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目的探讨舌下含服硝酸甘油倾斜试验(SNHUT)对儿童血管迷走性晕厥(VVS)的诊断价值。方法2001年3月至2005年5月在中南大学湘雅二医院儿童晕厥专科就诊或住院的不明原因晕厥(UPS)患儿143例,年龄4～18(12.10±3.03)岁,男58例,女85例。电动倾斜床直立倾斜70°行基础直立倾斜试验(BHUT),并对其阴性者中的64例在同一角度直接给予舌下含服硝酸甘油片0.2mg,再次评价试验结果。用SPSS11.0软件进行微机统计学处理。结果(1)BHUT阳性率29.4%(42/143),其中女性占73.8%(31/42);SNHUT64例,阳性44例,阳性率为68.7%。SNHUT显著地提高了VVS的检出率。(2)出现阳性结果的时间BHUT为(21.31±13.24)min,SNHUT为(5.41±4.23)min。(3)反应类型BHUT及SNHUT阳性患儿共86例,血管抑制型83.7%(72/86),女性占53.5%(46/86);心脏抑制型7.0%(6/86),均为女性;混合型9.3%(8/86),女性占62.5%(5/8)。(4)副反应舌下含服硝酸甘油64例,未见明显不耐受现象或其他副反应。结论SNHUT能提高儿童VVS诊断阳性率,副反应小,使用方便,可在儿科临床推广。     

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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。     

血管迷走性晕厥的诊断与防治

晕厥是引起一过性意识丧失最常见的原因,引起晕厥的病因主要有器质性晕厥、神经介导的反射性疾病、代谢性疾病和精神性疾病,其中自主神经介导性晕厥最常见.自主神经介导性晕厥主要包括血管迷走性晕厥(VVS)、体位性心动过速综合征(POTS)及直立性低血压(OH)等.直立倾斜试验(HUT)是VVS诊断中的重要客观检查手段,它包括基础直立倾斜试验(BHUT)和舌下含化硝酸甘油激发的倾斜试验(SNHUT).VVS的治疗主要包括直立训练等物理疗法、增加患儿盐和液体的摄入量及药物治疗等.     

儿童直立倾斜试验阳性反应时心律失常分析

目的探讨儿童直立倾斜试验(HUTT)阳性反应时心律失常发生情况及临床意义。方法对2000年9月~2005年7月在晕厥专科门诊就诊或住院的不明原因晕厥或接近晕厥患儿进行HUTT,阳性反应77例(男28例,女49例;年龄7~18岁),分析HUTT时心律失常发生情况,采用SPSS11.0软件进行统计。结果出现心律失常76例(98.7%),发生窦速71例(92.2%),其中60例在心率达最快后5min[平均(1.5±1.7)min]内发生阳性反应。发生缓慢性心律失常26例(33.8%),与舌下含服硝酸甘油倾斜试验(SNHUT)相比,基础直立倾斜试验(BHUT)中发生率高(P<0.05),而不同性别、年龄组(<12岁、≥12岁)间差异无显著性(P>0.05),以窦缓多见(20/26例),其次为交界性逸搏心律、窦性停搏、交界性逸搏。5例窦性停搏患儿停搏时间平均为18.2s(1.5~60s)。结论HUTT时心率突然明显增快时应警惕发生阳性反应;缓慢性心律失常在BHUT中发生率较高,常见类型有窦缓、交界性逸搏心律、窦性停搏、交界性逸搏等;应积极预防和处理严重阳性反应。     

直立倾斜试验阳性反应特点及临床特征

目的探讨儿童直立倾斜试验(HUT)阳性反应特点及临床特征。方法以49例不明原因晕厥及只有晕厥先兆症状患儿为研究对象,直立倾斜试验(HUT)(倾斜角度60度,试验持续时间45min)过程中动态观察血压、心率、心电图变化,分析其阳性反应血流动力学改变及其临床特征。结果HUT阳性反应时常见晕厥先兆症状为头痛、头晕、胸闷、气短、面色苍白、出汗、乏力、站立不稳、视物模糊、听觉下降及消化道症状。28例阳性反应中,血管抑制型反应7例(25%).表现为血压下降,心率加快;心脏抑制型反应3例(11%),表现为心率减慢,血压无变化;混合型反应4例(14%),表现为心率、血压均明显下降。体位性心动过速综合征(POTS)10例(36%),表现为HUT10min内心率增快30次以上或心率达120次/min以上。直立性低血压4例(14%).表现为HUT3min内血压明显下降。结论HVT阳性反应症状可作为儿童不明原因晕厥鉴别诊断的主要依据。     

舌下含服硝酸甘油直立倾斜试验对儿童血管迷走性晕厥的诊断价值

晕厥是儿科临床常见症状,病因复杂,儿童晕厥中以血管迷走性晕厥(vasovagal syncope,VS)最常见,50%以上的晕厥属于血管迷走性晕厥[1]。直立倾斜试验(head upright tilt test,HUT)对不明原因晕厥进行了诱发试验,已成为诊断VS的重要方法,为VS提供了直接依据和标准[2~4]。近几年来HUT的方法学已成为研究的热点,本组拟通过对58例不明原因晕厥患儿的试验观察,以探讨适用于儿童的简便易行的倾斜试验方法。对象和方法一、对象2001年8月以来在我院门诊和住院的不明原因晕厥患儿58例,男25例,女33例;年龄6~16岁,平均年龄(11.0±2.2)岁;病程5d~3年;…     

不明原因晕厥患儿直立倾斜试验中血流动力学反应模式及意义

目的　探讨不明原因晕厥患儿在直立倾斜试验中血流动力学反应模式及构成比例。方法　对 2 0 0 1年1月至 2 0 0 3年 12月北京大学第一医院儿科收治的 90例不明原因晕厥患儿在安静环境下进行直立倾斜试验或硝酸甘油激发的直立倾斜试验 ,持续监测患儿心率和血压变化。结果　 90例不明原因晕厥患儿直立倾斜试验中 ,经典的血管迷走性反应者 4 9例 ( 5 4 4 % ) ,其中血管抑制型 33例 ( 36 7% ) ,心脏抑制型 6例 ( 6 7% ) ,混合型 10例( 11 1% )。正常直立反应者 12例 ( 13 3% ) ,体位性心动过速反应者 2 8例 ( 31 1% ) ,直立性低血压反应者 1例( 1 1% ) ,没有发现自主神经反应障碍型及心脏变时功能障碍型。结论　不明原因晕厥患儿在直立倾斜试验中以经典的血管迷走性反应为主 ,其次为体位性心动过速综合征的反应 ,还可能出现体位性低血压等其他的异常血流动力学变化     

儿童体位性心动过速综合征的临床特征及随访研究

目的　探讨儿童体位性心动过速综合征 (POTS)的临床特征、诊断标准及治疗方案。方法　以符合诊断标准的 28例POTS患儿为研究对象,分析年龄分布、病程及基础血流动力学指标,同时观察其各种临床表现的发生频率及诊治效果。结果　诊断为POTS的患儿 28例,占 88例不明原因晕厥或起立后头晕就诊患儿的 32%,其中男 11例,女 17例。男∶女约为 1∶1.5。年龄 6~16岁,平均为(11.6±2.2)岁,其中 6~10岁 5例,占 18%, 10~16岁(包括 10岁)23例,占 82%。病程为 1个月~6年,平均(13.3±19.6)个月,半数以上在 6个月以内;最常见的临床表现为起立后出现头晕或眩晕、晕厥、胸闷、头痛、心悸、面色改变、视物模糊、倦怠、晨起不适等直立不耐受或直立调节障碍症状, 14例伴有恶心或呕吐等消化道症状;在直立试验(先安静平卧 10min,然后直立 10min)或直立倾斜试验(HUT)过程中,POTS患儿最常见的异常表现为在直立或倾斜后 10min内,心率增加≥35次 /min,部分患儿心率最大值≥120次 /min,出现异常表现的时间平均为 5min左右;但仅有 10例的患儿在直立后即出现异常表现, 18例患儿需要HUT确定诊断。12例POTS患儿曾被误诊为癫痫或心肌炎,误诊率达 43%。经过生活指导治疗及药物治疗的综合治疗后,大多数患儿症状可缓解或消失。结论　POTS常见     

血管迷走性晕厥患儿40例

目的探讨不同类型血管迷走性晕厥(VVS)患儿的临床特征及实验室检查指标间的差异。方法经常规病史询问、体格检查、卧立位血压、辅助检查、直立倾斜试验(HUT)确诊的VVS患儿40例,比较不同类型患儿的临床特征及实验室指标间的差异。结果VVS患儿的血流动力学类型以血管抑制性为主。不同类型VVS患儿的临床特征,包括晕厥的诱因、先兆、发作频率、持续时间、基础心率、血压及血清电解质水平等均无显著差异。结论血管抑制型反应是血管迷走性晕厥患儿的主要血流动力学类型。     

直立倾斜试验对不明原因晕厥患儿的诊断价值

目的：讨基础直立倾斜试验对不明原因晕厥(UPS)患儿的诊断价值。方法：UPS患儿30例,年龄6～18岁,平均(11.74±2.82)岁,采用电动倾斜床取头高脚低位直立倾斜70° 后每 5 min自动测量血压和心电变化,评价倾斜试验结果。结果：30例UPS中倾斜试验阳性10例(占 33.3%),反应类型为心脏抑制型及血管抑制型各5例,晕厥发作在倾斜站立10～40 min,平均(24.0±12.2) min。结论：倾斜试验是诊断儿童血管迷走性晕厥(VVS)的有效方法,对临床UPS患儿具有很好地诊断价值。     

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.