难治性儿童朗格汉斯组织细胞增生症的治疗进展

难治性朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)是指具有多脏器浸润尤其是具有危险器官(骨髓、肝、脾)浸润和初治治疗失败的LCH病例,预后极差,及时有效的治疗是患者存活的关键.目前有单药克拉屈滨(2-chlorodeoxyadenosine,2-CdA)、2-CdA联合大剂量阿糖胞苷、消炎痛、造血干细胞移植、靶向治疗等多种二线治疗方案.该文就近年儿童难治性LCH的相关治疗方案进展进行综述.     

多靶点治疗难治性成人肾病综合征疗效观察

目的：探讨多靶点治疗对难治性成人肾病综合征(RNS)患者肾功能的影响。方法选取2012年5月至2014年5月我院肾内科收治的78例RNS患者作为研究对象,采用数字随机表法分为对照组和观察组各39例,均给予常规治疗,观察组给予泼尼松+霉酚酸酯+他克莫司治疗,对照组给予泼尼松+环磷酰胺,治疗前后检测两组患者24 h尿蛋白定量、血清白蛋白(ALB)、血肌酐(Scr)水平,以及白细胞介素-8(IL-8)、尿白细胞介素-6(IL-6)及T淋巴细胞水平。评估两组患者的疗效及用药安全性。结果观察组患者治疗有效率为71.79%(28/39),明显高于对照组的41.03%(16/39),差异具有统计学意义(P<0.05);观察组患者治疗后24 h尿蛋白定量、Scr水平明显低于对照组[(0.71±0.82) g/24 h vs (1.30±1.06) g/24 h、(70.26±9.66)μmol/L vs (76.27±10.28)μmol/L],ALB明显高于对照组[(36.87±6.29) g/L vs (32.04±5.28) g/L],差异均具有统计学意义(P<0.05);观察组患者治疗后的IL-6、IL-8水平均明显下降,且低于对照组[(42.34±9.37) pg/ml vs (63.68±8.23) pg/ml、(59.86±9.85) pg/ml vs (81.06±5.29) pg/ml],差异均具有统计学意义(P<0.05);观察组患者治疗后的CD3+、CD4+、CD4+/CD8+均明显高于对照组[(63.39±5.29)%vs (58.67±6.07)%、(36.11±5.16)%vs (31.05±5.97)%、(1.53±0.71)%vs (1.27±0.86)%],差异均具有统计学意义(P<0.05)。观察组和对照组的不良用药反应率分别为25.64%(10/39)和30.77%(12/39),差异无统计学意义(χ2=0.25,P>0.05)。结论泼尼松+霉酚酸酯+他克莫司多靶点治疗RNS能够改善肾功能,维持机体免疫功能,提高临床疗效。     

他克莫司与甲氨蝶呤联合治疗难治性类风湿关节炎效果观察

摘 要 目的：探讨他克莫司与甲氨蝶呤联合治疗难治性类风湿关节炎的临床效果。方法：80例难治性类风湿关节炎患者随机分为观察组和对照组,每组40例。观察组给予他克莫司与甲氨蝶呤联合治疗,对照组给予环磷酰胺与甲氨蝶呤联合治疗,两组均治疗24周。观察两组患者治疗前后关节疼痛指数、关节肿胀指数及晨僵时间的变化,采用视觉模拟评分（VAS ）对患者疼痛情况进行评估;评价两组临床疗效;比较两组患者治疗前后红细胞沉降率（ESR）、C 反应蛋白（CRP）、肿瘤坏死因子α（TNF α）、血管内皮生长因子（VEGF）等指标变化。结果：观察组总缓解率为100.00%,显著高于对照组的67.50%（P<0.05）。治疗后两组关节疼痛指数、关节肿胀指数、晨僵时间及VAS 评分均较治疗前显著降低（P<0.05）,且观察组各项指标均明显低于对照组（P<0.05）。两组治疗后ESR、CRP、TNF α、VEGF均较治疗前显著降低（P<0.05）,且观察组明显低于对照组（P<0.05）。结论：他克莫司与甲氨蝶呤联合治疗难治性类风湿关节炎临床疗效好,安全性高,其作用机制可能与降低患者体内炎症反应及下调VEGF水平有关。     

支气管胸膜瘘合并难治性脓胸个体化综合治疗分析

目的总结和分析支气管胸膜瘘合并难治性脓胸个体化综合治疗的方法和疗效。 方法2015年7月—2019年11月共收治支气管胸膜瘘合并难治性脓胸患者12例,其中男性10例,女性2例;年龄23～78岁,平均(60.75±15.05)岁。初始手术为非小细胞肺癌4例,肺部良性疾病4例,包裹性胸腔积液3例,恶性胸腺瘤肺转移1例。胸膜瘘发生后,采取经脓胸引流控制感染和介入封堵瘘口治疗;病情基本稳定后,行肌瓣移植术填塞脓腔等综合措施,并根据病情进行个体化治疗。 结果全组无围手术期死亡病例;在平均17.45个月的随访中,1例患者因肿瘤转移死亡;1例复发小脓腔;其余10例无支气管胸膜瘘和脓胸复发,肌瓣存活,复查胸部CT/MRI显示脓腔被彻底填塞。 结论对支气管胸膜瘘合并难治性脓胸患者采取个体化综合治疗措施,能达到彻底根治的目的,成功率高,疗效显著,值得推广。     

Lacosamide as an adjunctive therapy in pediatric patients with refractory focal epilepsy

Purpose: To evaluate the efficacy and safety of lacosamide in pediatric patients with refractory focal epilepsy. Methods: We reviewed retrospectively the medical records of children younger than 18 years of age treated at Seoul National University Bundang Hospital, in whom oral lacosamide was used as an adjunctive treatment for refractory focal epilepsy. Clinical information regarding the patients’ epilepsy and the outcome of lacosamide treatment was gathered and analyzed. Results: Twenty-one patients (16 boys, 5 girls) were included, with a median age of 13.9 (range, 1.2–17.9) years. The mean number of concomitant antiepileptic drugs was 3.0 (range, 1–6) and the mean duration of follow-up was 10.1 (range, 6.1–13.0) months. The mean maintenance dose of lacosamide was 5.4 (range, 1.4–9.8) mg/kg/day. Fourteen patients (67%) were responders; four of these were seizure free at the last follow-up. Seven patients (33%) were nonresponders: two of these presented with <50% seizure reduction and five showed no change in seizure frequency. Two patients (10%) discontinued oral lacosamide because of adverse events (aggressive behavior and depression). Mild transient treatment-related adverse events were observed in eight of the 21 patients (38%). Conclusions: Lacosamide represents a useful drug that is effective for a wide range of pediatric refractory focal epilepsy and is well tolerated.     

曲美布汀及双歧三联活菌联合抗焦虑抑郁药治疗难治性腹泻型肠易激综合征的疗效观察

目的:观察曲美布汀及双歧三联活菌联合抗焦虑抑郁药氟哌噻吨美利曲辛片治疗伴有焦虑和(或)抑郁的难治性腹泻型肠易激综合征(RIBS-D)的临床疗效。方法:101例伴有焦虑和(或)抑郁的RIBS-D患者随机分为试验组(52例)与对照组(49例),两组均给予曲美布汀及双歧三联活菌,试验组加服氟哌噻吨美利曲辛片,对照组加服安慰剂,疗程4周。观察治疗前后两组患者症状严重程度、精神状态、生活质量的变化及药品不良反应。结果:试验组与对照组的总有效率分别为86.54%、12.24%(P<0.01);试验组焦虑及抑郁积分较对照组显著降低,生活质量积分较对照组显著升高,两者差异均有统计学意义(P<0.01);治疗过程中未发生严重不良事件。结论:曲美布汀及双歧三联活菌联合抗焦虑抑郁药氟哌噻吨美利曲辛片治疗伴有焦虑和(或)抑郁的RIBS-D患者,能明显改善其症状、焦虑抑郁状态及生活质量,且十分安全。     

低分子肝素联合激素、环磷酰胺治疗难治性肾病综合征临床效果评价

目的：分析低分子肝素联合激素、环磷酰胺治疗难治性肾病综合征的临床效果。方法收集2011年1月～2014年1月收治的96例难治性肾病综合征患者的临床资料,随机分为观察组与对照组,各48例,对照组给予激素、环磷酰胺治疗,观察组采取低分子肝素联合激素、环磷酰胺治疗。观察两组患者临床疗效。结果观察组患者治疗的总有效率为93.75%,明显优于对照组（75.00%）,差异有统计学意义（P＜0.05）;观察组患者的24h 尿蛋白定量、尿素氮、肌酐水平等指标的改善均明显优于对照组,差异有统计学意义（P＜0.05）。结论低分子肝素联合激素、环磷酰胺治疗难治性肾病综合征,能改善患者的临床症状,疗效显著,值得推广。     

微量注射泵泵入硝酸甘油、多巴胺、多巴酚丁胺治疗难治性心力衰竭的临床效果观察

目的：观察应用微量注射泵泵入硝酸甘油、多巴胺、多巴酚丁胺治疗难治性心力衰竭的临床疗效。方法选择2011年1月~2014年1月本院102例心功能Ⅲ~Ⅳ级(NYHA分级法)的住院患者,在卧床休息、控制饮食、吸氧、强心剂及利尿剂治疗的基础上,应用微量注射泵泵入硝酸甘油、多巴胺、多巴酚丁胺,疗程3~5d,观察患者临床症状、体征、心功能改善情况。结果102例患者用药后临床症状和心功能各项参数均明显改善,与治疗前比较,差异有统计学意义(P<0.05)。结论应用微量泵泵入硝酸甘油、多巴胺、多巴酚丁胺治疗难治性心力衰竭患者疗效明显,安全可靠。     

Mechanisms of exercise-induced asthma

In a previous review in this journal McFadden eloquently presented the findings which led him and his colleagues to propose that respiratory heat loss and the subsequent cooling of the airways are the initial reaction sequence leading to airway obstruction in hyperventilation and exercise-induced asthma [62]. He further concluded that: “Exercise per se is not essential and serves only as means to increase ventilation”. Our interpretation of currently available data has led us to conclude that while respiratory heat loss may play an important permissive role in initiating the bronchoconstriction which follows exercise, the weight of evidence indicates that exercise per se serves as the trigger mechanism and is not just a tool to increase ventilation. Moreover, we believe that the role of exercise in releasing chemical mediators has been established, although pathways by which the airway smooth muscle is affected are still uncertain.