgM-containing IC. The columns were sequentially eluted with 1 M ammonia and 0.1 M glycine-HCl buffer, pH 3.0. The isolated fractions were assayed for 19S IgM RF and 7S IgM RF by ELISA, IgG levels by immunodiffusion, and by preparative isoelectric focusing. The ammonia eluate from the alpha HIgM column revealed IgG, 19S IgM RF in six patients, and IgM RF in four patients. All were polyarticular-onset JRA patients. In the glycine-HCl eluate of sera, 19S IgM RF and IgG were also detected in 15 patients, all six seropositive, polyarticular-onset, six seronegative, polyarticular-onset, and three pauciarticular-onset patients. Significant 7S IgG RF titers were demonstrated in the glycine-HCl eluates of six patients, five seropositive, polyarticular-onset patients, and one seronegative, polyarticular-onset patient. Analysis by preparative isoelectric focusing of the IgM RF and IgG RF positive ammonia and glycine-HCl eluates showed IgM RF throughout the pH range (4-10), but the highest amount of IgM RF was in the pH range 4.0-5.5. Significant IgG RF titers were detected only in this restricted spectrotypic area of pH 4.0-5.5.(ABSTRACT TRUNCATED AT 250 WORDS)     

Serial IgG and IgM serum levels after infusion of different Ig-preparations (IgG or IgM-enriched) in preterm infants

Intravenous administration of Immunoglobulin (IVIG) has been used for prevention or treatment of neonatal sepsis. However, therapeutic efficacy of IVIG is dependent on pharmacokinetic factors. There have been no comparative studies in neonates between licensed IgG and IgM enriched preparations. The aim of this study was to investigate serial IgG and IgM serum levels during the neonatal period in two groups of non-septic, preterm infants treated prophylactically with IVIG. Twenty-two very low birth weight (VLBW) patients (1001-1500g) (gestational age 31.8±2.0 weeks and 1265±245g birth weight) and 12 extremely low birth weight (ELBW) patients (<1000g) (gestational age 28.6±2.5 weeks and 910-85g birth weight) received at random three standard doses of Sandoglobulin (SG) (0. 5 g/kg/day) or IgM enriehed Pentaglobin (PG) (5 ml/kg/day). IgG and IgM concentrations were assayed by rate nephelometry before treatment and at day 3, 5, 7, 14 and 28 of life. At any time IgG levels were higher in the SG-VLBW group (p < 0.01). no difference being observed in the ELBW group (p>0.5). IgM levels were higher at day 3 and 5 in the PG-VLBW group and until day 7 in the ELBW group (p < 0.01). This study indicates a wide range of IgG and IgM kinetics in the healthy premature and suggests frequent monitoring of immunoglobulin serum levels during efficacy studies.     

Antiperinuclear factor in the diagnosis of juvenile rheumatoid arthritis

The antiperinuclear factor (APF) was estimated by immunofluorescent microscopy in the sera of 32 children and adolescents with juvenile rheumatoid arthritis (JRA) in comparison to a group of 16 children and adolescents with other rheumatologic disorders and a group of 20 age-matched healthy subjects. The APF was detected in 17 children with JRA (53%), in only one patient in the group of other rheumatologic disorders (6%), and in 2 healthy children (10%). Accordingly, APF had a sensitivity of 53%, a specificity of 92%, and a diagnostic efficiency of 74% in our series. APF was found to have a higher diagnostic gain in rheumatoid factor (RF) seronegative cases than did the RF in APF negative cases, meaning a higher sensitivity of APF as compared to the RF. The APF seropositivity was neither altered by the use of corticosteroids nor influenced by the age, gender, duration of illness, or number of joints affected. Three out of 5 patients with JRA had the APF detected in their synovial fluid; they were running rather a severe course of illness. The use of the APF could be an aid in the diagnosis of JRA.     

散发型高IgM综合征CD40配体基因突变检测

目的揭示１例散发型高ＩｇＭ综合征（ＨＩＭ）患儿发病机制及其遗传类型，从而为其今后可能的基因治疗打下基础。方法用夹心酶联免疫吸附试验（ＥＬＩＳＡ）法检测患儿外周血单个核细胞分泌免疫球蛋白的能力；用单克隆抗体染色检测ＣＤ４０配体（ＣＤ４０Ｌ）表达情况；聚合酶链反应（ＰＣＲ）产物直接序列分析检测患儿ＣＤ４０Ｌ开放阅读框架基因突变。结果此例ＨＩＭ患儿Ｂ细胞体外刺激可产生ＩｇＧ，Ｔ细胞表面ＣＤ４０Ｌ染色阴性，序列分析发现６５４６５７位碱基缺失。结论患儿Ｂ细胞可能具有正常Ｉｇ分泌功能，Ｉｇ同种型转换障碍是由于其Ｔ细胞ＣＤ４０Ｌ基因缺失，并在国内首次从分子水平上确诊一例性联高ＩｇＭ综合征患儿     

Study of anti tetanus IgG and IgM levels in maternal and cord sera by enzyme linked immunosorbent assay (ELISA)

The study was done to determine IgG and IgM antitetanus antibodies in mothers after antenatal immunization, and extent of transfer to the baby as measured by cord levels of tetanus specific IgG and IgM by ELISA using enzyme penicillinase and to study the effect of nutritional status of mothers on antibody response to tetanus toxoid and corresponding cord levels also to determine the number of doses required for protective antibody levels in cord blood. The results obtained in sera of 100 mothers at the time of delivery and their respective cord sera and results of 43 different control sera are discussed using ELISA technique.     

The significance of factor VII-deficiency in infancy and childhood (author's transl)

Prolonged thromboplastin time but normal partial thromboplastin time in coagulation analysis lead to the diagnosis of factor VII-deficiency. The different forms of congenital and acquired factor VII-deficiency and the appropriate therapies are discussed, especially with regard to the recently available factor VII preparation.     

Clinical and ultrasound assessment of the knee in children with juvenile rheumatoid arthritis

Objective  To correlate clinical features with ultrasound (USG) findings in the detection, quantification and follow up of inflammatory signs of knee in children with mono or pauciarticular juvenile rheumatoid arthritis (JRA). Methods  Thirty patients (11 girls, 19 boys) with pauciarticular JRA (14 with monoarticular and 16 with bilateral knee involvement) were studied. Mean disease duration was 10 months (range 2 months to 5 yr). All knees were classified into two groups, according to the presence or absence of acute inflammation. Clinical assessment and ultrasound was done in all patients on the same day. All the patients received naproxen (15–20 mg/Kg/day) for a period of six months, after which clinical assessment and ultrasound study was repeated. Results  Synovial proliferation and effusion, was demonstrated in a much higher frequency in those clinically active (Group A) as compared to these in clinical remission (Group B). Statistically significant differences between clinical and USG indices were seen. Conclusion  USG of knee is more sensitive than clinical assessment in detection of synovial effusion and thickening and plays a useful role in monitoring evolution of the inflammatory process, its quantification and for follow up.     

Increased frequency of IgM antibodies to cow's milk proteins in Hungarian children with newly diagnosed insulin-dependent diabetes mellitus

We investigated the association between serum antibodies to cow’s milk proteins and insulin-dependent diabetes mellitus (IDDM) in Hungarian children. Forty-eight children 1.0–17.1 years of age with newly diagnosed IDDM and 74 control children 1.0–16.0 years of age were studied for serum IgG, IgA and IgM antibodies to cow’s milk, β-lactoglobulin, bovine serum albumin and ovalbumin by enzyme-linked immunosorbent assays. The specificity of IgM antibodies to β-lactoglobulin and bovine serum albumin was controlled by Western blot. The levels of IgG and IgA antibodies to cow’s milk proteins were similar in children with and without IDDM, with the exception of slightly increased levels of IgA antibodies to β-lactoglobulin in diabetic children (P = 0.05). The levels of IgM antibodies to cow’s milk were significantly higher in IDDM patients than in control children (P = 0.0002). Children with IDDM more often had IgM antibodies to β-lactoglobulin (46.3% vs 18.8%; P = 0.002) and bovine serum albumin (87.8% vs 49.3%, P < 0.0001) than control children. Neither the levels of IgG or IgA antibodies to ovalbumin nor the frequency of IgM antibodies to ovalbumin differed between diabetic and control children. Conclusion In Hungarian children, clinical manifestation of IDDM is often associated with IgM antibody response to cow’s milk protein and its fractions, β-lactoglobulin and bovine serum albumin, indicating a loss of immunological tolerance to these proteins. IgG and IgA antibodies to cow’s milk proteins, associated with an early introduction of cow’s milk in diet, seem to play a minor role in the development of childhood IDDM in Hungary. Received: 14 November 1995 Accepted: 3 March 1996     

肾母细胞瘤VEGF及其受体Flk-1的表达及其临床意义

目的 探讨肾母细胞瘤组织中血管内皮生长因子(VEGF)及其受体(Flk-1)的表达和肿瘤微血管密度(MVD)的临床意义.方法 应用免疫组织化学(SABC)法检测33例肾母细胞瘤、瘤旁组织和6例正常肾组织中VEGF、Flk-1表达和MVD计数.观察并分析三个指标之间的相关性,同时分析其临床意义.结果 33例肿瘤组织VEGF阳性表达率81.8%,Flk-1阳性表达率69.7%,瘤旁组织VEGF阳性表达率9.1%,Flk-1阳性表达率6.1%,6例正常肾组织VEGF及Flk-1均为阴性表达,三者之问差异具有统计学意义.肿瘤组织MVD值29.7±12.4,瘤旁组织MVD值10.3±9.6,正常肾组织MVD值9.8±2.3.肿瘤组织的血管密度与后二者比较差异具有统计学意义.VEGF表达与Flk-1、MVD计数正相关,Flk-1与MVD表达正相关,VEGF、Flk-1表达在不同临床分期,及转归之问均具有显著性差异.结论 肾母细胞瘤中VEGF及其受体Flk-1呈高表达,血管生成增多,VEGF、Flk-1表达与MVD密切相关,提示VEGF及其受体Flk-1参与肾母细胞瘤的血管形成过程,促进血管生成,与预后相关.     

抗环瓜氨酸肽抗体和隐匿性类风湿因子免疫球蛋白M型在诊断早期幼年类风湿关节炎中的意义

目的 检测抗环瓜氨酸肽(CCP)抗体及隐匿性类风湿因子IgM型(HRF-IgM),并探讨其在幼年类风湿关节炎(JRA)早期诊断中的临床意义。方法 用人工合成CCP链为抗原检测抗CCP抗体;对27例早期诊断的JRA做动态检测,通过阳性预测值(PPV)和阴性预测值(NPV)确定抗CCP抗体和HRF-IgM对早期诊断的JRA的特异性和敏感性。结果 抗CCP抗体和HRF-IgM总阳性率分别为58.5％、65.0％。后者敏感性要高于前者,病情越重或受累的关节越多,抗体检出率越高。对早期JRA的PPV、抗CCP抗体特异性要高于HRF-IgM。当两种实验联合应用时,对具有早期关节炎表现发展成JRA的PPV为93.7％。结论 抗CCP抗体和HRF-IgM在JRA患儿均有较高的检出率,并与疾病严重程度有关。抗CCP抗体与HRF-IgM联合应用时,可使JRA的PPV进一步提高。     

Effect of Recombinant Human Granulocyte-Colony Stimulation Factor (rhG-CSF) on Immune System in Pediatric Patients with Aplastic Anemia

To determine the effect of recombinant human granulocyte-colony stimulation factor (rhG-CSF) on the immune system, serum immunoglobulins, lymphocyte subsets, and serum cytokines were analyzed in eight pediatric patients with atlastic anemia (AA) during 8-week rhG-CSF therapy. The rhG-CSF was administered either subcutaneously (200 μglm^' × 4 weeks, followed by 400 Fglm' × 4 weeks) or intravenously (400 μglm^' × 4 weeks, followed by 800 μg/m² × 4 weeks). In response to rhG-CSF therapy, neutrophil counts exceeded the pretreatment counts by twofold during the first week except for one case that did not attain twofold increase until day 41. While serum IgC and IgA were not affected, serum IgM was elevated during treatment in six of the eight cases to more than 1.2-fold basal levels (P < 0.04); however, there was no increase in serum interleukin (IL)-6 and interferon-y levels. On the other hand, CD56 positive NK cells significantly dropped from 7.7% to 4.5% (P < 0.02). These results indicate that systemic administration of rhG-CSF affects not only the neutrophil count, but also serum IgM levels and the natural killer cell population in patients with AA.     

Treatment of intractable diarrhea in infancy with human milk (author's transl)

Progressive, severe diarrhea in a Turkish infant, beginning at age of 6 weeks, was refractory to conventional therapy, including casein hydrolysate and total parenteral nutrition. At age of 9 months, human milk as sole food together with small doses of prednisone was started. During the next 7 months of feeding human milk, body weight of the infant rose from 4750 to 7000 g and the stools became normal.     

表皮生长因子在血管瘤和血管畸形中的表达

目的 了解表皮生长因子(epidermal growth factor,EGF)与血管瘤及血管畸形的关系.方法 标本选自1998年3月至2005年10月经手术切除的先天性皮肤血管病变92例患儿,女52例,男40例,年龄为40 d至13岁,平均年龄为1.6岁.组织标本取出后立即浸入10%的中性甲醛溶液中固定,常规石蜡包埋,5μm连续切片,HE染色,同时采用含有正常血管的皮肤标本10例作为对照.采用免疫组织化学方法,对72例血管瘤和20例血管畸形组织切片进行EGF表达的测定.结果 EGF在血管瘤组织中高表达,而在血管畸形组织中低表达(P<0.01).在血管瘤增殖期、退化早期和退化完成期三者之间,EGF的表达存在着差异.在血管瘤中EGF的表达与其内皮细胞的增生程度有关.内皮细胞增生越强,EGF的表达越高(P<0.05).结论 提示EGF与血管瘤的增生及退化有关,而与血管畸形无明显关系.     

消化性溃疡患儿唾液,胃液和血清中表皮生长因子含量

目的了解表皮生长因子（ＥＧＦ）与小儿消化性溃疡病的发生、愈合之间的关系，探讨小儿溃疡病的可能发生机理。方法采用放射免疫法测定了５５例溃疡与５１例慢性胃炎患儿的唾液、胃液和血清中ＥＧＦ的含量。结果消化性溃疡患儿在溃疡活动期时，唾液和胃液中的ＥＧＦ水平明显低于慢性胃炎组，而在溃疡愈合期时，唾液、胃液、血清中ＥＧＦ水平显著升高，尤为胃液。结论消化性溃疡患儿存在ＥＧＦ的分泌异常，ＥＧＦ分泌水平的变化可能与消化性溃疡的发生与愈合有一定关系。