C-antineutrophil cytoplasmic antibody positivity in vasculitis patients is associated with the Z allele of alpha-1-antitrypsin, and P-antineutrophil cytoplasmic antibody positivity with the S allele

BACKGROUND.: Antineutrophil cytoplasmic antibodies (ANCA) in vasculitis haveeither cANCA or pANCA patterns as defined by immunofluorescence.The target autoantigen of cANCA is usually proteinase 3 (PR3),whereas that of pANCA is usually myeloperoxidase (MPO). Alpha-1-antitrypsin(1AT) is the major physiological inhibitor of PR3, while MPOis an inhibitor of 1AT. METHODS.: To determine whether there was an association between ANCA positivevasculitis, ANCA pattern, and 1AT deficiency alleles, we studied1AT phenotypes of 99 cANCA and 99 pANCA positive vasculitispatients by isoelectric focusing and immunoblotting, and comparedthem with 2310 controls from the same geographical area. RESULTS.: C-ANCA patients showed an increased frequency of the Z allele(0.055 versus 0.018 in controls), conferring a relative riskof 3. They showed no increase in frequency of the S allele.P-ANCA patients showed an increased frequency of the S allele(0.091 versus 0.046 in controls) conferring a relative riskof 2. The frequency of the Z allele also appeared to be increased(0.030 versus 0.018 in controls), but this was not statisticallysignificant. CONCLUSIONS.: These findings demonstrate an association between ANCA-positivevasculitis and deficiency phenotypes of 1AT, and suggest a rolefor 1AT in the development of systemic vasculitis.     

The biology,pathology and immunology of a virus induced rat osteosarcoma

Summary The immunogenicity of a virus-induced rat osteosarcoma was studied utilizing the lymphocyte microcytoxicity test. Lymphocytes from progressor animals (in which the tumour progressed and metastasized) demonstrated an ability to kill osteosarcoma cells in vitro, while serum from these animals abrogated or blocked the cell-mediated cytotoxicity.Lymphocytes from regressor animals (in which tumours failed to develop or regressed spontaneously) also showed cytolytic activity against osteosarcoma cells in vitro, but their serum failed to block the lymphocyte-mediated cytolysis. Both progressor and regressor animals demonstrated the presence of humoral cytotoxic antibodies to tumour antigens on the basis of the ability of their serum to kill tumour cells in vitro. In an attempt to alter the fatal course of the disease in progressor animals, immunoprophylaxis and immunotherapy of the osteosarcoma in F1 hybrid rats war carried out by injecting them with parentalor, third party, allogeneic lymphoid cells. Injection of parental spleen lymphocytes into F1 hybrids produced a transient graft versus host reaction (GVHR), and prolonged the survival of the animals when lymphocytes were injected three days before, seven days after and on the day of tumour induction. Injection of allogeneic, third party lymphoid cells produced no detectable GVHR and prolonged the survival of F1 hybrids with osteosarcoma only when injected on the day of tumour induction. The prolonged survival of the groups treated with parental lymphoid cells was a result of stimulation of the host's immunological mechanisms during a transient GVHR, whereas the prolongation of survival in the group given allogeneic cells was most likely the result of a direct action of the donor lymphocytes on tumour cells, and not connected to a GVHR.SICOT Fellowship Award Paper, presented at the XIV-th World Congress of SICOT, Kyoto, Japan, October 15–20, 1978     

Detection of α S1-casein in vomit from bottle-fed babies by enzyme-linked immunosorbent assay

Summary This study describes a sensitive enzyme-linked immunosorbent assay (ELISA) using rabbit anti-bovine S1-casein antibody for the detection of commercial milk and milk-containing vomit. The antibody does not react with other human body fluids such as breast milk. The stability of S1-casein antigenic activity was examined after storage at different temperatures and enzyme digestion. There was no decrease after storage for one year at room temperature but 40% of the activity was lost after 6 months at 37°C. Enzyme digestion (6 hours, 37°C) resulted in 65 70% loss of activity but the antibody reacted with the peptide fragments of S1-casein. Vomit samples from 3 normal infants were tested by ELISA, and a S 1-casein could be detected in 1 cm² stain.     

Echocardiographic and color flow Doppler assessment of systemic and pulmonary venous connection and drainage in the neonate with congenital heart disease

Systemic and pulmonary venous anomalies are frequently encountered either as isolated lesions or as a significant component of a more complex lesion in the newborn infant with congenital heart disease. Two-dimensional echocardiography and Doppler techniques (conventional and color flow) have become the primary diagnostic imaging modality in this setting. Precise pre-operative definition of these variable venous connection and drainage patterns is critical as the required surgical procedure may solely be based on exact understanding of the veins' anatomy and physiology. On the systemic venous site, anomalies of superior and inferior venae cavae, innominate vein, and coronary sinus can be equally well imaged with either echocardiography or angiography. However, on the pulmonary venous site, echocardiography and Doppler techniques including color flow mapping are superior to angiography for precise definition of the connection and drainage sites of the individual pulmonary veins.     

肺癌患者外周血T淋巴细胞亚群检测的临床研究

通过流式细胞仪对确诊原发性支气管肺癌患者外周血T淋巴细胞亚群检测研究 ,并与健康人作对比。结果显示 ,肺癌患者CD3 ( 68 88± 11 80 )和CD4 ( 3 8 83± 10 3 0 )以及CD4 /CD8 ( 1 5 3± 0 74)比例均比正常人 ( 74 2 1±5 5 1,43 92± 7 0 2 ,1 89± 0 5 4)下降 ,CD8 ( 2 9 2 6± 10 5 8)高于正常人 ( 2 4 5 7± 6 3 5 ) ,两组之间差异有统计学意义 ,P均 <0 0 5。肿瘤临床分期之间检测结果差异无统计学意义 ,而病理类型中未分化癌与鳞癌之间CD4 和CD4 /CD8 下降具有统计学意义。初步研究结果提示 ,原发性支气管肺癌患者存在细胞免疫功能低下 ,并与细胞分化类型有关。     

Clinical features of Japanese children and adolescents with systemic lupus erythematosus: Results of 1980–1994 survey

Marked advances have been made in the past decade in the management of adults with systemic lupus erythematosus (SLE). Therefore, a nationwide retrospective survey was conducted between 1980 and 1994 to investigate the clinical manifestations of SLE in Japanese children and adolescents. Questionnaires were sent to 340 hospitals. Of 405 patients reported by 176 hospitals, 373 patients, diagnosed by the criteria established by the Pediatric Study Group of the Japanese Ministry of Health and Welfare in 1985, were enrolled in the study. Forty-nine of the 354 patients (13.8%) had relatives with a connective tissue disease within the third degree of consanguinity. The frequent manifestations in 373 patients were the presence of antinuclear antibody (98.9%), immunologic disorders (93.0%), hypocomplementemia (87.1%), malar rash (79.6%) and fever (74.0%). Lupus nephritis was present in 148 of the 309 patients (47.9%) at their first visit to a clinic, and 261 of the 373 patients (70.0%) developed renal involvement during the observation period. Of 370 patients, 92 patients (24.9%) exhibited central nervous system lupus. Of 368 patients, 192 patients (52.2%) were treated by methylprednisolone pulse therapy and 148 patients (40.2%) received immunosuppressants in combination with steroid therapy at some stage during the observation period. Survival rate at 5 years from onset was 95.9%. Management of infection, coagulopathies, and central nervous system involvement is essential to improve the prognosis of SLE in Japanese children and adolescents.     

Anomalous Insertion of the Superior or the Inferior Vena Cava into the Right Atrium

Three cases with anomalous insertion of the superior or inferior vena cava into the right atrium are presented. One case was a 25-year-old healthy man with anomalous low insertion of the right superior vena cava into the right atrium. The remaining two cases were infants with complex cardiac anomalies showing anomalous high insertion of the inferior vena cava into the right atrium. The congenital anomalies of the connection between the superior and the inferior vena cava and the right atrium are rare. Angiographic and computed tomographic findings of these anomalies were reported.     

Acute effects on systemic circulation after intratracheal instillation of Curosurf or Survanta in surfactant-depleted newborn piglets

Systemic vasodilatation in surfactant-depleted newborn piglets is induced by 200 mg/kg of modified porcine lung surfactant (Curosurf™). The aim of this investigation was to study whether this effect is dependent on dose and could further be induced by instillation of a bovine surfactant preparation (Survanta™). Twenty-two 3–5-d old piglets were subjected to repeated saline lung lavage and then randomized to one of three groups. Instillation of either Curosurf 100 mg/kg ( n = 8), Survanta 100 mg/kg ( n = 7) or Curosurf 200 mg/kg ( n = 7) was performed through the endotracheal tube. Systemic vascular resistance decreased 7 (± 4)%, 15 (± 12)% and 18 (± 6)% in the three groups, respectively ( p < 0:05 in all three groups). A significant difference between the high and low dose Curosurf groups was found ( p < 0:05), whereas no significant difference was seen between the Curosurf 100 mg group and the Survanta group. The decrease in vascular resistance was compensated by an increase in cardiac output, resulting in a stable mean arterial blood pressure. In conclusion, both Curosurf and Survanta induce a significant decrease in systemic vascular resistance in surfactant-depleted newborn piglets. A more pronounced effect was observed after 200 mg/kg than after 100 mg/kg of Curosurf.     

Cutaneous metastases from adenocarcinoma of unknown primary

Cutaneous manifestations of malignancy are not uncommon, especially in advanced disease. They may also occur early in malignant disease or they may even signify recurrence particularly if they are paraneoplastic in nature. Clinical diagnosis can be difficult because of the wide spectrum of appearance of these lesions, and, in many cases, because of the lack of an identifiable underlying primary. Presented here is the case of a 65-year-old woman with multiple inflammatory cutaneous metastases, which were sclerodermoid in nature. These appeared 14 months after initial diagnosis of adenocarcinoma of unknown primary (ACUP) and signified the beginning of a rapid deterioration in her condition. The coexistence of limited systemic sclerosis (scleroderma) and ACUP initially raised several interesting diagnostic possibilities. Adenocarcinoma of unknown primary and the sclerodermoid reaction in malignancy are discussed.     

脐带血单个核细胞体外诱导成CIK细胞及其抗肿瘤效应的实验研究

目的:探索建立CIK细胞的原代培养方 法并阐明其生物学特性,为该细胞在肿瘤生物过 继免疫治疗中的运用奠定基础。方法:通过加入 4种细胞因子(IFN γ、IL 2、IL 1及OKT3)将脐带 血单个核细胞(CBMNCs)诱导成CIK细胞;利用 流式细胞仪测定细胞表型、细胞增殖情况及细胞 周期的分布;利用改良的MTT法测定效应细胞 的体外细胞毒活性。结果:四种细胞因子的联合 运用,可诱导出大量的CIK细胞,其相对百分率 增长了163倍(从0.14%到22%),增殖高峰位 于第10～12天,针对K562、Hela和YTMLC肿瘤 细胞株,CIK细胞在效靶比为64∶1时的杀伤活 性分别为78.57±3.48、77.7±1.90和81.2± 1.51,在32∶1时分别为62.00±2.31、60.83± 2.80和64.07±2.87,在16∶1时分别为51.43± 2.51、52.87±2.91和54.10±3.11,均显著高于 LAK细胞及CBMNCs,P=0.001;而在效靶比为 8∶1(25.10±2.66、21.4±1.55和28.77±3.56) 及4∶1(17.10±2.10、11.93±1.86和9.49±2.92) 时与后两者差异无统计学意义,P=0.083。结 论:1)脐带血可作为诱导CIK细胞的重要来源 2)联合运用细胞因子能诱导出大量的CIK细胞 其增殖高峰位于培养的第12天,适宜在此期进 行临床运用;3)CIK细胞较LAK细