目的报道1例蛋白C和蛋白S缺陷症致脑静脉血栓形成病例,总结临床诊疗思路。方法与结果男性患者,27岁,以头痛发病,平卧位加重、站立位稍缓解。影像学检查提示右侧横窦、乙状窦、窦汇血栓形成;实验室检查蛋白C和蛋白S活性降低(49%和51%),...目的报道1例蛋白C和蛋白S缺陷症致脑静脉血栓形成病例,总结临床诊疗思路。方法与结果男性患者,27岁,以头痛发病,平卧位加重、站立位稍缓解。影像学检查提示右侧横窦、乙状窦、窦汇血栓形成;实验室检查蛋白C和蛋白S活性降低(49%和51%),脑脊液压力>330 mm H_(2)O;基因检测提示PROC基因移码突变[c.574_577del(p.Val192Serfs*5)]及PROS1基因错义突变[c.1915T>G(p.Cys639Gly)和c.301C>T(p.Arg101Cys)]。予以低分子量肝素序贯新型口服抗凝药抗凝治疗以及降低颅内压、营养神经和镇痛治疗后,头痛症状缓解。出院后4个月头痛无复发,复查脑脊液压力降至正常(135 mm H_(2)O),蛋白C和蛋白S活性升至53%和54.10%(仍低于正常值范围)。结论蛋白C和(或)蛋白S缺乏是脑静脉血栓形成的重要病因,新型口服抗凝药可有效改善静脉回流障碍并避免华法林相关凝血机制紊乱,是优选治疗方案。临床应对青年患者行凝血蛋白活性检测及遗传学分析,以指导精准抗凝治疗。展开更多
The new technology of direct decomposition of H_(2)S into high value-added H_(2) and S,as an alternative to the Claus process in industry,is an ideal route that can not only deal with toxic and abundant H_(2)S waste g...The new technology of direct decomposition of H_(2)S into high value-added H_(2) and S,as an alternative to the Claus process in industry,is an ideal route that can not only deal with toxic and abundant H_(2)S waste gas but also recover clean energy H_(2),which has significant socio-economic and ecological advantages.However,the highly effective decomposition of H_(2)S at low temperatures is still a great challenge,because of the stringent thermodynamic equilibrium constraints(only 20% even at high temperature of 1010℃).Conventional microwave catalysts exhibit unsatisfactory performance at low temperatures(below 600℃).Herein,Mo_(2)C@CeO_(2) catalysts with a core-shell structure were successfully developed for robust microwave catalytic decomposition of H_(2)S at low temperatures.Two carbon precursors,para-phenylenediamine(Mo_(2)C-p)and meta-phenylenediamine(Mo_(2)C-m),were employed to tailor Mo_(2)C configurations.Remarkably,the H_(2)S conversion of Mo_(2)C-p@CeO_(2) catalyst at a low temperature of 550℃ is as high as 92.1%,which is much higher than the H_(2)S equilibrium conversion under the conventional thermal conditions(2.6% at 550℃).To our knowledge,this represents the most active catalyst for microwave catalytic decomposition of H_(2)S at low temperature of 550℃.Notably,Mo_(2)C-p demonstrated superior intrinsic activity(84%)compared to Mo_(2)C-m(6.4%),with XPS analysis revealing that its enhanced performance stems from a higher concentration of Mo_(2+)active sites.This work presents a substitute approach for the efficient utilization of H_(2)S waste gas and opens up a novel avenue for the rational design of microwave catalysts for microwave catalytic reaction at low-temperature.展开更多
文摘目的报道1例蛋白C和蛋白S缺陷症致脑静脉血栓形成病例,总结临床诊疗思路。方法与结果男性患者,27岁,以头痛发病,平卧位加重、站立位稍缓解。影像学检查提示右侧横窦、乙状窦、窦汇血栓形成;实验室检查蛋白C和蛋白S活性降低(49%和51%),脑脊液压力>330 mm H_(2)O;基因检测提示PROC基因移码突变[c.574_577del(p.Val192Serfs*5)]及PROS1基因错义突变[c.1915T>G(p.Cys639Gly)和c.301C>T(p.Arg101Cys)]。予以低分子量肝素序贯新型口服抗凝药抗凝治疗以及降低颅内压、营养神经和镇痛治疗后,头痛症状缓解。出院后4个月头痛无复发,复查脑脊液压力降至正常(135 mm H_(2)O),蛋白C和蛋白S活性升至53%和54.10%(仍低于正常值范围)。结论蛋白C和(或)蛋白S缺乏是脑静脉血栓形成的重要病因,新型口服抗凝药可有效改善静脉回流障碍并避免华法林相关凝血机制紊乱,是优选治疗方案。临床应对青年患者行凝血蛋白活性检测及遗传学分析,以指导精准抗凝治疗。
基金supported by the National Natural Science Foundation of China(22178295,21706225)Natural Science Foundation of Hunan Province(2025JJ50085)Hunan Collaborative Innovation Center of New Chemical Technologies for Environmental Benignity and Efficient Resource Utilization.
文摘The new technology of direct decomposition of H_(2)S into high value-added H_(2) and S,as an alternative to the Claus process in industry,is an ideal route that can not only deal with toxic and abundant H_(2)S waste gas but also recover clean energy H_(2),which has significant socio-economic and ecological advantages.However,the highly effective decomposition of H_(2)S at low temperatures is still a great challenge,because of the stringent thermodynamic equilibrium constraints(only 20% even at high temperature of 1010℃).Conventional microwave catalysts exhibit unsatisfactory performance at low temperatures(below 600℃).Herein,Mo_(2)C@CeO_(2) catalysts with a core-shell structure were successfully developed for robust microwave catalytic decomposition of H_(2)S at low temperatures.Two carbon precursors,para-phenylenediamine(Mo_(2)C-p)and meta-phenylenediamine(Mo_(2)C-m),were employed to tailor Mo_(2)C configurations.Remarkably,the H_(2)S conversion of Mo_(2)C-p@CeO_(2) catalyst at a low temperature of 550℃ is as high as 92.1%,which is much higher than the H_(2)S equilibrium conversion under the conventional thermal conditions(2.6% at 550℃).To our knowledge,this represents the most active catalyst for microwave catalytic decomposition of H_(2)S at low temperature of 550℃.Notably,Mo_(2)C-p demonstrated superior intrinsic activity(84%)compared to Mo_(2)C-m(6.4%),with XPS analysis revealing that its enhanced performance stems from a higher concentration of Mo_(2+)active sites.This work presents a substitute approach for the efficient utilization of H_(2)S waste gas and opens up a novel avenue for the rational design of microwave catalysts for microwave catalytic reaction at low-temperature.
