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381.
We prospectively documented the development of a fatal, secondarily acquired severe immunodeficiency in a 19-year-old man who underwent uncomplicated bone marrow transplantation. He had no graft v host disease (GVHD) and had normal recovery of his immune system as determined by lymphocyte phenotyping, mitogenic responses of his peripheral blood lymphocytes, and his ability to secrete immunoglobulin. This alteration in immunity was associated with the acquisition of antibody to HTLV-III. His only risk factor for the development of HTLV-III infection was the transfusions he had received during the transplant and recovery period. Two of his 54 transfusions were from an asymptomatic individual at high risk for acquired immunodeficiency syndrome (AIDS), who was subsequently found to be seropositive for anti-HTLV-III and from whom HTLV-III was isolated. The loss of immunocompetence in patients without chronic GVHD disease is unusual, and our data support the view that this patient's immunodeficiency was due to HTLV-III. When bone marrow transplant recipients without chronic GVHD develop late opportunistic infections, consideration should be given to transfusion-associated AIDS.  相似文献   
382.
Four patients with lymphoproliferative disease of granular lymphocytes (LDGL) coexpressing CD3 and the natural killer (NK)-related "p58" receptor for HLA-C alleles were studied. These CD3+p58+ LDGLs have been detected among a series of 44 CD3+ LDGLs analyzed. Two patients with LDGL (GI and BA) expressed only the p58 molecule defined by the GL-183 and CH-L monoclonal antibodies (MoAbs), while the cases of patients PU and MA also coexpressed the molecular form identified by EB6 anti-p58 MoAb. Three LDGL cases (GI, MA, and PU) displayed the CD8+4-CD16+ T- cell receptor (TCR)alpha/beta+ phenotype, while one patient (BA) was CD8+4+CD16+ TCRalpha/beta+. Freshly isolated granular lymphocytes (GL) from these cases displayed cytolytic activity in an anti-CD3 MoAb- triggered redirected killing assay against the Fcgamma-receptor+ (Fcgamma-R+) P815 target cell line. Lysis of P815 target cells, triggered by an anti-CD3 or by anti-CD16 MoAb, could be inhibited by the addition of anti-p58 MoAb in three fresh or interleukin (IL)-2- cultured GL tested (GI, MA, and PU). Triggering of cytotoxicity against the HLA-DR+ Fcgamma-R+ Daudi cell line induced by appropriate superantigens could also be inhibited by anti-p58 MoAb in patients PU and GI with LDGL. These data indicate that activation through the CD16, CD3, and TCR molecules can be modulated by p58 receptors in these LDGLs. On the contrary, IL-2-expanded cells of patient BA were induced to lyse P815 target cells by anti-p58 MoAb. In addition, anti-p58 MoAB enhanced anti-CD16 MoAb triggered lysis and did not inhibit activation via CD3. These data indicate that, in this particular patient with LDGL, p58 displays a stimulatory effect on cell triggering, rather than the typical inhibitory effect previously observed in p58+ T-cell clones derived from healthy donors. The anti-p58 MoAb did not induce CA++ mobilization in p58+ LDGLs and in a p58+CD3+ normal T-cell clone equipped with inhibitory p58 molecules, while Ca++ mobilization could be observed in cultured GL from patient BA, which could be activated by anti-58 MoAb. These findings suggest that stimulatory and inhibitory p58 molecules are equipped with different signal transducing properties, thus contributing to a better knowledge of the normal counterpart.  相似文献   
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Faden  H; Humbert  J; Lee  J; Sutyla  P; Ogra  PL 《Blood》1981,58(2):221-227
Live Newcastle disease virus (NDV) was used to investigate the in vitro effects of a viral infection on phagocytosis, chemiluminescence generation, superoxide production, oxygen consumption, NADPH-oxidase activity, and intracellular killing of bacteria by Ficoll-Hypaque separated human neutrophils. Phagocytosis of oil red O particles by NDV- treated PMN was inhibited by 50%. Chemiluminescence by PMN was inhibited 79% after zymosan stimulation and 86% after tetradeconyl phorbol acetate stimulation. Superoxide generation was inhibited by 68%. Oxygen consumption was inhibited in the presence of NDV by 37% after stimulation with phorbol myristate acetate, while membrane- associated NADPH-enzyme activity was decreased by 19%. The percent of surviving intracellular S. aureus was significantly elevated in NDV- treated PMN after 60 and 120 min of incubation. Purified bacterial neuraminidase markedly suppressed chemiluminescence, while neuraminic acid blocked the effects of the virus. These observations suggest that infections with myxoviruses may suppress a number of vital neutrophil functions. It appears that the effects may be partly mediated by the interaction of viral neuraminidase with the external neutrophil membrane.  相似文献   
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387.

Background

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF.

Methods

Single center study of EA/TEF infants referred from January 2000 to December 2015. Data collected included associated defects, neonatal morbidity and mortality and growth and neurodevelopmental outcomes at age 12–36 months. Multiple regression analysis was used to determine variables associated with adverse outcome.

Results

Of the 253 infants identified, 102 infants (40%) were preterm. Overall mortality was 8.3%, the majority from major cardiac malformations (p < 0.001) Neurodevelopmental assessments (n = 182) showed that 76% were within normal, while some delay was seen in 24%, most often in expressive and receptive language. Nine infants had hearing impairment and 5 had visual impairment. Gastrostomy tubes were required in 47 patients and 15% continued to have weight growth velocities less than the 10th centile. A number of specialist interventions were required, Speech/Language being frequent.

Conclusion

Mortality in EA/TEF is primarily related to concomitant anomalies, especially cardiac. Multidisciplinary follow up is important for early identification and intervention for growth failure and developmental delay.

Type of study

Retrospective study

Level of evidence

Level II  相似文献   
388.
阮坚  潘永寿  庾红玉  秦蕾  冯红兵  皮永前 《海南医学》2013,24(15):2248-2249
目的应用实时三维超声心动图(RT-3DE)评价心力衰竭患者的左室心肌质量。方法应用RT-3DE分别测量104例心力衰竭患者(观察组)和74例健康人(对照组)的左室心肌质量(LVM)和左室心肌质量指数(LVMI),并进行比较。结果观察组患者的LVM和LVMI测值明显大于正常对照组,差异有显著统计学意义(P<0.01)。结论 RT-3DE能准确评价心力衰竭患者LVM状况。  相似文献   
389.
斑蝥素毒性及其药(毒)动力学研究   总被引:9,自引:0,他引:9  
目的 研究斑蝥素对小鼠肝、肾毒性及其在小鼠体内的动态行为。方法 用小鼠半数致死量(LD50)及生化指标的变化来表征斑蝥素的毒性,用小鼠急性死亡率法测定斑螫素药物动力学参数。结果和结论 斑蝥素对小鼠肝、肾有明显的毒性作用,该药在小鼠体内动态变化符合一级动力学,呈二室开放模型,其表观药动学参数为:A=10.1mg/kg;α=1.56 h-1;t1/2α=0.44h;B=1.19mg/kg;β=0.123h-1;t1/2=5.63h;K21=0.274h-1;K10=0.700h-1;K12=0.709h-1;CL=0.071kg/kg·h-1;AUC=16.15 mg·h/kg;Vc=0.102kg/kg;Vp=0.264 kg/kg。  相似文献   
390.
食指背筋膜蒂岛状皮瓣的临床应用   总被引:1,自引:0,他引:1  
目的:探讨血管神经蒂食指背筋膜岛状皮瓣的应用效果。方法:采用食指背侧筋膜蒂岛状皮瓣对10例拇指软组织缺损,2例虎口软组织缺损进行了修复,结果修复后的拇指或虎口外形较好,功能恢复满意,皮瓣厚薄适中,色泽质地良好。结论:该皮瓣具有多源性血供的特点,不因第一掌背动脉的类型及出现率不稳定而影响手术成败,是修复拇指,虎口皮肤缺损的理想皮瓣。  相似文献   
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