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1. Three cases of hereditary hemolytic disease secondary to G-6-PD deficiency are described. Two of the cases were first cousins of Scotch-Irish-English descent and the mode of inheritance was believed to be sex-linked.The third case was of Turkish origin; no family studies were availale.2. The mothers, who were heterozygous for G-6-PD deficiency, showed onlyminimal expression of the defect, which was manifested by a slightly decreasedred cell survival in both mothers and an abnormal methemoglobin reductiontest in one of them.3. All three cases showed a more pronounced fall in erythrocyte ATP afterincubation with phenylhydrazine than that observed in primaquine-sensitiveNegroes whose red cells were less deficient in G-6-PD.4. It is suggested that the inability of the G-6-PD-deficient erythrocyte tomaintain adequate levels of ATP may be an important factor in the pathogenesis of the hemolytic process. Submitted on August 26, 1963 Accepted on October 24, 1963 相似文献
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Interventions in saphenous vein grafts present some of the most challenging problems in preventing acute complications and limiting restenosis. Available options include repeat bypass surgery, balloon angioplasty, directional atherectomy, transluminal extraction atherectomy, rotational atherectomy, laser angioplasty, and stenting. Stenting appears to provide the best acute and long-term results. Debulking with directional atherectomy prior to stenting may be helpful but its role is unproven. With any device, it is essential to attain the lowest possible residual stenosis with the least amount of manipulation. Complications with vein graft interventions are most commonly related to distal embolization, which occurs most frequently in older vein grafts with diffuse disease, large plaque volume or thrombus, or those with total occlusion. Use of thrombolytics, glycoprotein Ilb/IIIa receptor inhibitors, and thrombectomy devices may be helpful when thrombus is present. Calcium channel blockers may be beneficial when embolization of plaque debris results in slow flow or no-flow during interventions. 相似文献
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恰如其分的外周髓鞘形成取决于雪旺细胞增殖与分化进程间的平衡。丝氨酸/苏氨酸激酶(mTOR)整合多种环境因素,是细胞生长、代谢、发挥作用的中枢调节者。本文报道了一种mTOR的负性调节剂——结节性硬化复合体(TSC1),通过控制细胞增殖和髓鞘稳态,建立了雪旺细胞谱系进展和髓鞘形成的阶段依赖性程序。小鼠雪旺细胞祖细胞中TSC1的解离导致mTOR信号通路激活,继而导致雪旺细胞过量增殖,分化受阻,髓鞘形成减少。转录组分析显示,TSC1突变体中的mTOR活化使得polo样激酶(PLK)依赖性通路和细胞周期调节剂上调。弱化mTOR或者对PLK进行药理抑制部分挽救了因TSC1缺失导致的外周神经发育过程中的髓鞘形成减少。相较之下,成年小鼠成熟雪旺细胞中TSC1缺失可导致髓鞘的过度增殖和过度生长。本文的发现提示了TSC1-mTOR-PLK信号轴在控制雪旺细胞的发育过程中,从增殖到分化和髓鞘内稳态中起到的阶段特异性功能。 相似文献
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Patient nonadherence to medication in inflammatory bowel disease 总被引:4,自引:0,他引:4
Sewitch MJ Abrahamowicz M Barkun A Bitton A Wild GE Cohen A Dobkin PL 《The American journal of gastroenterology》2003,98(7):1535-1544
OBJECTIVE: The aim of this study was to identify determinants of nonadherence to medication in outpatients with established inflammatory bowel disease (IBD). METHODS: Ten gastroenterologists and 153 of their IBD patients participated in this prospective study. Demographic, clinical, and psychosocial characteristics, as well as patient-physician discordance, were assessed at an office visit. Nonadherence to medication was assessed 2 wk later. Separate generalized estimating equations were used to identify determinants of nonadherence. RESULTS: Physicians averaged 47.9 yr in age (range 30.1-57.5 yr), and 90% were male. Patients averaged 37.0 yr (SD = 15.1), and 87 (56.9%) were female. In all, 63 patients (41.2%) were nonadherent to medication; of these, 51 (81.0%) indicated unintentional nonadherence, 23 (36.5%) intentional nonadherence, and 11 (17.5%) both. Overall nonadherence was predicted by disease activity (OR = 0.55, p = 0.0022), new patient status (OR = 2.14, p = 0.0394), disease duration (OR = 0.50, p = 0.0001), and scheduling a follow-up appointment (OR = 0.30, p = 0.0059), whereas higher discordance on well-being was predictive only in psychologically nondistressed patients (p = 0.0026 for interaction). Unintentional nonadherence was predicted by age (OR = 0.97, p = 0.0072), new patient status (OR = 2.80, p = 0239), and higher discordance on well-being in psychologically nondistressed patients (p = 0.0504). Intentional nonadherence was predicted by disease duration (OR = 0.55, p = 0032), scheduling a follow-up appointment (OR = 0.12, p = 0.0001), certainty that medication would be helpful (OR = 0.99, p = 0.0409), and total patient-physician discordance (OR = 1.59, p =.0120). CONCLUSIONS: These findings suggest that the therapeutic relationship, as well as individual clinical and psychosocial characteristics, influence adherence to medication. 相似文献