Discoid lupus erythematosus

Discoid lupus erythematosus is a manifestation of chronic cutaneous lupus erythematosus with a small risk of systemic involvement. In this review article, the role of predisposing factors such as haplotype, hormones, antibodies and sunlight are discussed. The clinical features, including variants and associations, and management options are presented.     

Shedding of proangiogenic microvesicles from hypertrophic scar myofibroblasts

Hypertrophic scars are a common complication of burn injuries and represent a major challenge in terms of prevention and treatment. These scars are characterized by a supraphysiological vascular density and by the presence of pathological myofibroblasts (Hmyos) displaying a low apoptosis propensity. However, the nature of the association between these two hallmarks of hypertrophic scarring remains largely unexplored. Here, we show that Hmyos produce signalling entities known as microvesicles that significantly increase the three cellular processes underlying blood vessel formation: endothelial cell proliferation, migration and assembly into capillary‐like structures. The release of microvesicles from Hmyos was dose‐dependently induced by the serum protein α‐2‐macroglobulin. Using flow cytometry, we revealed the presence of the α‐2‐macroglobulin receptor—low‐density lipoprotein receptor‐related protein 1—on the surface of Hmyos. The inhibition of the binding of α‐2‐macroglobulin to its receptor abolished the shedding of proangiogenic microvesicles from Hmyos. These findings suggest that the production of microvesicles by Hmyos contributes to the excessive vascularization of hypertrophic scars. α‐2‐Macroglobulin modulates the release of these microvesicles through interaction with low‐density lipoprotein receptor‐related protein 1.     

Diastolic time intervals before and after nadolol in patients with hypertrophic cardiomyopathy

In 9 patients with hypertrophic cardiomyopathy, diastolic function was evaluated by noninvasive measurements of diastolic time intervals before and after nadolol administration. No significant variation of the intervals was observed after therapy. The method therefore appears scarcely useful in the evaluation of beta-blocker therapy in these patients.     

经冠状动脉消融肥厚心肌和外科治疗肥厚型梗阻性心肌病

目的　回顾性分析外科和经冠状动脉消融肥厚间隔心肌 (transcoronaryablationofseptalhypertrophy ,TASH)治疗肥厚型梗阻性心肌病 (hypertrophicobstructivecardiomyopathy ,HOCM )近、中期 (3～ 2 4个月 )的疗效和安全性。方法　 54例HOCM患者在我院分别接受外科 (11例 )或TASH(43例 )治疗。治疗前、后评价患者的主观症状和客观检查系列结果。结果　外科和TASH后即刻左室流出道压力阶差 (PGLVOT)下降显著 [外科 (75± 48)mmHg(1mmHg =0 13 3kPa) ,TASH(56± 2 8)mmHg] ,但下降率差异无显著意义 (76 8%比 74 0 % ,P >0 0 5)。6例PGLVOT 反弹 ,TASH后 5例 ,出现在术后 3～ 6个月 ,外科后 1例 ,出现在术后 2 4个月。术后患者主观症状改善显著 ,但改善程度二组患者差别不显著。围术期死亡 2例 ,分别死于脑出血 (外科 )和严重心动过缓 (TASH ,拒绝安装起搏器 )。结论　TASH治疗HOCM的近、中期效果与外科相似 ,与外科相比 ,其并发症也是可以接受的 ,远期效果及安全性还有待观察     

左心室中部肥厚型梗阻性心肌病临床及造影特征分析

目的 分析左心室中部肥厚型梗阻性心肌病患者的临床及心血管造影特征.方法 5例(男:女=3:2)临床诊断为左心室中部肥厚型梗阻性心肌病的患者,年龄16～73(44±22)岁.所有患者均接受左心导管及心血管造影检查,并记录左心室心尖部-左心室基底部-左心室流出道-升主动脉连续压力.结果 5例患者中胸闷气短者4例,其中2例合并晕厥.所有患者心前区均可闻及收缩期杂音,且均存在左心室壁肥厚,室间隔厚度为19～31(23.8±5.4)mm,左心室舒张末期横径为35～55(43.4±7.4)mm,左心室射血分数为53%～70%、平均为(64.2±6.9)%.5例患者均存在左心室高电压伴异常Q波,其中1例患者合并阵发性室性心动过速,另有1例患者合并完全性左束支传导阻滞.左心导管检查证实收缩期左心室中部均存在梗阻,其中1例患者同时合并左心室流出道梗阻.左心室中部收缩期压力阶差为45～102(68.6±24.1)mm Hg(1 mm Hg=0.133 kPa).心血管造影检查提示前降支中段肌桥1例,冠状动脉粥样硬化性心脏病1例.另外,2例患者合并左心室心尖部室壁瘤.结论 左心室中部肥厚型梗阻性心肌病的临床特征不同于其他类型的肥厚型心肌病,完善的左心导管检查及造影有利于诊断并指导临床治疗.     

经皮腔内室间隔心肌消融术对心电指标的影响

目的研究肥厚梗阻型心肌病患者经皮腔内室间隔心肌消融术对心电指标的影响。方法对50例肥厚梗阻型心肌病患者行经皮腔内室间隔心肌消融术,记录术前、术中和术后出现的心律失常类型,配对分析术前、术后心电指标的变化。结果术后与术前相比,QRS时限[(122.0±24.0)ms对(97.3±15.5)ms,P=0.000]明显延长,QTc[(469.3±32.2)ms对(434.3±41.5)ms,P=0.004]、PR间期[(169±26)ms对(162±24)ms,P=0.044]稍延长。术中心律失常发生率分别为:右束支传导阻滞70%(35/50),左束支传导阻滞8%(4/50),一过性AVB38%(19/50),频发室性早搏24%(12/50),短阵室性心动过速24%(12/50);未发生持续性室性心动过速和室颤。术后心律失常发生率分别为:右束支传导阻滞56%(28/50),左束支传导阻滞8%(4/50),交界区性心动过速4%(2/50),频发室性早搏16%(8/50),短阵室性心动过速8%(4/50)。无永久性起搏器植入及死亡病例。结论经皮腔内室间隔心肌消融术致心律失常的发生率高,右束支传导阻滞最为常见。严格选择适应证后谨慎地行PTSMA术是安全、可行的。     

FBP1 is highly expressed in human hypertrophic scars and increases fibroblast proliferation,apoptosis, and collagen expression

Purpose: FBP1, one of the far-upstream element binding proteins(FBPs), is a distal upstream binding protein of c-myc, which is highly expressed in tumor tissues. This study aimed to investigate FBP1 expression in human hypertrophic scars and to determine the effects of FBP1 on fibroblasts. Materials and methods: Human normal skin and scar specimens were collected during clinical surgery. One portion of each tissue specimen was embedded in paraffin and sliced to observe differences in histological features and FBP1 expression by immunohistochemistry and western blotting. The other portion of each tissue specimen was cultured to obtain fibroblasts. Fibroblasts from the second to the sixth passage were used for the experiments, which were divided into the following two groups: an experimental group, whose cells were transfected with an siRNA targeting FBP1, and a control group, whose cells where not transfected. MTT and TUNEL assays were performed, respectively, to assess fibroblast proliferation and apoptosis, and western blotting was performed to assess protein expression. Results: We obtained fibroblasts by primary tissue culture and found that FBP1 was highly expressed in hypertrophic scars. MTT assay showed that an siRNA targeting FBP1 significantly reduced fibroblast proliferation in siRNA-treated cells compared to control cells. TUNEL assay showed that there was no difference in apoptosis between the two groups; however, western blotting showed that collagen I, collagen III, c-myc, caspase-3, and caspase-9 expression levels were all decreased in the experimental group. Conclusion: FBP1 is highly expressed in human hypertrophic scars and increases fibroblast proliferation, apoptosis and collagen expression.     

肥厚型心肌病的局部心肌功能变化和分子遗传学基础

肥厚型心肌病是一类常染色体显性遗传性疾病,多数为编码心肌肌原纤维的基因突变所致.常见的突变基因为肌球蛋白重链基因、肌球蛋白结合蛋白C基因、肌钙蛋白基因、a-原肌球蛋白基因以及一些非肌小节基因.不同的基因突变导致的肥厚型心肌病其局部心肌收缩及舒张运动也出现不同程度的改变:收缩运动减弱;Ca2+敏感性增加,舒张功能减退.该文从遗传学角度阐述了以上常见基因突变造成的不同心肌功能变化,并从基因水平解释了不同基因突变造成不同心肌运动功能改变的原因.     

Electroanatomical Mapping‐Guided Endocardial and Epicardial Ablation of Sustained Ventricular Tachycardia Originating From Alcohol Septal Ablation‐Induced Scar in a Patient With Hypertrophic Obstructive Cardiomyopathy

Ventricular Tachycardia After Alcohol Septal Ablation. A 76‐year‐old female developed 2 different ventricular tachycardias (VTs) 5 years after alcohol septal ablation (ASA) for symptomatic hypertrophic obstructive cardiomyopathy. VT#1 was a small macroreentry at the anterior border of the low‐voltage zone, suggesting the ASA‐scar and eliminated by endocardial ablation at a site recording fractionated potentials covering the mid‐diastolic and presystolic periods. VT#2 was a focal VT and eliminated by epicardial cryoablation at the basal posterior left ventricle, suggesting the posterior border of the ASA‐scar. Using the electroanatomical mapping, we demonstrated that the mechanism of the VTs was reentry at the edge of the ASA‐scar. (J Cardiovasc Electrophysiol, Vol. 21, pp. 1296‐1299, November 2010)