扩张型心肌病患儿心率变异改变及与心脏结构和功能的相关性

目的 研究扩张型心肌病(DCM)患儿的心率变异(HRV)改变及其与心脏结构和功能的相关性.方法 对36例DCM患儿和30例健康儿童分别进行24 h心率变异分析,得出连续平均正常RR间期标准差(SDNN)、每5 min正常R-R间期平均值的标准差(SDANN)、每5 min正常RR间期标准差的平均值(SDNN index)、相邻RR间期≥50ms的百分数(PNN50)、相邻RR间期差的均方根(rMSSD)、总功率(TP)、低频成份(LF)、高频成分(HF)及LF/HF等指标.行常规超声心动图检查,取得左房舒张末期前后径(LAED)、左室舒张末期前后径(LVED)、左室射血分数(LVEF)、短轴缩短率(INFS)、二尖瓣峰值速度比值(E/A)等指标.对两组的上述指标进行比较,并分析DCM患儿的心率变异性与心脏结构和功能的相关性.结果 对照组男孩的SDNN、SDANN,SDNN index、rMSSD、PNN50、TP均高于女孩.DCM组男女的HRV差异无统计学意义,与对照组比较,DCM组的LF/HF显著升高,其余HRV指标明显下降;与对照组相比,DCM组的LAED和LVED显著增大,LVEF、LVFS及二尖瓣E/A均显著降低;DCM患儿的SDNN与LAED呈负相关关系,与LVEF、LVFS和E/A呈正相关关系(P均<0.05),其余HRV指标与心脏大小和功能无相关性.结论 DCM患儿存在明显的自主神经平衡失调,DCM患儿HRV的性别差异消失,均表现为迷走神经张力减退、交感神经相对激活.DCM患儿HRV中部分指标的改变与房室腔扩大和心功能下降有一定的相关性,部分HRV指标相对独立,将HRV指标与超声测量指标结合起来能更好地指导临床.     

心肌炎患儿与正常儿童心率及心率变异性比较分析

目的 研究不同年龄心肌炎患儿的心率及心率变异性(HRV)改变的特点和相关性.方法 对120例心肌炎患儿进行24 h全程动态心电图检查,与804例正常儿童的心率及HRV进行比较分析.结果 7岁以下患儿最慢心率较正常增快,最快心率比较变化无差异,7～18岁患儿心率监测指标均异常;3～7岁心肌炎患儿与正常儿童比较每5分钟R-R间期平均值的标准差(SDANN)无变化,各组其他HRV指标均降低.结论 心肌炎患儿的HRV普遍降低,提示心脏自主神经系统的总体功能受损,以迷走神经张力降低为主,交感神经张力相对增强.     

川崎病患儿心率变异性与冠状动脉损害的相关性研究

目的 探讨川崎病(KD)患儿的心率变异性(HRV)指标与肌钙蛋白I(cTnI)和氨基末端脑钠肽前体(NT-proBNP)的相关性及其在预后中的应用价值。方法 将130 例KD 患儿分为冠状动脉损害组(n=47, CAL 组)和无CAL 组(n=83, NCAL 组), 同期选取110 例健康儿童为对照组, 29 例非心血管疾病恢复期患儿为非KD 组。各组儿童均行长程HRV 指标检测及分析。检测KD 组及非KD 组患儿血清NT-proBNP 及cTnI 水平。结果 同年龄性别KD 组患儿正常窦性N-N 间期标准差(SDNN)、相邻N-N 间期标准差的平均值(SDNNindex)、相邻N-N 间期之差>50 ms 的心搏数占心搏总数的百分数(PNN50)、极低频功率(VLF)、低频功率(LF)和高频功率(HF)值较对照组均明显下降, LF/HF 值较对照组升高(P<0.05)。CAL 组SDNN、全部记录中每5 min N-N 间期平均值的标准差(SDANN)、SDNNindex、相邻N-N 间期差值的均方根值(rMSSD)、PNN50、VLF、LF 和HF 值均低于对照组和非KD 组, LF/HF 值高于对照组(P<0.05)。CAL 组及NCAL 组的cTnI 和NT-proBNP 水平均高于非KD 组(P<0.05)。KD 患儿cTnI 与SDNN、HF 呈负相关, 与LF/HF 呈正相关(P<0.05);NT-proBNP 与SDNN、SDANN、HF 呈负相关(P<0.05)。结论 HRV 指标对KD 患儿的CAL 判断具有一定的临床意义。     

蒽环类药物心脏毒性早期监测方法的评价

目的：蒽环类药物（ANT）对白血病、实体瘤疗效显著,但因其心脏毒性,严重影响患儿远期生活质量。本研究旨在评价二维超声（2DE）和血清生化指标早期监测ANT心脏毒性的价值。方法：70例接受ANT化疗患儿（ANT剂量124±73 mg/m2,随访22±13月）进行2DE检测,包括常规指标（左室内径及室壁厚度、射血分数、E/A等）、心肌工作指数（MPI）、组织多普勒（TDI）, 并采血检测血清肌钙蛋白(CTnI)、脑利钠肽(BNP)含量。37例健康体检儿为对照组。结果：病例组2DE常规指标与对照组比较差异无统计学意义。与对照组比较,病例组左、右室MPI明显增大（0.237±0.06、0.171±0.05 vs 0.203±0.06、0.140±0.04,P<0.01）,TDI示病例组左室侧基底段、中间段舒张晚期组织运动峰值速度（Am）,室间隔侧基底段、中间段Am,右室侧基底段、中间段Am均较对照组明显增高;左室侧中间段舒张早期组织运动峰值速度（Em）/Am,室间隔侧基底段、中间段Em/Am差异有统计学意义（P<0.05）。且随着ANT累积剂量增加,MPI和TDI变化更加明显。血清CTnI、BNP水平与对照组比较差异无统计学意义。结论：接受ANT治疗者需长期监测心功能;MPI、TDI能早期反映ANT对患者心功能的影响。     

小儿川崎病心率变异性指标与肌钙蛋白Ⅰ和氨基末端脑钠肽前体相关性分析

目的分析川崎病(KD)患儿心率变异性(HRV)指标与肌钙蛋白Ⅰ(c TnⅠ)和氨基末端脑钠肽前体(NT-pro BNP)的相关性。方法选取2013年10月至2015年10月青海省妇女儿童医院收治的急性期KD患儿118例为KD组,根据是否伴有冠状动脉损害(CAL)分为CAL组43例和非CAL(NCAL)组75例,同期非心血管疾病恢复期患儿32例为非KD组及健康儿童106例为对照组。各组均行长程HRV指标检测,检测KD组和非KD组c TnⅠ、NT-pro BNP水平。结果 KD组患儿恢复期LF/HF值显著低于急性期,其余HRV指标均显著高于急性期(P0.05或P0.01);KD组患儿急性期和恢复期LF/HF值显著高于对照组,其余各指标均显著低于对照组(P0.05或P0.01)。CAL组HRV时域各项指标及高频功率(HF)、低频功率(LF)、极低频功率(VLF)均低于对照组和非KD组(P0.05或P0.01),且全部记录中每5 min N-N间期平均值的标准差(SDANN)、正常窦性N-N间期标准差(SDNN)及相邻N-N间期之差50 ms的心搏数占心搏总数百分比(PNN50)亦低于NCAL组(P0.01),而LF/HF值高于对照组、非KD组及NCAL组(P0.05或P0.01)。CAL组和NCAL组c TnⅠ、NT-pro BNP水平显著高于非KD组,其中CAL组c TnⅠ水平高于NCAL组(P0.01)。KD患儿c TnⅠ水平与SDNN、HF呈负相关,而与LF/HF值呈正相关(P0.01);NT-pro BNP水平与SDANN、SDNN、HF均呈明显负相关(P0.01)。结论 KD患儿存在心脏自主神经功能损害,HRV指标对KD患儿发生CAL的判断具有一定临床价值。     

直立倾斜试验诱发QT 间期缩短1 例报告并文献复习

目的探讨继发性QT间期缩短的原因。方法回顾分析1例血管迷走性晕厥心脏抑制型患儿在直立倾斜试验中诱发QT间期缩短,同时复习相关文献。结果患儿,男,12岁,晨起刷牙时晕厥1次就诊。心电图示窦性心动过缓,心率55次/min,QT及QTc间期正常。动态心电图示窦性心律,平均心率70次/min,房性期前收缩3次,QT及QTc均值正常。彩色超声心动图示心脏内结构大致正常,左心室收缩功能正常。脑电图无异常。头颅CT无异常。空腹血糖5.2 mmol/L。基础直立倾斜试验为阳性,血管迷走性晕厥-心脏抑制型。直立倾斜试验过程中,动态心电图同步监测显示,患儿在直立倾斜16 min时出现窦性停搏,随即晕厥,窦性停搏分别为2.9 s和11.4 s,并行人工胸外按压。在出现窦性停搏之前可见QT间期缩短(QT=330 ms),QTc间期(按心率校正的QT间期=QT/(1/2)(RR))缩短(QTc=320 ms),QT/QTp=78%[QTp:QT间期预测值=656/(1+心率/100),正常QT间期的下限为QTp的88%]。结论迷走神经张力增高可诱发QT间期缩短。     

以晕厥为首发表现的先天性长QT综合征儿童的临床特征及随访

目的探讨儿童先天性长QT综合征(LQTS)的致病基因、临床特征及治疗随访情况。方法收集2016年8月至2023年3月于首都医科大学附属北京儿童医院心脏内科收治的16例以晕厥为首发表现的先天性LQTS患儿的临床资料、基因检测结果及随访资料, 并进行回顾性分析。结果 16例LQTS患儿, 首次晕厥发病年龄为1.3～13.3(7.37±3.41)岁, 首次晕厥到临床确诊时间间隔0～48(14.8±16.2)个月。13例(81.3%)患儿有明确的晕厥诱因, 其中9例为运动诱发, 4例为情绪激动诱发。13例LQTS患儿完成基因检测, 其中12例(92.3%)患儿发现了来自于KCNQ1、KCNH2、SCN5A基因的致病或疑似致病突变。16例患儿矫正QT间期(550.0±50.2)ms, 均大于460 ms。Schwartz评分为6.0(5.0, 6.0)分, 均≥4分。全部)患儿初始应用美托洛尔或普萘洛尔治疗, 其中14例随访至2023年9月, 3例患儿晕厥再发作, 5例患儿自行停药。1例大剂量美托洛尔治疗患儿(LQT2型)反复晕厥发作后换用美西律治疗有效。1例患儿不耐受大剂量普萘洛尔行左侧心脏交...     

儿童经胸植入左房左室心外膜永久双腔起搏器疗效探讨

目的 探讨植入左房左室心外膜永久双腔起搏器治疗儿童完全性房室传导阻滞的可行性、优越性及疗效.方法 本组11例完全性房室传导阻滞患儿,中位年龄4.0岁(0.5 ～7.6岁),其中男6例,女5例,药物治疗均无效,均植入心外膜左房左室永久双腔起搏器.本组术前均为右室临时或永久起搏方式,3例存在心功能不全.经左侧第4肋间腋前线开胸,将心外膜起搏电极固定于左心耳及左室心外膜,于腹部左季肋下制作囊袋置入脉冲发生器,经皮下隧道连接起搏电极导线.术后随访心功能变化、起搏参数和功能、心电图参数.结果 11例患儿均成功植入左房左室心外膜永久双腔起搏器.左室起搏QRS间期较术前右室起搏QRS间期明显缩短[(140+24)ms vs.(180±33)ms,t=8.8,P＜0.05].3例右室或右房右室起搏方式存在心功能受损患儿,植入左房左室心外膜起搏器随访2 ～ 14个月,左室射血分数逐渐恢复正常(65％±8％),与植入前左室射血分数(30％±15％)相比差异有统计学意义(t=5.6,P＜0.05).其余8例患儿随访期间心脏大小及左室收缩功能保持在正常范围.全部患儿随访显示起搏及感知功能良好.结论 在因条件限制需植入心外膜永久心脏起搏器的房室传导阻滞患儿,可选择左房左室心外膜起搏,以尽可能小的创伤、最大限度保护患儿的心脏功能,有效避免或逆转起搏器综合征,可作为植入心脏起搏器心外膜电极首选及常规部位.     

婴幼儿心脏肿瘤的治疗与预后

目的:探讨婴幼儿心脏肿瘤的诊断、治疗经验以及随访情况。方法:回顾性分析大连医科大学附属大连市儿童医院心脏中心2013年8月至2019年11月收治的9例婴幼儿心脏肿瘤患儿的住院资料及随访结果。其中,男7例,女2例;年龄(6.33±8.06)个月,范围在1 d至27个月;入院体重(6.57±2.89)kg,范围在3.7～1...     

氢氯噻嗪、依那普利、美托洛尔、螺内酯联合治疗扩张型心肌病疗效评估

目的研究氢氯噻嗪、依那普利、美托洛尔、螺内酯联合治疗扩张型心肌病(DCM)的疗效。方法DCM患儿23例,纽约心脏病协会(NYHA)心脏功能Ⅲ-Ⅳ级,确诊病例予氢氯噻嗪、依那普利、螺内酯口服,心功能Ⅳ级者加用小剂量地高辛口服,顽固性心衰患儿加用多巴胺、多巴酚丁胺静滴。心功能改善到Ⅱ-Ⅲ级时加用美托洛尔口服,2-4周将依那普利、美托洛尔剂量加倍,每2-4周抽血观察肾脏功能和血清钾。服药后1、3、6、12、24个月随访患儿.病情恶化者再次住院治疗。各随访阶段均判断心脏功能,检查同步12导联体表心电图(12-ECG)、二维超声心动图(2-DE),用2-DE测量患儿左心房(LA)、左心室(LV)内径及左室射血分数(EF)、短轴缩短率(FS)。结果随访1-26个月,病例均遵医嘱用药。随访死亡3例,死因为严重肺部感染、窒息。12-ECG示心房纤颤2例,未使用抗心律失常药物治疗,6个月后自行转复为窦性心律,每2-4周观察肾脏功能和血清钾结果正常。治疗前EF、FS分别为(42.45±13.07)%和(21.14±8.06)%,用药后随访3个月时接近60%和30%,12个月时>60%和.30%;LA、LV内径治疗前分别为(28.96±8.54)mm及(46.09±14.09)mm,随访3个月时(26.00±0.01)mm及(40.50±212)mm,治疗3个月后LA、LV明显缩小(t=-2.892,-4.509P<0.05,0.01)。结论联合应用氢氯噻嗪、依那普利、美托洛尔、螺内酯治疗小儿DCM能明显延缓或防止心肌重塑进展,改善心脏功能,提高生存质量。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.