充血性尽力衰竭和左室舒张功能的关系

利用二维彩色多普勒超声心动图（２Ｄ－ＣＦＭ）测定６４例充血性心力衰竭患儿左室舒张功能和收缩功能，结果舒张功能减退而收缩功能正常者为４２．２％，单纯收缩功能不全为２５．０％，两者同时受得占３２．８％。进一步研究提示舒张功能性心力衰竭多发生于心脏病、心肌炎和风湿性心脏病合并二尖瓣闭不全或主动脉瓣病变，多表现为“后向性衰竭”，对洋地黄、利尿剂治疗不敏感，易出现洋地黄中毒，而开搏通和心痛定有一定疗效。     

超声心动图评价新生儿窒息的左心功能

应用超声心动图（ＵＣＧ）对３５例窒息新生儿进行左心功能测定。显示：窒息儿心内结构测定结果明显高于正常新生儿；窒息儿左室功能测定结果均明显低于正常新生儿。将小于正常新生儿测值的下限数作为判定左室收缩功能降低的标准，则窒息新生儿左室收缩功能降低的检出率为ＬＶＳＶ７８．５７％，ＬＶＥＦ１００％，ＳＶＩ７１．４３％，△Ｄ％１００％。结果表明应用ＵＣＧ评价新生儿窒息的左心功能是敏感而实用的监测手段之一。     

哮喘儿童血浆降钙素基因相关肽的测定及其与心功能的关系

采用放免分析法对３４例哮喘儿童急性发作期、２０例缓解期及３４例正常儿童血浆降钙素基因相关肽（ＣＧＲＰ）进行测定，并联合应用Ｍ型及二维超声心动图测定哮喘儿童的左、右心收缩及舒张功能。结果：哮喘发作期血浆ＣＧＲＰ含量较缓解期及正常儿童显著降低，缓解期血浆ＣＧＲＰ含量亦较正常儿童显著降低，急性发作期左、右心收缩功能指标异常，而舒张功能指标均正常；血浆ＣＧＲＰ与发作期左、右心收缩功能呈低、中度相关。提示：血浆ＣＧＲＰ参与了哮喘的发病过程，是哮喘病理生理过程中的重要调节肽；哮喘发作期左、右心收缩功能异常；血浆ＣＧＲＰ与心功能改变有一定的关系。     

超声心动图评价新生儿窒息的左心功能

应用超声心动图（ＵＣＧ）对３５例窒息新生儿进行左心功能测定，显示：窒息儿心内结构测定结果明同于正常对新生儿；窒息儿左室功能测定结果明显低于正常新生儿，将小于正常新生儿测的下限数作为判定左室收缩功能降低的标准，则窒息新生儿左室收缩功能降低的检出率为ＬＶＳＶ７８．５７％，ＬＶＥＦ１００％ＳＶＩ７１．４３％△Ｄ％１００％，结果表明应用ＵＣＧ评价新生儿窒息的左心功能是敏感而实用的监测手段之一。     

病毒性心肌炎的心功能改变及相关因素分析

目的了解病毒性心肌炎(VM)的心功能改变及相关因素.方法对152例病毒学确诊的心肌炎患儿测定其心功能,并分析心功能改变与抗心肌线粒体和心肌酶之间的关系.结果超声心动图检查,VM患儿每搏射血指数显著低于健康儿童;左室侧壁射血分数、室间隔射血分数和左室射血率显著低于健康儿童;二尖瓣环收缩运动和舒张早期运动速度显著低于健康儿童.心功能下降与抗心肌线粒体抗体阳性与否无关,肌酸激酶同功酶(CK-MB)增高者心功能降低者多于CK-MB正常者.结论部分VM患儿心功能降低,心功能降低与CK-MB增高与否有关,与抗心肌线粒体抗体阳性与否无关.     

组织多普勒成像对早期新生儿心室功能的评价

目的 应用组织多普勒成像（TDI）检测新生儿房室环运动，探讨不同胎龄早期新生儿左右心室功能的特点。方法 采用TDI技术检测生后3—7d的36例胎龄32～36周早产儿（早产儿组）和33例足月新生儿（足月儿组）二、三尖瓣环运动，测量收缩期峰值速度（Sa）、舒张早期峰值速度（Ea）及舒张晚期峰值速度（Aa），以脉冲多普勒显像测量舒张期二、三尖瓣口血流E峰及A峰峰值速度。结果 早产儿组各部位Ea、Sa均显著低于足月儿组，早产儿组三尖瓣环Ea／Aa显著低于足月儿组，两组Aa及E／Ea无显著差异；各部位Ea、Sa与胎龄及出生体质量均呈正相关。结论 早产儿左、右室收缩及舒张功能均低于足月儿，早期新生儿心室功能与其出生前成熟程度有关，早产儿及足月新生儿生后早期左室舒张功能均明显增强。     

小儿无创性心功能测定的研究进展

近年来心功能测定的研究发展迅速，目前可用于心功能测定的方法有：（１）血液内分泌变化；（２）心室腔大小的变化；（３）心搏血量的变化；（４）心脏收缩间期和舒张间期的变化；（５）心瓣膜流速的变化；（６）心血管解剖的变化等。上述方法随着科技的发展将会更加广泛应用于临床。     

Tei 指数的临床应用和进展

心力衰竭是目前严重危害小儿生命健康的一大常见疾病,临床漏诊率及病死率较高。对于小儿心功能的准确、有效的评估对疾病的诊治、监测及预后判断具有重要的临床意义。Tei 指数是由日本学者提出的一种可以综合评价心室收缩及舒张功能的指标,定义为心室等容收缩间期与等容舒张间期之和与心室射血时间的比值。该指标不受心率及心室的几何构型等外界因素影响,具有测量方法简便、重复性强、无创等优点,其对于心室功能受损判定的敏感性高。在临床评价心功能,尤其是对小儿心功能的评估中,得到了越来越多的应用。该文就 Tei 指数的测量原理及测量方法、相关影响因素及儿科中的临床应用和进展等作一综述。     

小儿原发性扩张型心肌病左心室收缩、舒张功能研究

小儿原发性扩张型心肌病左心室收缩、舒张功能研究安徽医科大学第一附属医院（合肥，２３００２２）范晓晨杨正修都鹏飞林文龙１）杨世宽我们利用二维彩色多普勒超声心动图（２Ｄ－ＣＦＭ）对２３例原发性扩张型心肌病患儿的左心室收缩、舒张功能进行了检测，现将结果总结...     

垂体性生长激素缺乏症104例的睾丸发育

目的了解男性垂体性生长激素缺乏症的睾丸发育及生长激素对睾丸的影响。方法检测104例生长激素缺乏症者的睾丸发育情况及部分患者的垂体促性腺激素功能，30例用生长激素治疗，最长疗程＜1a。结果睾丸明显偏小，部分伴促性腺激素储备或分泌不足，GH治疗前后睾丸体积无变化。结论男性生长激素缺乏症大多数伴性腺发育不良，短期GH治疗对患者的性腺发育无改善。     

Cardiac mass and function, carotid artery intima media thickness, homocysteine and lipoprotein levels in children and adolescents with growth hormone deficiency

INTRODUCTION: Early atheromatic changes have been found in the carotid and the femoral arteries of young adults with growth hormone (GH) deficiency (GHD). It has been shown that adults with GHD have increased cardiovascular risk factors. The aim of this study was to compare atherosclerotic risk factors in children with GHD and a normal population. PATIENTS AND METHODS: Twenty-five patients (10 girls, 15 boys) with GHD qualified for GH replacement treatment were included in the study. The control group consisted of 22 healthy children (11 girls, 11 boys) with no family history of atherosclerosis and cardiovascular disease. Cardiac mass and function was evaluated by one-dimensional, two-dimensional echocardiography and the Doppler method. Common carotid intima-media thickness was measured with a linear probe (7.5 MHz). Lipid metabolism parameters (total cholesterol, HDL- and LDL-cholesterol, triglycerides), lipoprotein A (Lp(a)) and homocysteine levels were measured. RESULTS: Cardiac dimensions and systolic parameters were within normal values in both groups. The mean index of left ventricular mass (68.37 +/- 18.64 g/m2) in patients with GHD did not differ significantly compared to controls (68.48 +/- 15.56 g/m2). No significant differences between the study and control group were observed when comparing systolic and ejection fraction values. Significantly higher mean values of IVRT parameter were found (70.8 +/- 14.2 ms vs 64.09 +/- 8.54% ms; p < 0.05). Other parameters characterizing systolic function were not significantly different. The mean concentration of total (178.28 +/- 31.1 mg/dl) and LDL-cholesterol (106 +/- 28.68 mg/dl) was significantly higher in patients with GHD than in controls (157.59 +/- 22.39 mg/dl, 84.54 +/- 22.01 mg/dl; p <0.05). Lp(a) (40.34 +/- 12.45 mg/dl vs 11.02 +/- 4.82 mg/dl; p <0.05) and apolipoprotein B (ApoB) (71.12 +/- 18 mg/dl vs 56.72 +/- 11.46 mg/dl; p < 0.05) were significantly higher in patients with GHD. Carotid artery intima-media thickness (IMT) values were significantly higher in patients with GHD compared to healthy children (IMT-L 0.53 +/- 0.058 mm vs 0.41 +/- 0.045 mm; IMT-R 0.54 +/- 0.048 vs 0.42 +/- 0.42 mm, respectively). CONCLUSIONS: No significant differences in parameters of cardiac systolic function and left ventricular mass were found between patients with GHD and healthy children. However, significantly higher Isovolumetric relaxation time (IVRT) values in the group of patients may suggest impaired diastolic function. Significantly elevated IMT values in the common carotid artery were observed in patients with GHD in comparison with healthy children, which may prove the onset of atheromatosis.     

The right ventricle in Fabry disease

Aim: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. Methods: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. Results: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement <10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling patterns). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92±11.7 g/m^2.7).
Conclusion: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

The Effect of Recombinant Human Growth Hormone Therapy on Left-Ventricular Chamber Size and Function in Children With Growth Hormone Deficiency

This study was designed to assess the effect of recombinant human growth hormone (rhGH) therapy on left-ventricular (LV) chamber size and function in children with idiopathic isolated growth hormone deficiency (GHD) using conventional echocardiography and tissue Doppler imaging (TDI). Thirty patients (19 boys and 11 girls) with idiopathic isolated GHD were followed-up for 12 months. Mean age of patients was 11.0 ± 2.6 years (range 6.3–15.5). At baseline and at 3, 6, and 12 months of treatment, the structure of the left ventricle was assessed by conventional echocardiography and myocardial rates and time intervals by TDI. There was a significant increase in LV mass (LVM) compared with pretreatment values. Like LVM, relative wall thickness (RWT) was also increased significantly. The significant increase in LVM indexed to body surface area and RWT became apparent at month 3 of treatment with a significant increase in LVM indexed to height^2.7 at treatment month 6. Normalized LVM increased as early month 3 of treatment, and a steady increase was observed until month 12. However, no patient had LVM > +2 standard deviation scores at month 12 of treatment. No significant differences were observed in functional parameters of the left ventricle and the interventricular septum. The results of this study showed that rhGH therapy causes an increase in myocardial mass without changing the geometry or function of the myocardium. Therefore, the increase in myocardial mass appears to be concentric, thus causing remodeling instead of hypertrophy.     

The educational, vocational, and marital status of growth hormone-deficient adults treated with growth hormone during childhood

The goal of growth hormone therapy in childhood is to increase stature, thereby facilitating normal psychosocial development. To determine the social outcome of patients with growth hormone deficiency (GHD), we interviewed 116 adults with GHD across Canada, including 86 men and 30 women 18 to 38 years of age who were treated with growth hormone during childhood. The education of the 96 patients who had completed their formal education was similar to their siblings and to the general population. Of the patients in the labor force, 35.4% were unemployed; the unemployment rates for those patients less than 25 years of age and those 25 years of age or older were 45% and 23%, respectively, compared with national rates of 21.2% and 9.4% for the same age groups, respectively. Of the 90 patients with GHD who were not attending school, 70 lived with their parents or relatives. Only 15 patients were married; one was divorced. The percentage of patients with GHD who were married was less than 30% of the expected age-adjusted rate. No difference in the rate of employment or marriage was found between the patients with idiopathic isolated GHD and organic hypopituitarism. In summary, the achievements of patients with GHD seem to be normal in the education system, but the rate of employment and marriage are much lower than expected. This poor outcome was unrelated to the response to growth hormone therapy and emphasizes the need to develop strategies that lead to more satisfactory psychosocial integration of patients with GHD in adult life.     

高海拔地区重症肺炎婴幼儿心脏功能检测的意义

目的探讨高海拔地区重症肺炎婴幼儿左右心室收缩及舒张功能变化的特点。方法应用彩色多普勒超声诊断仪测量(海拔2260 m)2000年1月~2005年1月本院住院30例(男16例,女14例)西宁地区婴幼儿重症肺炎和32例(男17例,女15例)健康婴幼儿左右心室收缩及舒张功能参数,并进行比较分析。结果病例组右室射血前期/射血加速时间(RPEP/AT)明显延长,右心室射血分数(RVEF)、肺动脉最大血流速度(Vpa)降低,右心室(RV)、右心房(RA)扩大。二、三尖瓣A峰、A/E增高,左室等容舒张时间(LIRT)、右室等容舒张时间(RIRT)明显延长(Pa<0.01)。结论高海拔地区婴幼儿重症肺炎存在肺动脉高压,及明显右心室收缩功能及左右心室舒张功能异常,左心室收缩功能无明显异常。     

The Munich Triathlon Heart Study: Ventricular Function, Myocardial Velocities, and Two-Dimensional Strain in Healthy Children Before and After Endurance Stress

Intense exercise has been shown to have negative effects on systolic and diastolic ventricular function in adults. Very little is known about the normal reaction of the growing heart to endurance stress. For this study, 26 healthy children (18 males) with a mean age of 12.61 years (range, 7.92–16.42 years) took part in an age-adapted triathlon circuit. The athletes were investigated by two-dimensional (2D) echocardiographic/speckle tracking, M-mode, pulse-wave Doppler, color Doppler, and color-coded tissue Doppler at 2–4 weeks before and immediately after the race. After the competition, cardiac output increased, mediated by an increase in heart rate and not by an elevated preload, according the Frank–Starling mechanism. Two-dimensional speckle tracking showed a reduced longitudinal strain in the right and left ventricles and additionally reduced circumferential strain in the left ventricle. The late diastolic inflow velocities were increased in both ventricles, indicating reduced diastolic function due to an impairment of myocardial relaxation. Immediately after endurance exercise, systolic and diastolic functions were attenuated in children and adolescents. In contrast to adult studies, this study could show a heart rate-mediated increase in cardiac output. The sequelae of these alterations are unclear, and the growing heart especially may be more susceptible to myocardial damage caused by intense endurance stress.     

The right ventricle in Fabry disease

AIM: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. METHODS: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. RESULTS: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement < 10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling pattems). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92 +/- 11.7 g/m(2.7)). CONCLUSION: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

The catecholamine response to hypoglycemia in children with isolated growth hormone deficiency syndromes and multiple pituitary hormone defects

We examined the catecholamine response to insulin-induced hypoglycemia in 46 short children evaluated for growth hormone (GH) deficiency by both pharmacologic stimulation and integrated concentration of GH. Twelve patients had quantitatively normal GH secretion by both pharmacologic stimulation and integrated concentration of GH (GHNORM). Twenty-two patients had normal GH to pharmacologic stimulation but subnormal integrated concentration of GH (GHND). Twelve patients had GH deficiency by both tests (GHD): six had isolated GH deficiency (GHD type 1) and six had multiple hormone deficiencies (GHD type 2). There was no significant difference between the peak epinephrine, norepinephrine, and cortisol responses of GH-NORM, GHND, and GHD type 1 patients. The mean peak epinephrine response of GHD type 2 patients was significantly lower (564 +/- 561 pg/ml, p less than 0.03) compared to the other patient groups. There was no significant difference between the peak norepinephrine levels between GHD type 2 patients and the remaining groups. There was no correlation between decrease in blood glucose and either increase in growth hormone, catecholamine, or cortisol concentrations. There was a significant correlation between log peak epinephrine and peak cortisol response (r = 0.53, p less than 0.0002) of the 46 subjects. Neither the basal nor stimulated catecholamine levels correlated with the integrated concentration of cortisol. We conclude that isolated GH deficiency is not associated with impairment of the catecholamine response to hypoglycemia; impairment of the epinephrine response to hypoglycemia is only associated with multiple pituitary hormone deficiencies; in children, the degree of glucose lowering is not correlated with the magnitude of peak GH, catecholamine, or cortisol responses.     

Hypothalamic dysfunction after chemotherapy

Cranial irradiation with or without chemotherapy can cause hypothalamic-pituitary dysfunction. Chemotherapy without cranial irradiation has not been thought to cause such deficiency. In order to determine whether chemotherapy without cranial irradiation can lead to hormonal deficiency, we reviewed the medical records of 362 childhood cancer patients who underwent full hypothalamic-pituitary evaluation because of altered growth and development after oncological therapy (1987-2002). Of these, 31 received chemotherapy but no cranial or total body irradiation and had no CNS tumor: 18 had hematological malignancy and 13 had a solid tumor of the torso or extremity. Duration of follow-up was 13.0 +/- 4.1 years (mean +/- SD). Growth hormone deficiency (GHD) was identified in 15 (48%), central hypothyroidism (TSH-D) in 16 (52%), and pubertal abnormalities in 10 (32%). Pubertal abnormalities included precocious puberty in two (6%), gonadal failure in five of 27 who were old enough to assess puberty (19%), and gonadotropin deficiency in three of 27 (11%). GHD and TSH-D were co-existent in eight patients (26%). Overall, 81% (n = 25) had GHD, TSH-D, precocious puberty, and/or gonadotropin deficiency. None had ACTH or ADH deficiency or primary hypothyroidism. Of note, this was not a study of prevalence, but rather an evaluation of clinically referred patients. In conclusion, hypothalamic dysfunction may occur in survivors of non-CNS tumors who receive chemotherapy but do not receive cranial irradiation. We recommend at least annual observation of growth rate and pubertal development of all children treated for pediatric malignancies, with evaluation for GHD, TSH-D, pubertal abnormalities, and other hypothalamic dysfunction in all poorly-growing cancer survivors, even those not treated with cranial irradiation.     

Acid-base homeostasis in children with growth hormone deficiency

BACKGROUND: Although the primary use of growth hormone (GH) is to promote linear growth, it is also known to affect many metabolic processes and to influence renal function. In laboratory animals, growth hormone deficiency (GHD) causes a mild metabolic acidosis that is corrected by GH treatment. We observed a patient with GHD who initially presented with acidosis of unclear etiology and corrected the acidosis with GH treatment. OBJECTIVES: To determine: 1) whether children with GHD have lower mean serum bicarbonate concentrations than do children with short stature because of other causes; and 2) whether the presence of a low serum bicarbonate concentration increases the probability of GHD among children with short stature. METHODS: We collected data from the medical records of 143 children with short stature who had serum electrolyte concentrations measured as part of their initial evaluations, 66 with GHD and 77 with short stature as a result of other causes. We compared mean serum bicarbonate concentrations and bicarbonate standard deviation scores (SDS) between these two groups and determined the probability of GHD for patients according to bicarbonate SDS. RESULTS: The mean serum bicarbonate concentration was significantly lower in patients with GHD (mean standard deviation [SD]; 23.9 [0.4] mEq/L vs 25.2 [0.3] mEq/L) as was the bicarbonate SDS (-0.12 [0.14] SD vs 0.38 [0.10] SD). Twelve (75%) of 16 patients with bicarbonate SDS 1 SD. Patients with bicarbonate SDS between -1 SD and 1 SD had an intermediate probability of GHD, 46/102 (45%), similar to the overall prevalence of GHD in the study population (46%). Mean bicarbonate concentrations and bicarbonate SDS increased significantly in 9 patients who had repeat electrolyte measurements during treatment with GH (mean bicarbonate; 21.7 [1.1] mEq/L vs 26.9 [0.59] mEq/L, mean bicarbonate SDS; -1.24 [0.43] SD vs 0.55 [0.27] SD). CONCLUSIONS: Serum bicarbonate concentrations are lower in patients with GHD than in patients with short stature as a result of other causes. In addition, serum bicarbonate concentrations rise with GH treatment in patients with GHD. The probability of GHD is increased for patients with bicarbonate SDS 1 SD. These findings indicate a role for GH in maintaining normal acid-base homeostasis and suggest that GHD should be considered in children whose growth failure is attributed to other causes of acidosis.