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81.
Ali Çay İbrahim Aydoğdu Semih Lütfü Mirapoglu Hüseyin Toprak 《Journal of Medical Ultrasonics》2018,45(4):633-639
Neurenteric cyst is a very rare developmental anomaly. Prenatal diagnosis of mediastinal neurenteric cysts has been reported rarely. We present a case of neurenteric cyst associated with vertebral anomalies diagnosed by prenatal ultrasonography at 31 weeks of gestation, which was treated successfully in the early neonatal period. In addition, we searched the English literature for all cases of mediastinal neurenteric cyst diagnosed in the prenatal period reported to date. We found that only 17 cases were reported previously. We reviewed the reports of these 17 patients along with our case, and we investigated the prenatal and postnatal diagnosis and treatment approaches and the factors influencing the prognosis. Fetuses with mediastinal neurenteric cysts should be monitored regularly by ultrasonography. Fetuses with no signs of hydrops are more likely to survive with proper neonatal center transfer, regular follow-up, and appropriate postnatal approach. Fetuses with hydrops findings have a high risk of fetal and neonatal death. 相似文献
82.
83.
Hagen Sjard Bachmann Werner Meier Andreas du Bois Rainer Kimmig Jan Dominik Kuhlmann Winfried Siffert Jalid Sehouli Kerstin Wollschlaeger Jens Huober Peter Hillemanns Alexander Burges Barbara Schmalfeldt Behnaz Aminossadati Pauline Wimberger 《British journal of clinical pharmacology》2015,80(5):1139-1148
Aim
Despite promising preclinical findings regarding clinical utility of farnesyltransferase inhibitors (FTI), such as lonafarnib, success of clinical trials is limited. A multicentre AGO-OVAR-15 phase II trial reported an unfavourable effect of lonafarnib on the outcome of patients with advanced ovarian cancer. This study was performed as a genetic subgroup analysis of the AGO-OVAR-15 trial, and investigated the utility of the promoter polymorphism rs11623866 of the farnesyltransferase ß-subunit gene (FNTB) in predicting the clinical effectiveness of lonafarnib.Methods
The influence of rs11623866 (c.-609G > C) on FNTB promoter activity was investigated by electrophoretic-mobility-shift assay, luciferase-reporter assay and RT-qPCR. A total of 57 out of 105 patients from the AGO-OVAR-15 trial, treated with carboplatin and paclitaxel ± lonafarnib, was genotyped for rs11623866 by restriction fragment length polymorphism analysis. Genotype-dependent survival analysis was performed by Kaplan–Meier analysis.Results
The presence of the G allele was associated with increased FNTB promoter activity compared with the C allele. An unfavourable effect of lonafarnib was limited to patients carrying a GG genotype (HRPFS 6.2, 95%CI = 2.01, 19.41, P = 0.002; HROS 9.6, 95%CI = 1.89, 48.54, P = 0.006). Median progression free survival (PFS) for patients with the GG genotype in the lonafarnib treated arm was 10 months, whereas median PFS without FTI-treatment was 40 months. Median overall survival (OS) in the lonafarnib-treated group was 19 months, whereas median OS was not reached in the untreated group.Conclusions
Discrepancies between preclinical success and clinical failure may be due to the patients'' genetic variability of FNTB. Therefore, our results may encourage retrospective evaluation of FNTB polymorphisms in previous FTI studies, especially those reporting positive FTI response. 相似文献84.
Boulmier D Langanay T Clerc JM Muntean C du Haut-Cilly FB de Labriolle AC Bedossa M Le Breton H 《Archives des maladies du coeur et des vaisseaux》2003,96(2):135-139
Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital abnormality with a poor prognosis in the newborn. Adult forms are, therefore, very rare, presenting with angina, cardiac failure or sudden death. The authors report the case of a 41 year old woman who was asymptomatic until admitted as an emergency after ventricular fibrillation. Coronary angiography established the diagnosis. Despite the absence of reversible ischaemia on exercise myocardial scintigraphy, the patient underwent coronary bypass surgery of the left anterior descending artery with a pediculated internal mammary artery graft and closure of the left coronary ostium on the pulmonary artery. The echocardiographic abnormalities regressed within a few weeks. An automatic defibrillator was not implanted. The physiopathology of this rare cardiac lesion, the mechanisms of sudden death and the different techniques of surgical repair are discussed. 相似文献
85.
86.
Background and objectives: The aim of this study is to compare the relative sensitivity and specificity of commercial HIV-antibody assays using seroconversion, non-seroconversion panels, and negative blood donor samples. Materials and methods: We evaluated the sensitivity of five standard ELISA HIV-antibody assays: Vironostika HIV Uni-Form II, Abbott recombinant HIV-1/HIV-2 third-generation EIA, Biotest Anti-HIV-1/-2 recombinant, Recombigen HIV-1/HIV-2 EIA and Wellcozyme HIV 1+2 (VK54/55), and three rapid screening tests, Capillus HIV-1/HIV-2, Abbott Test Pack HIV-1/HIV-2 third-generation EIA, and Sensy-Test HIV 1/2. All tests were assessed using four panels of plasma samples obtained from individuals who were seroconverting and a low-titre HIV-antibody panel of samples. Specificity of the standard screening tests was determined on 3,500 HIV-antibody-negative blood donor samples. Results: There was no statistically significant difference in sensitivity between the five standard ELISA tests. One of these tests was significantly less specific than the others. The standard ELISA tests detected all the low-titre HIV-antibody-positive samples. Two of the rapid screening tests were significantly less sensitive on the seroconversion panels and all three tests failed to detect at least one of the positive samples in the low-titre panel. Conclusions: The additional risk of using one or other of the standard ELISA tests under review of not detecting all HIV-positive units of blood is not statistically significant. Using some of the rapid screening tests will, however, add a significant additional risk. A rapid screening test should therefore be adopted only after careful consideration of the effect of a possible lack of sensitivity on the safety of the blood supply. 相似文献
87.
Sari RA Kiziltunç A Taysi S Taysý S Akdemir S Akdemýr S Gündoğdu M 《Clinical rheumatology》2005,24(1):55-59
Behçets disease is a systemic vasculitis of unknown aetiology. Endothelial cell injury plays an important role in the pathogenesis and immunopathology of Behçets disease. E-selectin is expressed by activated endothelial cells. Because the selectin adhesion molecules are shed from activated cells, soluble forms of these proteins can be used as activation markers of endothelium (E-selectin). The pathogenesis of Behçets disease (BD) is closely related to endothelial cells, leucocyte functions and immunity. The aim of this study was to investigate circulating E-selectin adhesion molecules, which are known to play a significant part in the immune response especially by regulating interaction of the leucocytes with endothelium in BD. Plasma E-selectin concentrations were evaluated in 23 patients with BD and 20 healthy control subjects. The disease activity was evaluated by clinical manifestations (oral aphthous ulcer, genital ulceration, positive pathergy test, skin lesions, eye involvement, thrombophlebitis and arthritis) and by laboratory investigations [erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)]. The patients were newly or previously diagnosed cases not taking any drug for BD. Levels of E-selectin were measured with commercially available sandwich enzyme-linked immunosorbent assay (ELISA) kits using human sE-selectin (cat. no: BMS 205). Plasma E-selectin concentrations of patients and controls were compared with the Mann-Whitney U test. Statistical significance was assigned to p values lower than 0.05. Serum levels (mean±SD) of soluble E-selectin (sE-selectin) were significantly higher in 23 patients with BD than in 20 healthy controls (53.2±18.2 ng/ml vs 33.8±7.5 ng/ml, p<0.0001). A statistically significant positive correlation was observed between sE-selectin levels and CRP and ESR in patients with BD (r=0.78, p<0.001 and r=0.56, p<0.01, respectively). Increases in the E-selectin in BD may be a direct consequence of the leucocyte and endothelium activations observed during the disease process. The noninvasive investigations can be used as biochemical markers for inflammation. This may provide additional information regarding disease activity along with the traditional indices such as ESR and CRP. 相似文献
88.
The article summarizes some of the recent developments in the understanding of the mechanisms of regulation of the proximal tubule apical membrane Na+/H+ antiporter NHE3. NHE3 antiporter has a major role in HCO3- and NaCl reabsorption in the proximal tubule. NHE3 protein is associated with the regulatory factor NHERF which interacts with ezrin, an actin-binding protein. This multi-protein complex constitutes a link between a membrane protein, NHE3, and actin cytoskeleton. Cytoskeleton organization has a key role to control NHE3 activity under normal conditions. Pharmacological perturbations of actin polymerization interfere with NHE3 activity. Parathyroid hormone-induced NHE3 activity inhibition results first, from a protein kinase A-mediated phosphorylation without protein trafficking, and then from endocytosis involving dynamin. The stimulatory effect of systemic angiotensin II concentrations on NHE3 activity is protein kinase C-dependent and results, at least in part, from exocytic insertion of the protein in luminal membranes. It requires cytoskeleton integrity. 相似文献
89.
Catherine Riou Elsa du Bruyn Cari Stek Remy Daroowala Rene T. Goliath Fatima Abrahams Qonita Said-Hartley Brian W. Allwood Nei-Yuan Hsiao Katalin A. Wilkinson Cecilia S. Lindestam Arlehamn Alessandro Sette Sean Wasserman Robert J. Wilkinson 《The Journal of clinical investigation》2021,131(12)
T cells are involved in control of coronavirus disease 2019 (COVID-19), but limited knowledge is available on the relationship between antigen-specific T cell response and disease severity. Here, we used flow cytometry to assess the magnitude, function, and phenotype of SARS coronavirus 2–specific (SARS-CoV-2–specific) CD4+ T cells in 95 hospitalized COVID-19 patients, 38 of them being HIV-1 and/or tuberculosis (TB) coinfected, and 38 non–COVID-19 patients. We showed that SARS-CoV-2–specific CD4+ T cell attributes, rather than magnitude, were associated with disease severity, with severe disease being characterized by poor polyfunctional potential, reduced proliferation capacity, and enhanced HLA-DR expression. Moreover, HIV-1 and TB coinfection skewed the SARS-CoV-2 T cell response. HIV-1–mediated CD4+ T cell depletion associated with suboptimal T cell and humoral immune responses to SARS-CoV-2, and a decrease in the polyfunctional capacity of SARS-CoV-2–specific CD4+ T cells was observed in COVID-19 patients with active TB. Our results also revealed that COVID-19 patients displayed reduced frequency of Mycobacterium tuberculosis–specific CD4+ T cells, with possible implications for TB disease progression. These results corroborate the important role of SARS-CoV-2–specific T cells in COVID-19 pathogenesis and support the concept of altered T cell functions in patients with severe disease. 相似文献