目的探究成年血友病患者的骨健康状况及骨密度可能相关影响因素,以期为改善其骨健康状态,防治及康复干预骨质疏松症提供参考。方法本研究为回顾性分析。选取2022年7月至2024年2月在北京协和医院康复医学科就诊的成年男性A型血友病患者...目的探究成年血友病患者的骨健康状况及骨密度可能相关影响因素,以期为改善其骨健康状态,防治及康复干预骨质疏松症提供参考。方法本研究为回顾性分析。选取2022年7月至2024年2月在北京协和医院康复医学科就诊的成年男性A型血友病患者为研究对象。采用双能X线吸收法(dual energy X-ray absorptiometry,DXA)和高分辨率外周定量计算机扫描(high-resolution peripheral quantitative computed tomography,HR-pQCT)法对患者的骨密度(bone mineral density,BMD)进行检查。对患者左踝关节进行正侧位X线检查及Pettersson放射学评分。采用血友病关节健康评估表(haemophilia joint health score,HJHS)2.1版计算左踝HJHS评分并评价患者的关节健康状况。检测血清骨代谢指标包括总Ⅰ型前胶原氨基端肽(total procollagenⅠN-terminal propeptide,TP1NP)、β-胶原Ⅰ型胶原交联羧基末端肽(C-terminal crosslinkingβ-isomerized carboxy-telopeptide of typeⅠcollagen,β-CTX)和25-羟维生素D_(3)[25-(OH)D_(3)]。检测患者身体成分指标,包括统计体质量指数(body mass index,BMI)和骨骼肌质量指数(skeletal muscle mass index,SMI)。对BMD检测值及其可能的影响因素进行相关性分析。结果共入选33例成年男性A型血友病患者,包括重型22例,中型11例,平均年龄(31.1±8.4)岁。入选患者髋部BMD不同程度低于年龄预计值,最小Z值为-3.0。左踝关节Pettersson评分为(7.75±0.94)分,HJHS评分为(8.88±1.25)分。33.3%的患者β-CTX增高。33.3%的患者血清25-(OH)D_(3)缺乏,45.5%的患者25-(OH)D_(3)不足。21.2%的患者肥胖,42.4%的患者超重。21.2%的患者SMI值达到肌少症的诊断标准。DXA法股骨颈、Wards三角BMD与左踝HJHS评分呈负相关(r=-0.814,P=0.014;r=-0.733,P=0.039)。此外,β-CTX与TP1NP呈正相关(r=0.845,P=0.004)。结论成年血友病患者髋部BMD降低,关节功能障碍可能是血友病患者BMD降低的相关因素之一,其破骨细胞活性的增加可能伴随着代偿性的成骨细胞活性增加。展开更多
目的探索正念减压训练(mindfulness-based stress reduction,MBSR)法对空军青少年航空学校(Teenagers Aviation School of Air Force,TASAF)学生非病理性血压升高的改善作用。方法纳入2022年11月TASAF高一年度复查初测血压升高超标学生...目的探索正念减压训练(mindfulness-based stress reduction,MBSR)法对空军青少年航空学校(Teenagers Aviation School of Air Force,TASAF)学生非病理性血压升高的改善作用。方法纳入2022年11月TASAF高一年度复查初测血压升高超标学生共241名,将其随机分入MBSR组(n=121)和自行放松组(n=120)。比较2组学生临检时收缩压、舒张压和心率超标的改善程度,本研究设计量表调查受检学生身体疲劳-心理紧张积累情况。结果不同身体疲劳-心理紧张程度积分的TASAF学生初测收缩压测量值差异有统计学意义(F=15.670,P=0.012),事后两两比较结果显示身体疲劳-心理紧张程度积分越高,受检学生初测收缩压测量值越高(P均<0.05)。2组受检学生放松前的收缩压、舒张压和心率差异均无统计学意义(P均>0.05)。2组按照规定方法进行放松调节并按规定流程复测血压后,MBSR组学生放松前后收缩压差值、舒张压差值和心率差值均高于自行放松组,差异均有统计学意义(t=6.873、5.144、3.651,P=0.001、0.002、0.013)。结论MBSR法比自行放松法能更有效地改善TASAF学生血压和心率升高超标情况。可在TASAF学生中推广MBSR法,并在日常课程中加入MBSR训练内容,以提升血压和心率调节能力。展开更多
BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated w...BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.展开更多
文摘目的探究成年血友病患者的骨健康状况及骨密度可能相关影响因素,以期为改善其骨健康状态,防治及康复干预骨质疏松症提供参考。方法本研究为回顾性分析。选取2022年7月至2024年2月在北京协和医院康复医学科就诊的成年男性A型血友病患者为研究对象。采用双能X线吸收法(dual energy X-ray absorptiometry,DXA)和高分辨率外周定量计算机扫描(high-resolution peripheral quantitative computed tomography,HR-pQCT)法对患者的骨密度(bone mineral density,BMD)进行检查。对患者左踝关节进行正侧位X线检查及Pettersson放射学评分。采用血友病关节健康评估表(haemophilia joint health score,HJHS)2.1版计算左踝HJHS评分并评价患者的关节健康状况。检测血清骨代谢指标包括总Ⅰ型前胶原氨基端肽(total procollagenⅠN-terminal propeptide,TP1NP)、β-胶原Ⅰ型胶原交联羧基末端肽(C-terminal crosslinkingβ-isomerized carboxy-telopeptide of typeⅠcollagen,β-CTX)和25-羟维生素D_(3)[25-(OH)D_(3)]。检测患者身体成分指标,包括统计体质量指数(body mass index,BMI)和骨骼肌质量指数(skeletal muscle mass index,SMI)。对BMD检测值及其可能的影响因素进行相关性分析。结果共入选33例成年男性A型血友病患者,包括重型22例,中型11例,平均年龄(31.1±8.4)岁。入选患者髋部BMD不同程度低于年龄预计值,最小Z值为-3.0。左踝关节Pettersson评分为(7.75±0.94)分,HJHS评分为(8.88±1.25)分。33.3%的患者β-CTX增高。33.3%的患者血清25-(OH)D_(3)缺乏,45.5%的患者25-(OH)D_(3)不足。21.2%的患者肥胖,42.4%的患者超重。21.2%的患者SMI值达到肌少症的诊断标准。DXA法股骨颈、Wards三角BMD与左踝HJHS评分呈负相关(r=-0.814,P=0.014;r=-0.733,P=0.039)。此外,β-CTX与TP1NP呈正相关(r=0.845,P=0.004)。结论成年血友病患者髋部BMD降低,关节功能障碍可能是血友病患者BMD降低的相关因素之一,其破骨细胞活性的增加可能伴随着代偿性的成骨细胞活性增加。
文摘目的探索正念减压训练(mindfulness-based stress reduction,MBSR)法对空军青少年航空学校(Teenagers Aviation School of Air Force,TASAF)学生非病理性血压升高的改善作用。方法纳入2022年11月TASAF高一年度复查初测血压升高超标学生共241名,将其随机分入MBSR组(n=121)和自行放松组(n=120)。比较2组学生临检时收缩压、舒张压和心率超标的改善程度,本研究设计量表调查受检学生身体疲劳-心理紧张积累情况。结果不同身体疲劳-心理紧张程度积分的TASAF学生初测收缩压测量值差异有统计学意义(F=15.670,P=0.012),事后两两比较结果显示身体疲劳-心理紧张程度积分越高,受检学生初测收缩压测量值越高(P均<0.05)。2组受检学生放松前的收缩压、舒张压和心率差异均无统计学意义(P均>0.05)。2组按照规定方法进行放松调节并按规定流程复测血压后,MBSR组学生放松前后收缩压差值、舒张压差值和心率差值均高于自行放松组,差异均有统计学意义(t=6.873、5.144、3.651,P=0.001、0.002、0.013)。结论MBSR法比自行放松法能更有效地改善TASAF学生血压和心率升高超标情况。可在TASAF学生中推广MBSR法,并在日常课程中加入MBSR训练内容,以提升血压和心率调节能力。
基金Supported by Traditional Chinese Medicine Research Program of Hebei Provincial Administration of Traditional Chinese Medicine,No.2025313 and No.2025448.
文摘BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.
