Myocardial ischemia and left ventricular dysfunction have been documented in young adults with familial hypercholesterolemia. We investigated whether speckle-tracking echocardiography can be used to detect subclinically impaired global and regional myocardial function in patients with this lipid disorder. This single-center study included 47 patients with familial hypercholesterolemia and 37 healthy control subjects who underwent transthoracic Doppler echocardiography and speckle-tracking echocardiography from January 2003 through December 2016. Conventional echocardiographic and strain parameters in the 2 groups were analyzed and compared. Left ventricular dimensions were significantly larger at end-diastole (P=0.02) and end-systole (P=0.013), left ventricular walls were significantly thicker (P <0.0001), and the early transmitral/early diastolic mitral annular velocity ratio was significantly higher (P=0.006) in the patient group than in the control group. In the patient group, global longitudinal and circumferential strain values were significantly lower (P <0.0001) and global radial strain values significantly higher (P=0.006); all segmental longitudinal strain (P <0.04) and most segmental circumferential strain values (P ≤0.01) were significantly lower; and some segmental radial strains, especially at the apex, were significantly higher (P ≤0.04). However, average longitudinal, circumferential, and radial strains in the different segments of the 3 main coronary artery territories were significantly lower in the patient group (P <0.01). Global longitudinal strain (r=0.561; P=0.001) and global circumferential strain (r=0.565; P <0.0001) were inversely correlated with low-density-lipoprotein cholesterol levels. We conclude that speckle-tracking echocardiography can be used to detect subclinical global and regional systolic abnormalities in patients with familial hypercholesterolemia.

目的 探讨肢体横纹肌肉瘤的超声诊断价值.方法 回顾性分析经手术病理确诊的22例肢体横纹肌肉瘤患者的临床、超声及影像学结果.结果 22例肿瘤病理分型:腺泡性19例,胚胎性2例,多形性1例.病变均位于肌层,其中大腿5例,小腿6例,上臂2例,前臂3例,手部6例.9例肿瘤呈不规则形,7例呈椭圆形,6例呈分叶形,22例肿瘤均未见明显包膜,13例边界清晰,9例肿瘤边界不清.肿瘤内部均表现为实性不均匀低回声,其中17例肿瘤内可见类似肌纤维纹理样高回声,4例肿瘤内可见液化坏死区,肿瘤内均未见钙化.多数肿瘤血流信号丰富(n = 20).频谱多普勒示肿瘤内部动脉阻力指数(RI)为0.77±0.09.结论 肢体横纹肌肉瘤的超声表现具有一定特征性,能为临床诊断提供重要信息.

患儿男,9岁,因"右踇趾伸直受限近2年"就诊.肌电图检查:右腓总神经损伤,踇长伸肌呈中度损伤表现.右膝正位X线片:右腓骨近端疣状骨性突起,边界清晰,内见骨小梁结构(图1A).MR检查:脂肪抑制T2WI见右腓骨近端骨性突起呈高信号(图1B),与腓骨相延续,周围组织受压.超声:右腓骨上段表面见1.91 cm×0.64 cm较宽基底强回声突起,边缘与腓骨皮质相延续,顶端可见低回声区,CDFI未见明显血流信号;腓总神经连续,局部受压隆起?变细,宽仅0.12 cm,神经近端增粗,宽0.22 cm(图1C);小腿外侧肌群回声增强,肌肉纹理结构不清.超声提示:右腓骨上段骨软骨瘤;腓总神经局部受压变细;右小腿外侧肌群萎缩.于局麻下行骨肿瘤切除术,术中见右腓骨近端外侧2.00 cm×1.24 cm×1.11cm"扁片样"骨软骨瘤,腓总神经受压变扁,色泽暗淡.术后病理诊断:右腓骨近端外侧骨软骨瘤(图1D).

Background: The leptin receptor-deficient db/db mouse is a well-established type II diabetes animal model used to investigate diabetic cardiomyopathy. Diabetes mellitus induces functional and structural abnormalities of the coronary microvascular circulation, which can play a role in the development of diabetic cardiomyopathy. The objective of this study was to use vasodilator stress echocardiography to assess coronary microvascular function in vivo with diabetic db/db mice in order to study early changes of the coronary microvascular circulation in type II diabetes model. Materials and method: Male diabetic db/db mice and age-matched control mice from C57BL/6J strain at 8, 12 and 16 weeks of age were subjected to echocardiography. Mice were anesthetized with 2% isoflurane vaporized with 100% oxygen. At the mid-papillary level in the parasternal left ventricular short-axis view, end diastolic and systolic left ventricular diameter, interventricular septal thickness and posterior wall thicknesses, ejection fraction, fractional shortening were determined by M-mode echocardiography. Isoflurane was reduced to 1% to measure baseline left anterior descending coronary artery flow under a modified parasternal left ventricle long-axis view, and then increased to 3% to measure maximal coronary flow. Coronary flow velocity reserve (CFVR) was calculated as the ratio of peak diastolic velocity (PDV) at the highest flow attained during the high level of isoflurane (3%) to PDV at the minimum baseline flow obtained at the lowest level of isoflurane (1%): CFVR = Hyperemic coronary flow velocity (PDVh)/Basal coronary flow velocity (PDVb). Results: There was no significant difference in interventricular septal thickness, posterior wall thicknesses, end diastolic and systolic left ventricular diameter, ejection fraction and fractional shortening between db/db and age-matched control mice at 8, 12 or 16 weeks of age (P > 0.05). At 8 weeks of age, there was no significant difference in PDVb, PDVh and CFVR of the left anterior descending artery. At 12 weeks of age, CFVR in db/db mice was lower than in control mice (P < 0.01). At 16 weeks of age, PDVh and CFVR was significantly decreased in dbdb mice (P < 0.01), although PDVb remained unchanged. A significant negative correlation was noted between CFVR and the blood glucose level in db/db and C57BL/6 mice (r =-0.612, P < 0.01). Conclusion: The present study confirmed that db/db mice showed the decreased CFVR from the age of 12 weeks, suggesting the impairment of coronary microvascular function. The presence of hyperglycemia may be a significant contributor to the development of impaired coronary vasodilator response.

Objective To explore the value of layer-specific strain in assessment of left ventricular function in patients with heterozygous familial hypercholesterolemia (HeFH) with normal left ventricular ejection fraction (LVEF).Methods Thirty-four patients with diagnosed HeFH and underwent transthoracic echocardiography were included as HeFH group,while 29 healthy volunteers were taken as control group.EchoPAC software was used to obtain endocardial longitudinal strain (LSendo),myocardial longitudinal strain (LSmyo) and epicardial longitudinal strain (LSepi) of the epicardial,and then statistical analysis was performed.Results LSendo and LSmyo in HeFH group were significantly lower than those in control group (P＜0.001).LSendo and LSmyo were negatively correlated with total cholesterol and low density lipoprotein cholesterol (all P＜0.05).Conclusion Layer-specific strain of left ventricular is of great value in assessing early myocardial damage in patients with HeFH.

Objective Familial hypercholesterolemia ( FH ) is the most common and serious monogenic disorder of lipid metabolism, causing premature coronary heart disease ( CHD ) due to accelerated atherosclerosis from birth, and the study of left ventricular ( LV ) function of this disease is seldom. The purpose of this study was to explore the value of layer‐specific strain on assessing the early damage of LV function in asymptomatic and left ventricular ejection fraction ( LVEF ) well‐preserved patients with heterozygous FH (He FH ). Methods A total of 49 patients aged 38.7±8.7 diagnosed with heterozygous familial hypercholesterolemia and who had undergone transthoracic echocardiography from 2010 to 2016 were included in this study. A total 32 healthy volunteers aged 35.6±10.3 were included as control group. Longitudinal and circumferential strains of the endocardium, myocardium, and epicardium ( LS endo, LS myo, and LS epi and CS endo, CS myo, and CS epi) were obtained by a software enabling the analysis of strains in three myocardial layers. Results In longitudinal strain ( LS ), the LS of endocardium ( LS endo) and the LS of myocardium ( LS myo) are significantly reduced in patients with He FH ( P <.001 in both). In circumferential strain ( CS ), only the CS of endocardium ( CS endo) is significantly reduced ( P <.001). The degree of reduction in strain is positively correlated with the TC and LDLC . Conclusions Layer‐specific evaluation of the left ventricle has great value in evaluating early impairment of LV in patients with FH . And this relatively novel technique may made it possible to help us understand the process of LV impairment in patients with FH better, thus preventing further damage.

AbstractAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and sudden death in adults. Transthoracic echocardiography (TTE) plays an important role in early detection and diagnosis of ALCAPA as a noninvasive modality. However, its diagnostic value is not well studied. The purpose of this study is to determine the performance of TTE in the diagnostic assessment of ALCAPA as compared with coronary CT and invasive coronary angiography.A total of 22 patients (13 women and 9 men, mean age, 12.9 ± 19.5 years) with ALCAPA who underwent echocardiographic examination for clinical diagnosis were retrospectively reviewed and analyzed. Transthoracic echocardiographic features of ALCAPA were analyzed and its diagnostic value was compared with invasive coronary angiography and coronary CT angiography (CTA) with surgical findings serving as the gold standard. Surgery was performed in all of the patients to establish the dual coronary artery system. Five underwent the Takeuchi procedure and 17 had re-implantation of the anomalous left coronary artery. Of 20 patients, echocardiographic diagnoses were in good agreement with findings at surgery, resulting in the diagnostic accuracy of 90.9%. Two cases were misdiagnosed—one as the right coronary artery to pulmonary artery fistula and the other as rheumatic heart disease. The echocardiographic features of these patients with ALCAPA included: abnormal left coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow in 20 patients; enlargement of the right coronary artery in 17 patients; abundant intercoronary septal collaterals in 17 patients; and moderate and significant mitral regurgitation in 14 patients. The diagnostic accuracy of invasive coronary angiography (in 17 patients) and coronary CTA (in 9 patients) was 100%.This study shows that TTE is an accurate, noninvasive imaging modality for displaying the origin of coronary arteries and demonstrating the coronary courses as well as other associated abnormalities in patients with ALCAPA.

目的:评价新型可回收自膨胀型肺动脉支架瓣膜经胸微创置换肺动脉瓣膜的可行性、安全性和有效性.方法:健康清洁级实验猪12只,随机分三组,A组:3只,仅行单纯小切口开胸手术;B组:3只,开胸经球囊损伤肺动脉瓣建立反流模型;C组:6只,开胸穿刺右心室前壁置入带瓣膜支架置换自身肺动脉瓣膜.术后行心血管造影(DSA)及超声检查评估瓣膜功能,最后放血处死,取心、肝、脾、肺、肾和脑等重要器官行肉眼、光镜及钙含量检查.结果:12只实验猪手术均获成功.心血管造影、超声证实:B组成功建立肺动脉瓣反流模型,;C组置入支架瓣膜位置满意,启闭良好、无反流,未见血栓及赘生物形成;与术前相比,B组右心室容积明显增大、射血分数显著降低;A组和C组血流动力学指标则无明显差异.肉眼:瓣膜位置良好,锚定牢靠;光镜:内皮细胞形态饱满、排列有序的覆盖支架瓣膜,未见钙化灶及周围组织炎症反应,肝、脾、肺、肾、脑等重要器官未见血栓形成.结论:可回收自膨胀型肺动脉支架瓣膜经胸微创置换肺动脉瓣膜方法可行,手术成功率高,无相关并发症,效果理想.

Aim: The purpose of the present study was to determine the characteristics of myocardial damage at different stages of diabetes mellitus (DM) using layer-specific myocardial strain. Material and methods: Thirty six New Zealand white rabbits were randomly divided into either the control group (n =18) or the DM group (induced with alloxan) (n=18). For the myocardial deformation studies echocardiography and layer-specific strain were performed at baseline and after 3, 6, and 9 months in all of the rabbits. Three-layer longitudinal strain (LS) was calculated in the apical 4-chamber view, and three-layer circumferential strain (CS) in the short-axis view at the level of mitral valve. Layer-specific longitudinal and circumferential strains were assessed from endocardium, mid-myocardium and epicardium. For histomorphological study of the heart structure, the rabbits were sacrificed at 3, 6 and 9 months. Routine hematoxylin and eosin staining was performed. Results: The highest absolute values of left ventricular longitudinal strain (LS) and circumferential strain (CS) were registered in the endocardium and the lowest in the epicardium in both groups. At 3 months, there was no significant difference in three-layer LS and CS (p>0.05), but at 6 months the LS of endocardium (LSendo) and CS of endocardium (CSendo) were lower in the DM group compared with the control group; at 9 months, the rest of the parameters were also decreased (p<0.05). Moreover, in ROC analysis at 6 months LSendo yielded better sensitivity and specificity in the detection of diabetic cardiomyopathy (AUC of LSendo was 0.897 and AUC of CSendo was 0.617). With the progression of untreated diabetes, the histopathological abnormalities intensified gradually beginning at 6 months. Conclusion: The progressive impairments in LV myocardial deformation and structure occurs early in diabetic rabbits, the myocardial damage may be nontransmural, and endocardial function is more susceptible to be affected by DM. Layer-specific myocardial strain echocardiography may identify subtle myocardial dysfunction in the early stages of DM.

Neural tube defects (NTDs) are common congenital malformations. Mitogen-activated protein kinases (MAPKs) pathway is involved in many physiological processes. HMGB1 has been showed closely associated with neurulation and NTDs induced by hyperthermia and could activate MAPKs pathway. Since hyperthermia caused increased activation of MAPKs in many systems, the present study aims to investigate whether HMGB1 contributes to hyperthermia induced NTDs through MAPKs pathway. The mRNA levels of MAPKs and HMGB1 between embryonic day 8.5 and 10 (E8.5-10) in hyperthermia induced defective neural tube were detected by real-time quantitative polymerase chain reaction (qPCR). By immunofluorescence and western blotting, the expressions of HMGB1 and phosphorylated MAPKs (ERK1/2, JNK and p38) in neural tubes after hyperthermia were studied. The mRNA levels of MAPKs and HMGB1, as well as the expressions of HMGB1 along with phosphorylated JNK, p38 and ERK, were downregulated in NTDs groups induced by hyperthermia compared with control. The findings suggested that HMGB1 may contribute to hyperthermia induced NTDs formation through decreased cell proliferation due to inhibited phosphorylated ERK1/2 MAPK.

目的 回顾性分析46例三尖瓣下移畸形的外科治疗结果及术前超声资料,以期为术前评估三尖瓣下移畸形不同手术方式的选择指征提供线索.方法 2010年3月-2013年9月收治三尖瓣下移畸形46例,其中男性28例,女性18例,年龄0.4～35岁,平均体重(26.2±18.6)kg.Carpentier分型A型4例,B型26例,C型15例,D型1例.行双心室矫治33例,一个半心室矫治10例,单纯行双向Glenn术1例,肺动脉环缩术2例.分析全组术前超声资料,对比双心室矫治及一个半心室矫治病例房化右心室与功能右心室参数及手术结果.结果 本组术后死亡1例(2.2％).全组功能右心室/房化右心室比值(FRV/ARV比值)为0.79±0.31,双心室矫治组FRV/ARV比值为0.99±0.35,一个半心室矫治组FRV/ARV比值为0.43±0.23,2组差异存在统计学意义(t=5.9520,P=O.0000).12例术后出现心包积液(26.09％),其中双心室矫治组出现5例,一个半心室矫治组7例.34例术后无心包积液患者FRV/ARV为1.07±0.92,术后心包积液患者FRV/ARV为0.49±0.41,2组差异存在统计学意义(t'=2.8897,P＜0.05).随访2～44个月无死亡,无严重并发症发生.结论 三尖瓣下移畸形的手术方式的选择应参考左右心室发育情况,功能右心室/房化右心室比值对抉择三尖瓣下移畸形手术方式可能有一定意义.

Objective To explore the effects of hyperthermia on the development of hamster embryonic heart .Methods Ham-sters were randomly divided into 2 groups.Experimental groups were subjected to hyperthermia (42℃in hot water for 20min) in day 8 post coitus.The embryos were obtained at 12,24,36,48h after treatment respectively,and serial sections of embryonic heart were prepared .The expressions of proteins p-JNK1/2 in the embryonic heart were observed with immunofluorescent staining technique .Results At 12,24,36 and 48 hours,expressions of p-JNK1/2 in the experimental groups were decreased ,and the 36 and 48hours were decreased significantly .Con-clusion The expressions of p-JNK1/2 decrease significantly in the embryonic heart of hamster after hyperthermia ,suggesting p-JNK1/2 may play an important role in the normal development of heart .

Neural stem cells (NSCs) are involved in neural tube formation. As the high-mobility group box 1 (HMGB1) protein is involved in neurulation and is present at elevated levels in neural tube defects (NTDs) induced by hyperthermia, we have now investigated the effects of HMGB1 on proliferation, differentiation, and MAPK signaling pathways of NSCs in vitro. We constructed a lentivirus vector with HMGB1 siRNA and used it to infect NSCs. Down-regulation of HMGB1 expression was confirmed. Proliferation of NSCs was determined by MTS and nestin/BrdU double-labeling. Differentiation of NSCs was assessed using β-tubulinIII and GFAP. Knockdown of HMGB1 significantly suppressed NSC proliferation but hardly affected differentiation, which was regulated by decreased expression of MAPK signaling pathways. Thus, HMGB1 has beneficial effects on neurulation and may serve as a new target for the prevention of NTDs.

目的 研究吡格列酮对ERK1/2与Bcl-2信号转导通路在大鼠心肌缺血再灌注损伤中的作用.方法 取半日龄到一日龄Wistar大鼠幼鼠心室肌细胞进行体外培养,并建立缺血再灌注模型.体外培养的细胞分为正常对照组(C组)、缺血再灌注组(Ⅰ组)、缺血再灌注组+吡格列酮处理组(P组)和P组+ERK1/2抑制剂PD98059处理组(P+Pd组).采用Western-bloting和免疫细胞化学染色法检测细胞内Bcl-2蛋白的含量.结果 Western-bloting与免疫细胞化学法显示,Ⅰ组Bcl-2蛋白表达水平高于C组(P＜0.05),P组的Bcl-2蛋白表达高于Ⅰ组,P+Pd组Bcl-2蛋白表达水平低于P组(P＜0.05).结论 吡格列酮对心肌细胞缺血再灌注损伤的保护可能与下调ERK1/2和Bcl-2转导通路有关.

Objective To investigate the application of modified ultrafiltration (MUF) and conventional ul-trafiltration(CUF) during and after cardiopulmonary bypass (CPB) for pediatric open heart surgery.Methods From January 2007 to December 2010 in our hospital,40 children with congenital heart diseases undergoing operations were classified randomly into modified ultrafiltration group (MUF,n =20) and conventional ultrafiltration group (CUF,n =20).The clinical data of all the infants,including hematocrit(Hct) before CPB,intraoperative hematocrit,hematorit after CPB,volume of thoracic drainage within 24 hours after operation,mechanical ventilation support time after operation ,vol-ume of postoperative blood transfusion were observed .Results No infant was dead and no MUF-related complications occurred in our research.The difference of Hct before CPB between the 2 groups was not significant(P <0.05).Accord-ing to some degree of the blood diluted,the level of Hct was lower than before after CPB in both groups ,but compared with Hct level,MUF group was significantly higher(P <0.05).The dosage of bank blood,postoperative chest drain loss within 24 hours and ventilator time were significantly lower in MUF group (P <0.05).Conclusion MUF can filter sur-plus water efficiently,decrease blood transfusion requirement and remove the tube earlier ,which is helpful in the conva-lescence of infants.

Objective:To explore the surgery treatment effect of Ebstein's anomaly in different pathological classification through analyzing 39 patients.Methods:39 patients with Ebstein's anomaly underwent operation from July,2010 to Febrary,2013 Male 23,female 16.The mean age was(7.7±8.5) years;The preoperative ECG shows 5 patients of 39 patients(12%) had WPW syndrome.The surgical strategies included:two-ventricle repair in 26 patients,one-and-a-half-ventricle repair in 10 patients,Glenn procedure in 1 patient,pulmonary artery banding in 2 patients.Results:Postoperative mortality was 2.5%(1/39).No serious complications were found.Conclusion:Surgical strategies should be chosen according to the condition of tricuspid valve and annulus size,and the ARV/FRV.The surgery skill is a useful way to treat Ebstein's anomaly.

<正>临床资料患者,女,59岁。心功能Ⅳ级。以风湿性心脏病、二尖瓣中度狭窄合并轻度关闭不全、三尖瓣轻度关闭不全收入院。拟在全身麻醉下行二尖瓣置换术。患者无药物、食物及其它致敏物质过敏史。术前左心室射血分数60%,血常规、出凝血时间、心电图、胸部X线片、肝肾功能未见明显异常。入手术室后行心电监测:血压96/52 mm Hg、心率130次/分、脉搏血氧饱和度(SpO2)97%。12点39分采用长托宁1 mg、咪达唑仑1 mg、依托咪酯15 mg、舒芬太尼80 mg、罗库溴铵

Objective To investigate the expression of Notch 4 in esophagus squamous cell carcinoma and its relationship with clinicopathological significance and prognosis.Methods A retrospective study was performed of 97 patients with midthoracic esophageal squamous carcinoma who accepted surgical treatment from 2007 to 2010.The expression of Notch 4 was examined by immunohistochemistry.The survival rate was calculated by Kaplan-meier method and the difference was determined by the log-rank analyze.The cox regression analysis was performed to determine the independent risk factors for lymph-metastastic recurrence.Results Notch 4 protein expression was detected in 42 patients of 97 with positive rate of 45.2％.The positive rate of Notch 4 in T1,T2,T3 and T4 patients was separately 66.7 ％ 、54.5 ％ and 28.6％ with significant difference(P =0.009).The positive rate of Notch 4 expression in NO and N1 patients was separately 57.1％ and 29.2％ with significant difference(P =0.005).the positive rate of Notch 4 expression in low-middle differentiation and high differentiation patients was separately 31.3 ％ and 56.3 ％ with significant difference (P =0.006).the 5-year survival rate of patients with Notch 4expression and without Notch 4 expression was separately 47.6％ and 18.2％ with significant difference(P =0.000).The Cox regression analysis showed that the negative expression of Notch 4 and the lymphometastasis were independent risk factors.Conclusion The low expression of Notch 4 protein was detected in esophagus squamous cell carcinoma and had a correlation with the differentiation,T classification and lmphometastasis.Cox regression analysis showed that the low expression of Notch 4protein is an independent risk factor and can be a biomarker of poor prognosis.

目的 探讨高温致神经管畸形(neural tube defects,NTD)的分子机制,为防治神经管畸形提供理论依据.方法 利用高温致金黄地鼠NTD动物模型,应用免疫荧光染色技术,观察NTD发生过程中磷酸化c-Jun氨基末端活化蛋白激酶(p-JNK)和磷酸化p38(p-p38)在胚胎神经管上皮的表达变化.结果 p-JNK和p-p38的免疫阳性产物表达于胚胎神经管上皮细胞及其周围间充质细胞的胞浆中,高温后不同时间点胚胎神经管上皮细胞内的p-JNK和p-p38的表达量均较对照组减弱.结论 磷酸化JNK和p38参与了神经管的正常发育,其表达量降低可能是高温致NTD发生的重要途径之一.