直立不耐受患儿晕厥发作的情况及相关因素研究

目的 探究直立不耐受患儿晕厥发作的情况及相关因素.方法 选择首都医科大学附属北京潞河医院儿科诊治的46例直立不耐受的患儿,探究直立不耐受患儿晕厥发作的情况及相关因素.结果 46例直立不耐受患儿发生晕厥发作的共18例,占39.13%,晕厥组患儿的体重指数为(16.49±2.96) kg/m2,明显低于非晕厥组,且晕厥组伴随冷汗、视物模糊、面色苍白症状明显多于非晕厥组,差异均具有统计学意义(P<0.05).通过相关影响因素Logistic分析,体重指数、持久站立、晕车晕船史是导致直立不耐受患儿出现晕厥发作的危险因素(P<0.05).结论 直立不耐受患儿晕厥发作率较高,且与体重指数、持久站立、晕车晕船史密切相关,临床医师要有根据性的给予预防措施.     

体位性心动过速综合征109例临床特征分析

探讨儿童体位性心动过速综合征（POTS）的临床特点。方法　2008年5月至2009年10月于北京大学第一医院儿科门诊就诊,经直立试验或直立倾斜试验确诊POTS的患儿109例（POTS组）,平均年龄（11.79±2.55）岁;20名健康儿童为对照组,平均年龄（11.55±3.65）岁。对每例POTS患儿详细询问病史并进行体格检查,对比分析两组儿童在生活习惯、家族史及体质特征方面特点,总结POTS组患儿发病的临床特征。结果　与对照组相比,POTS组患儿在性别比例、年龄、身高、体重、平卧心率、平均动脉压方面差异无统计学意义。POTS患儿主要症状以晕厥多见（52.3%）,42.2％症状发作频繁（就诊时 > 10次）,主要症状发生季节以夏秋季多见（42.1%）, 发作持续时间多在1 min以内（29%）。83.5%患儿发作前有诱因,发作诱因以持久站立为多见（50.5%）,发作前多伴有先兆症状（78.0%）,其中以头晕、黑矇、大汗、面色苍白最为常见。18例（16.5%）患儿有伴随症状,32例（29.4%）发作后仍有不适,以乏力最常见（24例）。90例（82.6%）患儿无既往疾病史,30例（27.6%）有直立不耐受家族史,46例（42.2%）有晕车经历。POTS组清淡饮食者居多（41.3%）,水摄入较少（63.3%）。结论　儿童POTS多发生于学龄期及青春期,晕厥发生率较高,发作季节以夏秋季多见,持久站立、体位改变、精神紧张以及感染为常见诱因。部分患儿有直立不耐受家族史,且易伴有晕车经历。     

儿童血管迷走性晕厥反复发作相关因素分析

目的探讨影响儿童血管迷走性晕厥(VVS)反复发作的相关因素。方法收集125例确诊为VVS患儿的临床资料,根据晕厥首次发作至直立倾斜试验之前5年内的发作次数,分为晕厥发作次数2、3次的低频次组及≥4次的高频次组,对两组患儿资料进行统计分析。结果 125例VVS患儿中,低频次组84例(67.2%),高频次组41例(32.8%)。单因素分析结果显示,直立倾斜试验检查年龄、晕厥发作时间、发作诱因、晕车史、阳性家族史是VVS高频次发作的相关因素。非条件logistic回归分析结果显示,发作诱因(OR=3.723,95%CI:1.163~11.918,P=0.027)、晕车史(OR=5.929,95%CI:2.066~17.015,P=0.001)、阳性家族史(OR=6.794,95%CI:2.006~23.013,P=0.002)是VVS高频次发作的独立危险因素。结论非持久站立引起的其他发作诱因、晕车史、阳性家族史对预测VVS患儿高频次晕厥发作具有重要临床意义。     

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目的探讨儿童体位性心动过速综合征(POTS)的临床特点。方法2008年5月至2009年10月于北京大学第一医院儿科门诊就诊,经直立试验或直立倾斜试验确诊POTS的患儿109例(POTS组),平均年龄(11.79±2.55)岁;20名健康儿童为对照组,平均年龄(11.55±3.65)岁。对每例POTS患儿详细询问病史并进行体格检查,对比分析两组儿童在生活习惯、家族史及体质特征方面特点,总结POTS组患儿发病的临床特征。结果与对照组相比,POTS组患儿在性别比例、年龄、身高、体重、平卧心率、平均动脉压方面差异无统计学意义。POTS患儿主要症状以晕厥多见(52.3%),42.2%症状发作频繁(就诊时>10次),主要症状发生季节以夏秋季多见(42.1%),发作持续时间多在1min以内(29%)。83.5%患儿发作前有诱因,发作诱因以持久站立为多见(50.5%),发作前多伴有先兆症状(78.0%),其中以头晕、黑矇、大汗、面色苍白最为常见。18例(16.5%)患儿有伴随症状,32例(29.4%)发作后仍有不适,以乏力最常见(24例)。90例(82.6%)患儿无既往疾病史,30例(27.6%)有直立不耐受家族史,46例(...     

儿童晕厥的病因及临床特征分析

目的分析儿童晕厥的病因及临床特征。方法回顾性分析以晕厥为主诉的128例住院患儿的临床资料。结果根据晕厥的定义及临床检查结果,排除其中非晕厥性疾病20例,108例患儿确诊为晕厥;其中自主神经介导性晕厥(NMS)70例(64.81%),心源性晕厥8例（7.41%),不明原因晕厥24例（22.22%)。发作前有诱因者85例（78.71%),有晕厥家族史者21例（19.44%),有猝死家族史者1例（0.93%)。NMS及心源性晕厥常有反复发作的特征。NMS中,血管迷走性晕厥（VVS）前兆常为四肢无力、面色苍白、眼前发黑、听力下降、恶心、胸闷等表现;体位性心动过速综合征（POTS）表现为心慌、胸闷、乏力等;直立性低血压（OH）表现为眼前发黑,面色苍白、听力下降等;心源性晕厥表现为心前区不适、疼痛、胸闷。结论VVS是儿童晕厥最常见原因之一,各类型晕厥的发作常有诱因及前兆,有反复发作的特征。     

儿童自主神经介导性晕厥与心因性假性晕厥的临床特征及鉴别

目的分析并比较儿童自主神经介导性晕厥(NMS)与心因性假性晕厥(PPS)的临床特征。方法回顾分析以晕厥为主诉,并排除心源性晕厥、境遇性晕厥、药源性晕厥及神经源性及代谢性疾病的短暂意识丧失患儿的临床特征,对比NMS与PPS临床特征的异同。结果入选106例晕厥患儿中,NMS85例(80.2%)、PPS13例(12.3%)、诊断不明晕厥8例(7.5%)。PPS多以近期精神刺激因素为诱因,晕厥次数频繁,直立不耐受评分高,先兆较少,发作持续时间长。NMS常以持久站立、运动、体位改变为主要诱因,先兆多以头晕、视物模糊、消化道症状为主,持续时间短5 min。结论 NMS是儿童晕厥最常见的原因,PPS是非晕厥型短暂意识丧失的重要原因,两者在临床特征上有相似点,应在诊断中予以鉴别。     

儿童直立性高血压的临床特征及其分型研究

目的探讨儿童直立性高血压(OHT)的发病情况及临床特征。方法收集2009—2015年在北京大学第一医院儿科住院的以晕厥、头晕等为主要表现的1348例儿童的病例资料,检出OHT患儿24例,分析其人口学特征、各种临床表现的发生率、诱发因素及卧立位血压变化情况。结果 (1)OHT男14例,女10例,男女患病率≤12岁者与12岁者所占比例差异均无统计学意义;(2)直立性高收缩压(s OHT)与直立性高舒张压(d OHT)≤12岁者与12岁者所占比例、男女所占比例差异均无统计学意义;(3)就诊时最常见的症状为晕厥、头晕、胸闷等,其中发生晕厥和胸闷症状者以d OHT患儿居多,s OHT与d OHT患儿出现头晕症状者比例相当;多在患儿突然体位变化(41.7%)或持久站立(37.5%)、持久坐位(37.5%)时发生;其中持久站立和持久坐位是诱发s OHT的主要原因,而突然体位变化是d OHT的主要诱因。结论 OHT患儿发病与分型均不存在年龄及性别差异,以晕厥、头晕和胸闷为主要临床症状;持久站立和持久坐位是诱发s OHT的主要原因,而突然体位变化是d OHT的主要诱因。     

血管迷走性晕厥的发病机制

血管迷走性晕厥(vasovagal syncope,VVS)是最常见的晕厥类型,据统计在院外发生晕厥事件中,VVS发生率达40%。诱因包括持久站立、精神紧张、疲劳、疼痛、饥饿、处于闷热环境中等,发作时表现为头晕、恶心、黑朦、冷汗、面色苍白、瞳孔散大、全身乏力、血压下降、心动过缓,难以维持自主体位,意识丧失,继而跌倒,躺平或平卧位后症状迅速缓解。预后大多良好,很少威胁生命,但如果发作频繁,生活质量受到影响,甚至造成严重伤害,如跌伤、头颅外伤等。VVS发病机制较为复杂,因此现将其研究进展简要介绍如下。一、血流动力学异常实验研究表明,轻度失血…     

儿童直立不耐受的诊断及治疗

一、概述直立不耐受(orthostatic intole_rance,OI)系多因直立而发生的一系列临床症状,平卧后症状缓解。对于“直立不耐受”这个术语在临床中有时会有一些误解,有人认为只有在身体直立时才会出现心率及血压的异常,因而导致患者出现症状。事实上,这些异常表现通常出现在直立体位,但也可发生在其他体位[1]。OI通常分为急性OI和慢性OI。急性OI表现为晕厥,即因脑血流灌注减少引起的短暂意识及肌张力的丧失,不能维持站立体位。慢性OI的症状主要有:经常发生的头晕、疲乏、视物模糊、眼前发黑、心悸、胸闷等。大部分患儿同时伴有头痛、发抖、…     

儿童不明原因晕厥诱因分析

目的：分析儿童不明原因晕厥的常见诱因。方法：详细询问2006年1月至2011年10月就诊的434例不明原因晕厥儿童（3.0～17.9岁,男192例,女242例）的晕厥病史及诱因,分析诱因在不同年龄、性别、晕厥频次、直立倾斜试验（HUTT）结果之间的差异。结果：（1）偶发晕厥常见诱因依次为持久站立（30%）、运动（13%）、体位改变（9%）、坐位（7%）、玩耍（6%）等。①性别比较：持久站立引起的偶发晕厥女性较男性多见(P<0.01）;排尿晕厥主要见于男性;闷热环境主要诱发女性晕厥。②年龄比较：体位改变诱发偶发晕厥在≥12岁组较<12岁组常见（P<0.05）,其余诱因在不同年龄间差异未见统计学意义。③不同HUTT结果比较：体位改变诱发偶发晕厥多见于HUTT阴性组（P<0.05）。（2）所有偶发晕厥诱因均可诱发反复晕厥,56.2%的反复晕厥为同一诱因所致。结论：儿童不明原因晕厥常见诱因为持久站立、运动、体位改变等,临床上避免诱因有助于预防晕厥发作。     

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.