顾锡镇教授从脾肾论治肌萎缩侧索硬化症经验

肌萎缩侧索硬化症是一种病因不清的致死性神经系统罕见病，临床表现为进行性加重的肌无力、肌肉萎缩及延髓麻痹，最终累及呼吸肌致呼吸衰竭而死亡。顾锡镇教授从脾肾论治，运用健脾补肾法治疗该病在延缓疾病进展与改善生活质量方面取得了一定疗效。文章总结了顾教授辨治肌萎缩侧索硬化症的临床经验，并例举1个典型医案加以佐证。     

垂黄清脉冲剂干预治疗冠心病稳定型心绞痛热毒痰瘀证患者NF-κB信号通路的研究

目的 探讨垂黄清脉冲剂对冠心病稳定型心绞痛（热毒痰瘀证）患者NF-κB信号通路的调控。方法 本试验研究方法采用单盲、随机、对照法，选取就诊于宁夏医科大学总医院临床诊断为冠心病稳定型心绞痛（热毒痰瘀证）患者94例，将其随机分入对照组48例和试验组46例。两组均给予内科常规西药治疗，对照组加服银丹心脑通软胶囊，试验组加服垂黄清脉冲剂，疗程为3个月。于治疗前、后记录患者的基本情况，并采集血清样本，用酶联免疫吸附测定法（Elisa法）检测血清中TNF-α、NF-κB、IL-6、ICAM-1、VCAM-1、ET-1、NO浓度水平进行对比，用SPSS22.0将数据进行统计学分析。结果 与治疗前比较，两组治疗后Gensini积分、血清中TNF-α、NF-κB、IL-6、ICAM-1、VCAM-1、ET-1浓度水平均较治疗前显著降低，NO浓度水平显著升高（P < 0.05，P < 0.01），其中试验组显著程度尤为明显（P < 0.01）。与治疗后对照组比较，试验组Gensini积分、血清中TNF-α、NF-κB、IL-6、ICAM-1、VCAM-1、ET-1浓度水平降低，NO浓度水平升高趋势更明显（P < 0.01）。结论 垂黄清脉冲剂治疗冠心病稳定型心绞痛（热毒痰瘀证）在改善患者症状；改善冠状动脉管腔狭窄程度；通过降低炎症标志物TNF-α、NF-κB、IL-6、ICAM-1、VCAM-1的水平，减小ET-1/NO比值，从而对NF-κB信号通路进行部分调控，以有效抑制或截断血管内皮的炎症级联反应过程，清除炎症损伤因素以及保护血管内皮功能等方面均有显著优势，临床疗效确切值得肯定和推广应用。     

完全型雄激素不敏感综合征2例临床特点及处理

雄激素不敏感综合征(androgen insensitivity syndrome,AIS)又称为睾丸女性化综合征(testicular feminization syndrome,TFS),是一种X连锁遗传病,是男性假两性畸形中较常见的类型,可分为完全型AIS和不完全型AIS,其原因主要是雄激素受体(androgen receptor,AR)基因的突变导致其对雄激素产生抵抗和不应答。本文回顾南京医科大学附属妇产医院2例CAIS患者的临床资料及诊疗过程,以期能进一步提高对该病的认知及诊治水平。     

外源性神经节苷脂相关性吉兰—巴雷综合征的发病机制及诊治研究进展

吉兰―巴雷综合征（GBS）是一种以快速进行性四肢麻木无力为特点的急性炎性脱髓鞘性多发性多神经根神经病。随着外源性神经节苷脂在临床的广泛应用，该药引起的副作用也逐渐显现，外源性神经节苷脂相关性GBS是其最严重的并发症，临床上主要表现为轴索型GBS，以四肢弛缓性瘫痪为首发症状，表现为急性、严重且快速进展的周围神经受累，较其他轴索型GBS病情重，恢复时间长，预后差。目前发病机制尚不明确。静脉注射人免疫球蛋白是其具有循证医学证据的治疗方法，目前已取代血浆置换成为GBS首选治疗方法，推荐剂量为0.4 g/（kg·d），连续静滴5 d，大剂量激素治疗的效果还有待进一步探讨。早发现、早诊断、尽早停用外源性神经节苷脂、及时应用人血免疫球蛋白冲击治疗和康复治疗，可改善预后。     

Gray platelet syndrome: Management of perioperative bleeding in redo cardiac surgery

Gray platelet syndrome (GPS) is a rare (<1/1 000 000) and inherited platelet function disorder characterized by macrothrombocytopenia, α-granule deficiency, and hemorrhages. Bleeding intensity does not correlate with platelet count nor with functional test results. We hereby describe the perioperative bleeding prevention and management of a patient with GPS requiring multiple redo cardiac surgeries.     

Searching for Serum Antibodies to Neuronal Proteins in Patients With Myalgic Encephalopathy/Chronic Fatigue Syndrome

PurposeA role for the immune system in causing myalgic encephalopathy/chronic fatigue syndrome (ME/CFS) is long suspected, but few studies have looked for specific autoantibodies that might contribute to the symptoms. Our aim was to look for evidence of antibodies to neuronal proteins in patients with ME/CSF.MethodsSera samples from 50 patients and 50 healthy individuals were sent coded to the Neuroimmunology Laboratory in Oxford. Screening for antibody binding to neuronal tissue was performed on brain tissue and neuronal cultures. Specific serum antibodies were assessed by antigen-specific cell-based assays and radioimmunoassays. After antibody testing, the associations between seropositive status and clinical data were investigated.FindingsOverall, 8 patients and 11 participants were found to have some serum immunoreactivity toward neuronal or neuromuscular junction proteins, but only 1 patient and 2 participants had specific serum antibodies. Nevertheless, seropositive status in patients with ME was associated with shorter duration since onset and a more severe disease.ImplicationsThe results indicate no overall increased frequency of antibodies to neuronal proteins in ME/CSF and no evidence of a specific antibody that might be causative or contribute to clinical features in patients. However, the association of seropositive status with shorter duration of disease and more severe symptoms suggests a possible role of antibodies at onset in some patients and should be the focus of future studies.     

Risk factors associated with postoperative prosthetic graft patency in Leriche syndrome

Background

In Leriche syndrome, postoperative graft thrombosis remains one of the most significant clinical challenges.