儿童先天性心脏病室间隔缺损致病因素分析

目的 :对儿童先天性室间隔缺损因素进行分析。方法 :报告 2 5 8例先天性室间隔缺损儿童 ,男性 14 4例 ,女性 114例 ,年龄 1～ 14岁。同时对 2 5 8例儿童的父母进行调查 ,调查对象为患先天性室间隔缺损儿童 ,儿童的父亲、母亲孕前和孕期多方面因素 ,将引起室间隔缺损的可能原因进行分析。结果 :发现家族中有遗传史 ,父母亲饮酒史 ,母孕期有感冒病史 ,母孕期服用抗病毒药物 ,父母孕期使用黄体酮及母孕期接触X射线 ,有较高先天性室间隔缺损的发病率。同时对患先天性室间隔缺损儿童血型分析 ,表明A型儿童和AB型儿童患先天性室间隔缺损频率较高 (分别为 4 2 2 5 %和 11 2 4 % )比值比(OR)分别为 1 6 3和 2 2 1。与对照组比较差异有显著性 (即A型 χ2 =7.0 2 ,P <0 0 0 1,AB型 χ2 =5 71,P <0 .0 5 )。A O ,AB O抗原患者组与对照组比较 ,OR分别为 1 72和 2 6 2 ,χ2 值分别为 6 72和 7 5 1均差异有显著性 (P <0 .0 0 1) ,而O型则相反患先天性室间隔缺损的机率相对减少。结论 :先天性室间隔缺损与家族遗传史、血型及父母孕期饮酒、服药等多方面因素有关。     

阵发性心房颤动局灶消融后复发病例的大静脉肌袖电隔离治疗

对肺静脉内点消融治疗术后随访 2 2± 1 1 .1个月内的心房颤动 (简称房颤 )复发病例 ,在Lasso标测导管指引下 ,进行再次心内电生理检查及大静脉肌袖电隔离治疗。探讨点消融治疗阵发性房颤后复发的机制 ,并对再次经验性大静脉肌袖电隔离治疗的结果进行分析与评价。 5例患者共接受心内电生理标测和电隔离治疗 6次 (一例行第二次电隔离 ) ,除一例行右下肺静脉靶肌袖的电隔离外 ,其余病例均进行了经验性全部大静脉的电隔离 ,共电隔离心脏大静脉 1 5根 ,其中肺静脉 1 3根、上腔静脉 2根 ,即刻电隔离成功率 1 0 0 %。术后随访 1 1 .8± 8.9个月 ,均无房颤复发。因此 ,对于阵发性房颤导管射频点消融后复发病例进行经验性全部大静脉肌袖电隔离治疗可以满意控制房颤的发作。     

主动固定螺旋电极在右室流出道间隔部起搏中的应用体会

目的探索采用主动固定螺旋电极行右室间隔部起搏的临床可行性。方法随机选择54例需要安装双腔起搏器的患者行右室流出道间隔部起搏,将心室起搏螺旋电极先后定位于右室心尖部及右室流出道间隔部并测试起搏参数。结果右室流出道间隔部电极定位成功率为98.15%,该部位起搏参数满足起搏要求,同时起搏的QRS波时限较心尖部变窄(130.45±18.24msvs153.11±20.10ms,P<0.001)。结论采用主动固定螺旋电极行右室流出道间隔部起搏安全性高、可行性好。     

Comparison of the Masaoka-Koga staging and the International Association for the Study of Lung Cancer/the International Thymic Malignancies Interest Group proposal for the TNM staging systems based on the Chinese Alliance for Research in Thymomas retrospective database

Background

To compare the predictive effect of the Masaoka-Koga staging system and the International Association for the Study of Lung Cancer (IASLC)/the International Thymic Malignancies Interest Group (ITMIG) proposal for the new TNM staging on prognosis of thymic malignancies using the Chinese Alliance for Research in Thymomas (ChART) retrospective database.

Methods

From 1992 to 2012, 2,370 patients in ChART database were retrospectively reviewed. Of these, 1,198 patients with complete information on TNM stage, Masaoka-Koga stage, and survival were used for analysis. Cumulative incidence of recurrence (CIR) was assessed in R0 patients. Overall survival (OS) was evaluated both in an R0 resected cohort, as well as in all patients (any R status). CIR and OS were first analyzed according to the Masaoka-Koga staging system. Then, they were compared using the new TNM staging proposal.

Results

Based on Masaoka-Koga staging system, significant difference was detected in CIR among all stages. However, no survival difference was revealed between stage I and II, or between stage II and III. Stage IV carried the highest risk of recurrence and worst survival. According to the new TNM staging proposal, CIR in T1a was significantly lower comparing to all other T categories (P<0.05) and there is a significant difference in OS between T1a and T1b (P=0.004). T4 had the worst OS comparing to all other T categories. CIR and OS were significantly worse in N (+) than in N0 patients. Significant difference in CIR and OS was detected between M0 and M1b, but not between M0 and M1a. OS was almost always statistically different when comparison was made between stages I–IIIa and stages IIIb–IVb. However, no statistical difference could be detected among stages IIIb to IVb.

Conclusions

Compared with Masaoka-Koga staging, the IASLC/ITMIG TNM staging proposal not only describes the extent of tumor invasion but also provides information on lymphatic involvement and tumor dissemination. Further study using prospectively recorded information on the proposed TNM categories would be helpful to better grouping thymic tumors for predicting prognosis and guiding clinical management.     

Contractile dysfunction in hypertrophied hearts with deficient insulin receptor signaling: possible role of reduced capillary density

Diabetics have worse outcomes than nondiabetics after a variety of cardiac insults. We tested the hypothesis that impaired insulin receptor signaling in myocytes worsens cardiac remodeling and function following injury, even in the absence of hyperglycemia. Mice with cardiomyocyte-restricted knock out of the insulin receptor (CIRKO) and wild type (WT) mice were treated with isoproterenol (ISO) for 2 or 5 days. Heart rates and cardiac mass increased comparably following ISO in WT and CIRKO mice. After 5 days, WT hearts were hyperdynamic by echocardiographic and left ventricular pressure measurements. However, CIRKO hearts had a blunted increase in contractility and relaxation following ISO. Interestingly, single myocytes isolated from both CIRKO ISO and WT ISO hearts had increased cellular shortening with prolonged time to peak shortening vs. respective shams. Thus, loss of myocytes or extramyocyte factors, rather than intrinsic dysfunction of surviving myocytes, caused the blunted inotropic response in ISO treated CIRKO hearts. Indeed, CIRKO ISO mice had increased troponin release after 2 days and greater interstitial and sub-endocardial fibrosis at 5 days than did ISO WT. Apoptosis assessed by TUNEL and caspase staining was increased in CIRKO ISO compared to WT ISO hearts; however, very few of the apoptotic nuclei were clearly in cardiac myocytes. After 5 days of ISO treatment, VEGF expression was increased in WT but not in CIRKO hearts. In keeping with this finding, capillary density was reduced in CIRKO ISO relative to WT ISO. Basal expression of hypoxia-inducible factor-1alpha was lower in CIRKO vs. WT hearts and may explain the blunted VEGF response. Thus, absence of insulin receptor signaling in the cardiac myocyte worsens catecholamine-mediated myocardial injury, at least in part, via mechanisms that tend to impair myocardial blood flow and increase ischemic injury.     

Switch from ritonavir-boosted to unboosted atazanavir guided by therapeutic drug monitoring

Plasma concentration of atazanavir (ATV) may be reduced when coadministered with tenofovir (TDF) or proton pump inhibitors. Boosting ATV exposure with ritonavir (r) may make it possible to overcome these drug interactions. However, jaundice and loss of the metabolic advantages of ATV are more frequent using ATV/r than ATV alone. Herein, we assessed whether therapeutic drug monitoring (TDM) could make it possible to identify the subset of patients in whom removal of ritonavir could be attempted without risk of suboptimal plasma ATV exposure and subsequent virological failure. A total of 56 patients with undetectable plasma HIV-RNA under a stable triple regimen containing ATV 300/100 mg qd were switched to ATV 400 mg qd. Plasma ATV concentrations were measured using a reliable high-performance liquid chromatography method. Median plasma ATV C(min) fell from 880 to 283 ng/ml (p = 0.03) after removal of ritonavir. While all patients on ATV/r showed ATV plasma concentrations within therapeutic values (IC(min) above 150 ng/ml) before switching, four patients (7%) fell below this threshold after switching to ATV 400 mg qd. However, only one of this group experienced virological failure at week 24 of follow-up. TDF was part of the antiretroviral regimen in all four cases. From a total of 29 (52%) patients on ATV/r showing grade 3-4 hyperbilirubinemia, only 7 (12%) remained on it upon switching to ATV 400 mg qd (p < 0.001). Patients with complete viral suppression under ATV/r 300/100 mg qd may benefit from switching to ATV 400 mg qd guided by TDM, which may make it possible to minimize adverse events without compromising antiviral efficacy in most cases.     

Family history of diabetes and risk of atherosclerotic cardiovascular disease in Korean men and women

The importance of family history of type 2 diabetes (FHD) as a risk factor for atherosclerotic cardiovascular disease (ASCVD) remains controversial. A report of diabetes in parents and siblings was used to establish FHD in a cohort of 1,005,230 Koreans aged 30-95 years insured by the National Health Insurance Corporation who had a biennial medical evaluation during 1992-1995. ASCVD morbidity and mortality from 1993 to 2005 were examined in relation to FHD and other ASCVD risk factors. The risk of ischemic heart disease (IHD) increased significantly (19%) in men with FHD but not in women. A strong interaction was observed between FHD and personal history of diabetes for the occurrence of ASCVD; men with both diabetes and FHD were at significantly increased risk of developing IHD, cerebrovascular disease and ASCVD with hazard ratios (HR) of 2.28, 2.07, and 2.12, respectively, compared to those who had neither FHD nor type 2 diabetes. Corresponding risks were 2.64, 2.03, and 2.10 in women, respectively. This study demonstrates that risk of ASCVD is increased among those with diabetes and a family history of diabetes; suggesting that genetic factors associated with occurrence of familial diabetes may increase risk of ASCVD beyond the risk among people without FHD.     

金属蛋白酶-9及其抑制物-1在实验性心动过速性心肌病中的变化

目的观察心动过速性心肌病(TIC)时金属蛋白酶(MMP)-9及其抑制剂(TIMP)-1的变化。方法对11只犬采用快速右房起搏建立TIC模型组,6只犬作假手术组(对照组)。应用超声心动图和心导管检查测量在窦性心律下的血流动力学数据。应用电子显微镜观察左室组织的超微结构改变。MMP-9及TIMP-1的相对变化通过免疫印迹法获得。结果8周后,与对照组相比,TIC组犬左室舒张末期容积及收缩末期容积均显著增加(P<0.05);左室射血分数在起搏1周后显著减小(P<0.05)。左室压力最大上升速率显著下降,最大下降速率绝对值显著下降(P<0.05和0.001),左室舒张末期压显著增大,左室松弛时间常数明显延长(P<0.05),二尖瓣血流频谱A波峰值流速和E波峰值流速均显著减小。心肌细胞超微结构发生变化,同时MMP-9增加,TIMP-1减少。结论快速右房起搏可制造TIC模型,TIC时MMP-9增加,TIMP-1减少。     

多层螺旋CT门静脉血管成像与内镜对食管、胃静脉曲张诊断的比较研究

目的对比研究多层螺旋CT门静脉血管成像(CTP)和内镜对食管、胃静脉曲张的诊断。方法采用16排多层螺旋CT门静脉血管成像,对57例临床和实验室检查提示门静脉高压的患者进行CTP,观察门静脉及其分支走形、分布,并结合横断面图像仔细观察食管和胃有无静脉曲张,并记录其部位、曲张静脉深浅、观察曲张静脉来源、有无其他部位曲张静脉或其他病理情况。同期对这些病例行胃镜检查,观察食管、胃是否存在静脉曲张及其他病变。结果CTP显示食管及胃静脉曲张病例51例,其中胃底静脉曲张合并食管静脉曲张39例,单纯胃底静脉曲张9例,胃底静脉曲张合并胃体静脉曲张3例。内镜发现食管及胃底静脉曲张46例,其中胃静脉曲张合并食管静脉曲张24例,单纯胃静脉曲张5例,单纯食管静脉曲张17例。CTP可发现内镜无法诊断的肌层或管腔外静脉曲张。结论CTP可清晰显示门静脉高压患者食管、胃底静脉曲张及主要侧支血管的走行、分布。在食管静脉曲张检查方面,CTP与内镜效果相当,CTP有几例假阳性,可作为普通内镜检查的良好补充;对于胃静脉曲张的检查,CTP效果较胃镜敏感,且CTP可清晰显示内镜无法观察的胃腔周围和食管周围静脉曲张。     

Polytopic and recurrent reflex sympathetic dystrophy in lower limbs in two siblings

Reflex sympathetic dystrophy (RSD) has been related to a variety of inciting and predisposing factors. However, there are few reports of a familiar or genetic background in RSD. This paper describes two cases of RSD polytopic and recurrent in lower limbs of two brothers with similar HLA.