预测心内膜弹力纤维增生症疗效的生物标志物分析

目的:寻找预测激素联合丙种球蛋白对于心内膜弹力纤维增生症患儿疗效的生物标志物.方法:对2014至2018年,首都医科大学附属北京安贞医院,诊断为心内膜弹力纤维增生症的患儿58例资料进行回顾分析,每例患儿均给予基础对症治疗、激素免疫治疗及丙种球蛋白2g/kg冲击治疗.以患儿治疗后LVEF绝对值上升≥10％或治疗后LVEF...     

川崎病心血管症状的护理及不同剂量丙种球蛋白的疗效观察

目的探讨川崎病心血管症状的观察与护理及运用不同剂量丙种球蛋白对川崎病患儿进行治疗的效果。方法选择临床确诊为川崎病而在我院接受治疗的84例患儿作为研究对象,根据使用丙种球蛋白剂量的不同分为两组,A组患儿(42例)接受常规剂量治疗及护理干预;B组患儿(42例)接受大剂量治疗及护理干预,观察比较组间的临床治疗护理效果。结果 B组患儿的总有效率明显高于A组患儿,差异有统计学意义(P<0.05);B组患儿的症状体征消退时间明显短于A组患儿,差异有统计学意义(P<0.05);B组患儿的冠状动脉损害率和不良反应发生率较A组有所下降,但差异无统计学意义(P>0.05);B组患者的左心室短轴缩短率(LVFS)、左心室射血分数(LVEF)以及二尖瓣口血流流速E峰与A峰的比值(E/A)比值明显高于A组,差异有明显统计学意义(P<0.05)。结论使用大剂量丙种球蛋白治疗川崎病患儿,可进一步提高治疗护理效果,缩短治疗护理时间,具有较高的临床应用价值。     

卡维地洛治疗小儿原发性心内膜弹力纤维增生症临床效果评价

目的本文就经卡维地洛治疗小儿原发性心内膜弹力纤维增生症治疗中的临床价值进行了研究和探讨。方法选择我院自2013年1月至2014年9月期间收治的小儿原发性心内膜弹力纤维增生患儿70例,按照入院的先后顺序将其平均分为实验组和对照组,两组患儿均35例,对照组采用洋地黄进行治疗,实验组采用卡维地洛治疗,对两组患儿的心内膜弹力纤维治疗效果,不良反应发生率及生活质量评分进行观察和统计。结果实验组患儿治疗总有效率明显高于对照组患者(P0.05);实验组患儿不良反应发生率明显低于对照组患者(P0.05);实验组患儿生活质量评分明显高于对照组患者(P0.05)。结论卡维地洛应用于小儿原发性心内膜弹力纤维增生治疗中的效果显著,有效改善了患儿的病情和生活质量,值得在临床医学中推广。     

不同剂量丙种球蛋白对ITP患儿血小板参数的影响

将42例特发性血小板减少性紫癜（ITP）患儿随机分为A、B两组，A组应用静脉注射丙种球蛋白（IVIG）1g／kg、B组应用IVIG 2g／kg加地塞米松静滴。两组患儿治疗第3、7、10、14天血小板计数均较治疗前明显升高，但两组同一时间血小板计数、出盒消失时间及治疗有效率均无统计学差异。认为中剂量IVIG是治疗ITP的有效方法，可适当减少费用，降低血液制品风险。     

小儿左冠状动脉起源于肺动脉临床分析

目的:总结小儿左冠状动脉起源于肺动脉(ALCAPA)的临床特点,提高对ALCAPA的认识。方法:对2006年8月至2008年12月期间我院心脏中心门诊以"心内膜弹力纤维增生症"收治但最终诊断为ALCAPA的8例患者的临床特点、检查情况进行回顾性分析。结果:8例ALCAPA的患者女性7例,男性1例。发病年龄2～10个月,平均年龄5.3个月。8例患者均表现不同程度的气促、喘息、声哑、多汗及喂养困难等心力衰竭(心衰)症状。病初均于首诊医院误诊为心内膜弹力纤维增生症。患儿心电图、心脏超声及心脏增强CT均有较为特征性的改变。结论:小儿ALCAPA病例临床常易与心内膜弹力纤维增生症混淆。对于临床诊断心内膜弹力纤维增生症的患儿应注意其心电图表现。其中符合ALCAPA心电图特点的病例,反复探查其心脏彩超并进行心脏增强CT、心脏冠状动脉造影检查有助于ALCAPA的诊断。     

大剂量一次性静脉注射IVIG治疗新生儿ABO溶血病40例临床观察

目的探讨大剂量一次性静脉注射丙种球蛋白(IVIG)治疗新生儿ABO溶血病的可行性及安全性。方法采用双盲法将40例新生儿ABO溶血病患儿随机分为观察组和对照组,每组20例。对照组患儿采用常规IVIG剂量(0.4 g/kg)治疗,观察组患儿采用大剂量一次性IVIG(1 g/kg)治疗,观察比较两组患儿治疗前后血清总胆红素值、血红蛋白(Hb)、网织红细胞(Re)的变化以及患儿皮肤黄疸消退时间、蓝光照射累计时间、住院时间及转归情况。结果两组患儿治疗后24 h、48 h、72 h血清胆红素值均有所下降,观察组患儿下降程度明显优于对照组患儿(P0.05)。观察组患儿治疗前后Hb、Re无明显变化(P0.05),没有出现明显溶血;对照组患儿Hb、Re治疗后明显低于治疗前,治疗后仍有溶血存在。观察组患儿黄疸消退时间、蓝光累计照射时间以及住院天数均明显短于对照组患儿,差异均具有统计学意义(P0.05)。结论大剂量一次性静脉注射丙种球蛋白治疗新生儿ABO溶血病,可有效降低患儿血清总胆红素值,缩短患儿住院时间、光疗时间以及黄疸消退时间,临床疗效显著,值得推广应用。     

静脉注射丙种球蛋白辅治婴幼儿重症肺炎的临床疗效及免疫功能观察

目的 探讨静脉注射丙种球蛋白(IVIG)治疗婴幼儿重症肺炎的临床疗效和对免疫功能的影响.方法 选取我院63例重症肺炎患儿在常规抗感染及对症治疗的同时给予IVIG治疗.结果 治疗组患儿临床症状,肺部啰音消失,心功能的改善及X线胸片炎症吸收时间均明显缩短,血清IgG水平上升.结论 IVIG用于治疗婴幼儿重症肺炎可以缩短病程,提高治愈率,增强机体免疫功能.     

大剂量静脉注射免疫球蛋白辅助治疗儿童系统性红斑狼疮

目的　观察大剂量静脉注射免疫球蛋白 (IVIG)治疗儿童系统性红斑狼疮 (SLE)的疗效。方法　 6 9例SLE患儿随机分成两组 ,IVIG组 33例用大剂量IVIG和激素及环磷酰胺 (CTX) ;对照组 36例用激素和CTX治疗。观察大剂量IVIG对SLE疾病活动指数 (SLEDAI)、临床表现和SLE血清学指标的影响。结果　IVIG组治疗 3个月后SLEDAI明显低于对照组 (P <0 0 1) ,在降低蛋白尿、ANA及抗dsDNA抗体以及控制院内感染、病情稳定方面显著优于对照组。结论　大剂量IVIG是治疗儿童SLE有效的辅助措施。     

大剂量静注丙种球蛋白治疗系统性红斑狼疮33例

近年来,大剂量静脉注射丙种球蛋白(IVIG)已成为治疗许多免疫性疾病的有效手段之一.笔者采用IVIG治疗系统性红斑狼疮(SLE)33例,并与传统的糖皮质激素+环磷酰胺治疗比较,效果良好,报告如下.     

超声心动图诊断心内膜弹力纤维增生症

目的 :了解心内膜弹力纤维增生症 (EFE)患儿的心内结构、心功能改变以指导临床诊断。方法 :EFE 10例 ,年龄 2个月～ 2 .5岁 ,<1岁 8例 ,正常对照组 ,为年龄、性别、身长、体重一致的健康婴幼儿 10例。超声测量两组心脏房室内径、室壁及心内膜的厚度 ,瓣膜血流及心功能指数。结果 :EFE组射血分数、心室短轴缩短率、每搏指数及心排血指数均明显下降。 10例左室明显增大 ,搏动弱 ;80 %有心内膜回声增强、增厚 (3～ 6mm)、二尖瓣或 (和 )腱索增厚、挛缩。结论 :超声心动图检查为诊断EFE提供了一个实用的新方法 ;E/A对鉴别EFE与扩张型心肌病有重要参考价值     

Persistent left ventricular disease in clinically "cured" primary endocardial fibroelastosis.

We studied by serial cardiac catheterisation eight patients with the dilated form of primary endocardial fibroelastosis in whom congestive heart failure disappeared with treatment. All remained without symptoms for at least three years before recatheterization. Four patients showed regression of the abnormal electrocardiographic findings, three showed persistence, and one showed progression of electrocardiographic left ventricular overload pattern. On first cardiac catheterisation all patients had a dilated left ventricle with a mean ejection fraction of 0.36. In six of the patients repeat cardiac catheterisation showed left ventricular dilatation with a diminished ejection fraction (mean 0.32). Left ventricular end-diastole pressure was raised (12 to 28 mmHg, mean 19 mmHg). In this group were included the three patients with persistence and one with progression of the abnormal electrocardiographic findings, and two of the four patients with regression of these findings. The highest left ventricular end-diastolic pressure was found in a patient in whom the abnormal electrocardiographic findings almost reverted to normal. In the two remaining patients with reversion of the electrocardiographic abnormalities repeat cardiac catheterisation showed nothing abnormal. Our findings indicate that "cure" in primary endocardial fibroelastosis is incomplete. These findings may be the cause of sudden death or late clinical deterioration in some reported patients with "cured" primary endocardial fibroelastosis. The electrocardiogram is of little value in assessing these processes.     

右心室心尖部不同起搏比率与左心室大小可能有关联

目的:探讨传统右室心尖部起搏与右室流出道间隔起搏对心功能的影响,以及右室心尖部不同起搏比例与左室大小的关系。方法:66例缓慢性心律失常患者行起搏治疗:46例为右室心尖部起搏,20例为中下位室间隔起搏,随访12个月,心脏超声评价术前、半年及1年的左房及左室内径、左室射血分数;对心尖部起搏组按心室起搏比率分为≥30%、<30%两组,同样心脏超声评价左房及左室内径、左室射血分数。结果:右心室心尖部组及右心室室间隔组,在手术前、手术后6个月及12个月其左心房内径、左心室舒张末内径及左室射血分数均无显著差别。心尖部起搏比率≥30%组与<30%组间左心房内径、左室射血分数未见显著区别,而左心室在半年及1年可见左心室舒张末径变化有显著差异,随着起搏比率增加,左心室舒张末内径增大。结论:右室起搏1年,起搏部位对左心结构及功能影响无统计学意义;右室心尖部高心室起搏比率(≥30%)可引起左心室舒张末内径的增大。     

Intravenous immune globulin in the therapy of peripartum cardiomyopathy.

OBJECTIVES: We sought to evaluate the effect of therapy with intravenous immune globulin on recovery of left ventricular function in women presenting with peripartum cardiomyopathy. BACKGROUND: Peripartum cardiomyopathy is a rare complication of pregnancy that results in significant morbidity and mortality in women of childbearing age. Intravenous immune globulin has been reported to improve left ventricular systolic function in patients with acute dilated cardiomyopathy and myocarditis, but its effectiveness in peripartum cardiomyopathy is unknown. METHODS: In this retrospective study, we compared the clinical outcomes of six women with peripartum cardiomyopathy treated with intravenous immune globulin (2 g/kg) with those of 11 recent historical control subjects. All women in the study were referred between 1991 and 1998 with class II to IV heart failure and a left ventricular ejection fraction of <0.40. Left ventricular ejection was reassessed during early follow-up (6.1+/-2.9 months). RESULTS: The two groups did not differ in terms of baseline left ventricular ejection fraction, left ventricular end-diastolic diameter, months to presentation, age or multiparity. The improvement in left ventricular ejection fraction in patients treated with immune globulin was significantly greater than in the conventionally treated group (increase of 26+/-8 ejection fraction units vs. 13+/-13, p = 0.042). CONCLUSIONS: In this small retrospective study of women with peripartum cardiomyopathy, patients treated with immune globulin had a greater improvement in ejection fraction during early follow-up than patients treated conventionally. Given the poor prognosis of women with peripartum cardiomyopathy who do not improve, this therapy merits further study.     

Increase in radionuclide left ventricular ejection fraction after cardioversion of chronic atrial fibrillation in idiopathic dilated cardiomyopathy.

To assess the potential improvement in left ventricular ejection fraction after cardioversion of chronic atrial fibrillation to sinus rhythm in idiopathic dilated cardiomyopathy, we studied prospectively 17 patients, aged 58 +/- 6 years, by radionuclide angiocardiography at rest. Left ventricular ejection fraction was determined before treatment and at a mean delay of 4.7 months after cardioversion. Return to sinus rhythm was obtained in 12 patients, pharmacologically or by electrical cardioversion. Five patients remained in atrial fibrillation. No clinical, echocardiographic or haemodynamic finding could predict the success of cardioversion. In chronic atrial fibrillation, the ejection fraction did not change significantly: 30.0 +/- 9.1% (19 to 44%) at the first evaluation and 29.5 +/- 8.3% (22 to 41%) after 4.7 months. After successful cardioversion, left ventricular ejection fraction improved from 32.1 +/- 5.3% (24 to 41%) to 52.9 +/- 9.7% (37 to 71%) (P less than 0.001). The difference was 20.8 +/- 11.3% and left ventricular ejection fraction was normalized in 50% (6/12) of the patients. There was a significant reduction in the cardiothoracic ratio on chest X-rays and of the left ventricular end-diastolic diameter on echocardiography; fractional shortening increased (27.7 +/- 4.3% vs 20.3 +/- 2.7%, P less than 0.01). A third evaluation was realized after a mean delay of 11.7 months in the patients with successful cardioversion. Sinus rhythm was present in 83% (10/12) of the patients: seven patients were reevaluated by radionuclide angiography. The improvement in left ventricular function observed at the 4.7 months evaluation was still present. In two patients with recurrence of atrial fibrillation, there was a severe deterioration of left ventricular systolic function.(ABSTRACT TRUNCATED AT 250 WORDS)     

Persistent left ventricular disease in clinically "cured" primary endocardial fibroelastosis

We studied by serial cardiac catheterisation eight patients with the dilated form of primary endocardial fibroelastosis in whom congestive heart failure disappeared with treatment. All remained without symptoms for at least three years before recatheterization. Four patients showed regression of the abnormal electrocardiographic findings, three showed persistence, and one showed progression of electrocardiographic left ventricular overload pattern. On first cardiac catheterisation all patients had a dilated left ventricle with a mean ejection fraction of 0.36. In six of the patients repeat cardiac catheterisation showed left ventricular dilatation with a diminished ejection fraction (mean 0.32). Left ventricular end-diastole pressure was raised (12 to 28 mmHg, mean 19 mmHg). In this group were included the three patients with persistence and one with progression of the abnormal electrocardiographic findings, and two of the four patients with regression of these findings. The highest left ventricular end-diastolic pressure was found in a patient in whom the abnormal electrocardiographic findings almost reverted to normal. In the two remaining patients with reversion of the electrocardiographic abnormalities repeat cardiac catheterisation showed nothing abnormal. Our findings indicate that "cure" in primary endocardial fibroelastosis is incomplete. These findings may be the cause of sudden death or late clinical deterioration in some reported patients with "cured" primary endocardial fibroelastosis. The electrocardiogram is of little value in assessing these processes.     

Plasma BNP levels are determined by the severity of left ventricular systolic dysfunction but not the types of underlying heart disease

OBJECTIVES: The primary aim of the study was to investigate whether there is a difference in plasma B-type natriuretic peptide (BNP) levels among the left ventricular systolic dysfunction caused by different types of heart disease. METHODS AND RESULTS: Plasma BNP was measured in patients with left ventricular systolic dysfunction as a result of mitral valve regurgitation (n=26), hypertension (n=36), coronary heart disease (n=37) and dilated cardiomyopathy (n=32). The left ventricular end-diastolic diameter and ejection fraction were assessed with echocardiography. The valvular heart disease group had more women and was younger (p < 0.05). There was no significant difference in the New York Heart Association functional class, left ventricular end-diastolic diameter and ejection fraction among the four groups (p < 0.05).The average plasma BNP was also similar among the four groups of patients. In each group, a significant correlation between the levels of BNP and the left ventricular end-diastolic diameter or ejection fraction was identified (p < 0.001). CONCLUSION: Plasma BNP concentrations during left ventricular systolic dysfunction are associated with left ventricular diameter and function, but they are not determined by the causes of the heart failure.     

不同剂量阿托伐他汀联合二丁酰环磷酰苷钙对冠心病合并慢性心衰患者心功能及NT-proBNP的影响

【摘要】目的：探讨不同剂量阿托伐他汀联合二丁酰环磷酰苷钙对冠心病合并慢性心衰患者心功能及NT-proBNP的影响。方法：选择2015年6月到2016年12月之间在我医院心内科进行住院治疗的,冠心病合并慢性心力衰竭患者共116例,按照患者所采用的治疗方法进行分组,分别为对照组,低剂量组,中剂量组和高剂量,每个组29名患者,对照组患者在入院后施行常规治疗,观察组患者在对照组治疗方法的基础上,给予阿托伐他汀联合二丁酰环磷酰苷钙,不同剂量组使用的阿托伐他汀剂量不同,对治疗的效果进行对比。结果：四个组别患者在治疗前的NT-proBNP,C反应蛋白,左心室射血分数和左心室舒张末期内径数据差异不具有统计学意义（P>0.05）,经过治疗后四个组别的患者在四个项目上的数据与治疗前的数据差异均具有统计学意义（P<0.05）,从结果上看NT-proBNP,C反应蛋白和左心室舒张末期内径三个项目上,均为治疗后的数据低于治疗前的,左心室射血分数为治疗后高于治疗前的,在治疗后的数据中,NT-proBNP,C反应蛋白,左心室射血分数和左心室舒张末期内径四个项目中,不同组别的数据差异均具有统计学意义（P<0.05）,从结果上看,四个项目均只有高剂量组和其他三组之间的数据差异具有统计学意义,其余组间数据差异不具有统计学意义,四个组别患者在不同级别的治疗效果上的数据差异经过Kruskal-Wallis检验显示具有统计学意义（χ2=35.119,P<0.05）,四个组别的治疗有效率差异也具有统计学意义（χ2=8.627,P<0.05）,bonferonni检验两两比较的χ2检验结果显示,高剂量组与其他三组数据差异具有统计学意义（P<0.05）,对照组,低剂量组和中剂量组时间的数据差异不具有统计学意义（P>0.05）。结论：使用高剂量阿托伐他汀联合二丁酰环磷酰苷钙对冠心病合并慢性心衰患者进行治疗,可以改善心功能,改善NT-proBNP和C反应蛋白的指标,提升治疗的效果,值得在更广大临床范围内进行推广。     

心肌梗死合并室间隔穿孔的临床特征及近期预后分析

目的 初步探讨急性心肌梗死(AMI)并发室间隔穿孔(VSR)的临床特点、高危因素及近期预后情况.方法选择我院2001年1月至2012年6月期间AMI后VSR的患者73例,分析其临床特征、治疗方案及近期(1月内)死亡率.结果 73例AMI后VSR患者,前壁心肌梗死明显多于下壁、后壁,穿孔部位亦以室间隔心尖部常见.在梗死后1个月内有34例(46.6%)患者存活,39例(53.4%)死亡.与近期存活组相比,近期死亡组的女性比例、VSR直径、左室舒张末期内径、左室射血分数、是否置入IABP差异有统计学意义(P＜0.05).Logistic回归分析显示,左室射血分数低及未使用IABP辅助与近期死亡相关.结论 AMI合并VSR近期死亡率高.左室射血分数低、VSR直径大、左室舒张末期内径大、女性、未置入IABP辅助为近期死亡的高危因素,左室射血分数低及未使用IABP辅助为独立危险因素.对于AMI后VSR有近期死亡高危因素的患者,在积极药物治疗及使用循环辅助装置基础上尽早外科手术治疗可能改善近期预后.     

Echocardiographic evaluation of left ventricular function in Duchenne's muscular dystrophy

Thirty-six patients with Duchenne's muscular dystrophy were studied by echocardiography. The ejection fraction and percent shortening of left ventricular diameter (% ΔLVD) were significantly lower than that of an age-matched control group. The ejection fraction was less than 55 in 21 percent of the patients. There was no left ventricular enlargement, left ventricular hypertrophy or left atrial enlargement. Maximal diastolic endocardial velocity (MDEV) (105 ± 18 mm/sec) was lower than that in the control group (149 ±23 mm/sec); it was decreased in 90 percent of the patients and its severity showed poor correlation (r = 0.13) with abnormality in the ejection fraction. There was no correlation between abnormal MDEV and increased anterior forces on the electrocardiogram. Maximal systolic endocardial velocity (MSEV) was not significantly decreased. Electrocardiographic abnormalities were present in 92 percent of the patients. Patients were classified according to skeletal muscle functional capacity with group I patients having mild to moderate impairment and group II patients having severe disease with no significant difference between heart rate, ejection fraction, % ΔLVD, MDEV or MSEV of the two groups. Patients were subdivided into those less than 12 years old (group A); and those over 12 (group B). There was more severe skeletal muscle disability but no increased abnormality in parameters of left ventricular function in the older patients.Decreased MDEV is common in Duchenne's muscular dystrophy but is a poor predictor of left ventricular dysfunction which occurs in the minority of patients and does not correlate with the severity of skeletal muscle weakness. Skeletal muscle disease progresses with age with no significant progression in left ventricular dysfunction.