永钢高压气瓶钢4147(/%:0.46~0.50C,0.15~0.35Si,0.8~1.0Mn,≤0.015P,≤0.008S,0.85~1.10Cr,0.15~0.25Mo,0.02~0.04Al)的冶炼工艺为110 t EBT电弧炉-LF-VD-Φ500 mm圆坯连铸。通过使用炉料80%铁水+20%废钢,控制(Pb+As+Sn+Sb+Bi)≤150...永钢高压气瓶钢4147(/%:0.46~0.50C,0.15~0.35Si,0.8~1.0Mn,≤0.015P,≤0.008S,0.85~1.10Cr,0.15~0.25Mo,0.02~0.04Al)的冶炼工艺为110 t EBT电弧炉-LF-VD-Φ500 mm圆坯连铸。通过使用炉料80%铁水+20%废钢,控制(Pb+As+Sn+Sb+Bi)≤150×10^(-6),EAF终点[C]≥0.08%,终点[P]≤0.006%,并在出钢过程加1.0 kg/t Al;以及采用LF精炼合成渣(/%:40~55CaO,20~30Al_2O_3,≤6MgO,≤4.0SiO_2,≤1.5FeO),成品硫含量≤0.002%,T[O]≤17×10^(-6),[N]≤32×10^(-6),[H]≤0.9×10^(-6),(Pb+Sn+Sb+As+Bi)≤0.013 7%;连铸圆坯中心疏松、缩孔≤1.5级,轧材各类夹杂物均≤0.5级,满足高压气瓶钢质量要求。展开更多
Activated Phosphoinositide 3-kinase d syndrome(APDS)is a newly recognised primary immunodeficiency disease.It has currently been a hot topic of clinical research and new data are emerging regarding its pathogenesis,cl...Activated Phosphoinositide 3-kinase d syndrome(APDS)is a newly recognised primary immunodeficiency disease.It has currently been a hot topic of clinical research and new data are emerging regarding its pathogenesis,clinical manifestations and treatment.Patients with APDS syndrome have significant autoimmune manifestations and lymphoproliferation.It is important to differentiate APDS from the usual polygenic CVID in view of the availability of targeted therapy like mTOR inhibitors such as Rapamycin and selective PI3Kd inhibitors.We provide a comprehensive review on this interesting disorder focusing light on its etiology,genetic research and emerging therapy.展开更多
文摘Activated Phosphoinositide 3-kinase d syndrome(APDS)is a newly recognised primary immunodeficiency disease.It has currently been a hot topic of clinical research and new data are emerging regarding its pathogenesis,clinical manifestations and treatment.Patients with APDS syndrome have significant autoimmune manifestations and lymphoproliferation.It is important to differentiate APDS from the usual polygenic CVID in view of the availability of targeted therapy like mTOR inhibitors such as Rapamycin and selective PI3Kd inhibitors.We provide a comprehensive review on this interesting disorder focusing light on its etiology,genetic research and emerging therapy.