自主神经介导性晕厥儿童在直立倾斜试验中阳性反应与并发症的早期发现和处理

目的探讨如何早期发现并及时处理自主神经介导性晕厥(NMS)患儿在直立倾斜试验(HUTT)中的阳性反应与并发症。方法回顾分析行HUTT检查阳性确诊NMS患儿的临床资料。结果 201例NMS患儿中,男性95例、女性106例,年龄7岁4月～16岁9月,血管迷走性晕厥(VVS)103例、体位性心动过速综合征(POTS)98例。患儿在HUTT中,晕厥再现5例(2.5%),均为VVS;出现并发症200例(99.5%),其中窦性心动过速182例(90.5%)、窦性心动过缓38例(18.9%),包括心率骤升骤降20例(10.0%),心脏停搏2例(1.0%),交界性逸搏心律2例(1.0%),II度房室传导阻滞1例(0.5%),房性早搏1例(0.5%),抽搐2例(1.0%),暂时性失语2例(1.0%)。达到阳性反应后迅速将倾斜床恢复至水平位,晕厥者予以吸氧并抬高及按摩双下肢,心脏停搏者予以胸外心脏按压,部分清醒后予口服牛奶等处理,全部患儿短时间内临床表现消失,意识、心率、血压、心电图恢复正常,无遗留后遗症及死亡病例。181例(90.0%)患儿在HUTT中有晕厥先兆,108例(53. 7%)先有晕厥先兆其后达到阳性反应标准,其中103例在晕厥先兆出现后的8分钟内达到阳性反应标准;47例(23.4%)晕厥先兆与到阳性反应几乎同时出现。结论 NMS儿童在HUTT时存在一定风险,早期发现并及时处理阳性反应和并发症,是降低试验风险的关键。     

儿童血管迷走性晕厥直立倾斜试验中心电图的变化

目的 探讨儿童血管迷走性晕厥(VVS)直立倾斜试验(HUTT)中ECG的变化.方法 选取本院门诊及住院晕厥患儿123例,经详细询问患儿病史,行体格检查、常规12导联ECG及头颅CT等一系列检查,疑诊VVS 65例.疑诊VVS患儿停用可能影响自主神经功能的饮食,停用血管活性药物5个半寿期以上(6～8 d),行HUTT或舌下含化硝酸甘油倾斜试验.结果 VVS患儿HUTT中可出现各种心律失常,以窦性心动过速多见[40例(61.5%)],结性逸搏心律次之[23例(35.4%)].HUTT阳性类型:血管抑制型45例(69.2%),混合型16例(24.6%),心脏抑制型41例(6.2%).结论 VVS儿童HUTT中可出现各种心律失常.VVS患儿应严密观察ECG变化,必要时积极干预,以免发生意外.     

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目的探讨直立倾斜试验(HUTT)对儿童血管迷走性晕厥(VVS)反复发作的预测价值。方法2001-01—2006-08在中南大学湘雅二医院晕厥专科门诊就诊或住院的不明原因晕厥患儿251例,男112例,女139例,年龄418(12.25±3.27)岁。依临床晕厥发生频次分A组(晕厥发作仅1次,n=54)、B组(晕厥发作24次,n=137)与C组(晕厥发作≥5次,n=60)。HUTT在取得知情同意后采用基础直立倾斜试验(BHUT,n=251)及舌下含服硝酸甘油倾斜试验(SNHUT,n=92)。结果(1)HUTT阳性率与晕厥频次关系:BHUT阳性率随晕厥频次增加而递增(χ2=4.285,P>0.05),SNHUT阳性率与晕厥频次不呈线性关系(χ2=1.316,P>0.05),HUTT总阳性率(指BHUT阳性率+SNHUT阳性率)亦随晕厥频次增加而递增(χ2=3.809,P>0.05)。(2)HUTT反应类型与晕厥频次关系:无论是BHUT还是SNHUT,反应类型以血管抑制型为主,BHUT或SNHUT在不同晕厥频次组间比较无明显差异(分别为χ2=3.008,P>0.05;χ2=2.426,P>0.05)。结论HUTT与儿童VVS临床晕厥反复发作频次无明显关系,对儿童VVS临床反复晕厥发作没有预测价值。     

直立倾斜试验导致暂时性失语1例

直立倾斜试验(HUTT)广泛应用于鉴别儿童不明原因晕厥,是诊断血管迷走性晕厥(VVS)的准金标准[1]。HUTT再现晕厥发作存在一定风险,能诱发严重心律失常等并发症[2]。本文报道HUTT时引起的儿童暂时性失语1例。1病例资料患儿女,10岁。因头晕4d于2011-03-01来中南大学湘雅二医院儿科就诊。患儿头晕均发生在早晨起床时,     

直立倾斜试验受试儿童心理恐惧的评估

目的 探讨直立倾斜试验（HUTT）的不同倾斜角度及不同试验反应对受试儿童心理恐惧的影响。方法 不明原因晕厥或先兆晕厥儿童107 例,年龄5.5~17.8（12.0±2.8）岁,男52 例,女55 例,随机选取60°、70°或80°进行基础直立倾斜试验（BHUT）,BHUT 阴性者保持同一倾斜角度进行舌下含化硝酸甘油倾斜试验（SNHUT）。采用Wong-Baker 面部表情疼痛量表,在试验结束时让受试儿童对HUTT 过程中心理恐惧程度进行自我评定。结果 倾斜角度为60°、70°、80°的3 组受试儿童HUTT 阳性率、血流动力学改变、反应类型分布差异均未见统计学意义（P>0.05）。60°、70°、80° 3 种倾斜角度对受试儿童心理恐惧程度的影响呈递增趋势,但差异无统计学意义（P>0.05）。HUTT 阳性儿童（n=76）心理恐惧程度明显高于HUTT 阴性儿童（n=31）（P<0.01）。结论 HUTT 可引起受试儿童心理恐惧;60°、70°、80° 3 种倾斜角度受试儿童心理恐惧呈递增趋势,但差异不显著;HUTT 阳性反应可显著增加受试儿童心理恐惧。     

血管迷走性晕厥患者心电图改变研究进展

部分血管迷走性晕厥(VVS)患者在直立倾斜试验(HUTT)诱导晕厥发作过程中出现各种心律失常,其中以缓慢性心律失常最为常见.心电图各波形态在HUTT阳性和HUIT阴性VVS患者间也有所不同,其主要原因与VVS患者本身存在自主神经功能紊乱有关.了解VVS患者的心电图改变及其对HUTT结果、治疗效果及预后等各方面的预测价值将对VVS的临床诊治提供可靠依据.     

血管迷走性晕厥患者心电图改变研究进展

部分血管迷走性晕厥(VVS)患者在直立倾斜试验(HUTT)诱导晕厥发作过程中出现各种心律失常,其中以缓慢性心律失常最为常见.心电图各波形态在HUTT阳性和HUIT阴性VVS患者间也有所不同,其主要原因与VVS患者本身存在自主神经功能紊乱有关.了解VVS患者的心电图改变及其对HUTT结果、治疗效果及预后等各方面的预测价值将对VVS的临床诊治提供可靠依据.     

血管迷走性晕厥患者心电图改变研究进展

部分血管迷走性晕厥(VVS)患者在直立倾斜试验(HUTT)诱导晕厥发作过程中出现各种心律失常,其中以缓慢性心律失常最为常见.心电图各波形态在HUTT阳性和HUIT阴性VVS患者间也有所不同,其主要原因与VVS患者本身存在自主神经功能紊乱有关.了解VVS患者的心电图改变及其对HUTT结果、治疗效果及预后等各方面的预测价值将对VVS的临床诊治提供可靠依据.     

儿童血管迷走性晕厥的临床特征及血浆和血小板中5-羟色胺的变化

目的 探讨小儿血管迷走性晕厥的临床特征和血浆、血小板中5-羟色胺(5-HT)的变化.方法 2006年10月-2009年2月在首都儿科研究所经直立倾斜试验(head-up tilt test,HUTT)确诊为血管迷走性晕厥(VVS)患儿41例(HUTT阳性组),诊断标准参照基础HUTT对儿童不明原因晕厥的诊断研究,男17名,女24名,年龄6～14岁,平均年龄(10.5 ±1.8)岁.匹配健康儿童(对照组):当地幼儿园和中小学36名健康小儿,男16名,女20名,年龄9～14岁,平均年龄(10.7±1.5)岁.分析晕厥诱因和先兆症状、HUTT反应方式、晕厥发作时间、VVS患儿静息状态各亚型血压和心率变化等临床特点.全体研究对象抽取静脉血3 ml,用双抗体夹心酶标免疫分析(ELISA)法对41例血管迷走性晕厥患儿及36名健康儿童的血浆和血小板中5-HT进行测定.结果 ①41例血管迷走性患儿平均年龄为(10.5±1.8)岁,女童比例高于男童,为1.4:1.②VVS先兆症状:患儿中33例存在晕厥先兆(80.4%),其中头晕发生率高达78.8%.③VVS发生诱因:儿童VVS发作前常存在诱发因素,包括:长久站立、劳累、情绪影响等.其中长久站立比例最高,达90.2%.④HUTT平均反应时间及晕厥持续时间:基础直立倾斜试验(BHUT)阶段平均反应时间为(20.6±8.6)min;舌下含化硝酸甘油激发倾斜试验(SNHUT)阶段平均反应时间(5.0±2.2)min.晕厥持续时间均短于5 min.⑤HUTT不间反应类型的分布:血管抑制型61.0%,混合型24.4%,心脏抑制型14.6%.⑥血压和心率的比较:VVS患儿和正常儿童静息状态下基础心率、收缩压、舒张压相比差异无统计学意义;VVS患儿中血管抑制型、混合型和心脏抑制型静息状态下基础心率、收缩压、舒张压相比差异无统计学意义.⑦VVS患儿基础状态和HUTT阳性时血浆中5-HT较对照组差异无统计学意义[(27.51±1.32)μg/Lvs.(27.28±2.48)μg/L,t=0.518,P=0.606;(27.51±1.32)μg/L vs.(28.05 ±1.40)μg/L,t=2.044,P=0.167],基础状态下血小板5-HT与对照组之间差异无统计学意义[(82.30 ±6.06)10~9ng/L vs.(79.88±5.79)10~9ng/L,t=1.788,P=0.780].⑧VVS患儿基础状态下和HUTT阳性时的血小板5-HT比较差异有统计学意义[(82.30±6.06)10~9ns/L vs.(97.90±6.59)10~9ng/L,t=11.26,P=0.00].结论 VVS患儿具有明显的临床特征;VVS患儿基础状态和晕厥(或晕厥先兆发生时)血浆中5-HT变化不明显;VVS患儿晕厥或晕厥先兆发生时血小板5-HT明显升高,提示中枢5-HT系统可能参与了VVS的发病过程.     

直立倾斜试验诱发迷走神经性房颤1 例报告

目的探讨直立倾斜试验中诱发出阵发性心房颤动的原因。方法回顾分析1例在直立倾斜试验中诱发出迷走神经性房颤的血管迷走性晕厥患儿的临床资料,同时复习相关文献。结果患儿,女,7岁,1年内晕厥2次。患儿无阳性体征。血常规、血生化检查均无异常,脑电图、头颅CT、心电图、心脏彩色多普勒超声检查结果均无异常。患儿在直立倾斜试验中出现5.3s窦性静止,继而发生房室连接处逸搏心律、房性早搏、心房颤动。患儿经健康教育和自主神经功能锻炼治疗,半年后随访,未出现晕厥,未发生心房颤动。结论直立倾斜试验中迷走神经张力增高可诱发迷走神经性房颤。     

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.     

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   

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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.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

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.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.