先天性心脏病肺高压患儿血浆肾上腺髓质素浓度的测定及其临床意义:附33例报告

用特异性放射免疫非平衡法测定左向右分流型先天性心脏病 (CHD)患儿血浆肾上腺髓质素 (AM )水平 ,并观察心脏手术后AM的动态变化。结果显示 :CHD肺高压组AM明显高于无肺高压组及正常对照组 (P<0.05 ,P<0.01)。且AM随着肺高压病情加重而升高 ;中、重度肺高压组AM高于轻度肺高压组 ,差异有显著性 (P<0.01)。肺高压组术后AM明显下降 ,与术前比较差异有显著性 (P<0.05 ,P<0.01)。CHD组患儿AM与肺动脉收缩压 (PASP)呈显著正相关 (r=0.77,P<0.05)。本资料表明 ,AM参与了左向右分流型CHD合并肺高压的病理生理过程 ,AM的升高是机体的一种防御性反应 ,对维持肺循环稳定 ,减缓肺高压的发展有积极作用     

先天性心脏病并心力衰竭患儿血浆肾上腺髓质素和内皮素水平变化的意义

目的探讨左向右分流先天性心脏病(CHD)患儿并充血性心力衰竭(CHF)血浆肾上腺髓质素(ADM)和内皮素(ET-1)变化的意义。方法左向右分流CHD并CHF患儿18例。正常对照组20例,检测CHD患儿心力衰竭期和症状、心衰体征消失的恢复期及对照组儿童血浆ADM和ET-1。结果CHF急性期ADM、ET-1明显高于恢复期和正常对照,恢复期ADM或ET-1仍高于正常对照组(P<0.01或0.02)。结论ADM和ET-1均参与了左向右分流CHD并CHF的病理生理过程,观察其血浆水平变化,可能对判断心衰患者病情有一定意义。     

先天性心脏病并肺动脉高压患儿血清一氧化氮和血浆内皮素变化的意义

目的　探讨血清一氧化氮 (NO)和血浆内皮素 1(ET 1)对先天性心脏病 (CHD)患儿肺动脉高压 (PH)形成的影响。方法　选择健康儿童 3 0例为对照组 (A组 ) ;3 0例肺动脉压正常的CHD患儿为B组 ;3 0例CHD并PH患儿为C组。采用硝酸还原法对其血清NO 2 /NO 3 、放免法对其血浆ET 1浓度进行测定。结果　B组血清NO 2 /NO 3 浓度较A组明显增高 ;C组血清NO 2 /NO 3 浓度较A组低 ;无论是否并PH ,A、B两组与对照组相比 ,其血浆ET 1浓度均增高。结论　血清NO 2 /NO 3 浓度和血浆ET 1浓度及其间平衡关系共同影响PH的形成及其程度     

血浆内皮素测定在儿科临床的初步应用

采用放免法测定血浆内皮素(ET)变化,结果示血浆ET水平在不同性别、年龄的43例健康儿童无显著性差异(P均>0.05);60例急性肺炎、39例左向右分流型先心、48例哮喘、87例肾病综合征患儿均明显高于对照组(P均<0.01);伴有心衰的重症肺炎患儿明显高于一般肺炎患儿,伴有肺高压的先心患儿明显高于不伴肺高压的先心患儿(P均<0.01)。提示这些疾病患儿均存在血管内皮细胞损伤;血浆ET的增高可能与左向右分流型先心或肺炎患儿的肺高压发生发展有关,并可能在哮喘的发病过程中起重要作用。     

先天性心脏病肺动脉高压患儿肺组织内皮素-1与胶原合成关系的研究

目的研究左向右分流先心病肺动脉高压(肺高压)患儿肺组织内皮素-1(ET-1)与肺组织Ⅰ、Ⅲ型胶原合成的关系,探讨左向右分流先心病患儿肺高压形成的机理。方法选择左向右分流先心病患儿18例,根据肺动脉收缩压分为肺高压组(n=10),非肺高压组(n=8)。所有患儿在外科手术体外循环开始前,取右肺中叶组织(1cm×1cm×1cm),放射免疫法测定患儿血浆及肺组织ET-1含量;RT-PCR法检测肺组织Ⅰ、Ⅲ型胶原mRNA表达。结果先心病肺高压患儿血浆及肺组织ET-1含量明显高于肺动脉压正常者(P<0.01)。肺高压患儿肺组织Ⅰ、Ⅲ型前胶原mRNA的表达与肺动脉压正常者相比明显增加(P<0.05)。结论ET-1可能通过刺激肺动脉平滑肌细胞胶原合成增加参与肺高压的形成。     

内皮素、一氧化氮在毛细支气管炎和哮喘患儿中的变化

目的　 探讨血内皮素 (ET)和一氧化氮 (NO)在毛细支气管炎 (毛支 )和婴幼儿哮喘 (哮喘 )中的变化及意义。 方法 　用放射免疫分析法和分光光度比色法分别检测毛支、哮喘患儿急性期和缓解期血ET和NO ,并与正常儿童进行比较。 结果 　血浆ET在毛支组和哮喘组 ,急性期均明显高于正常组 (P均 <0 0 1) ,缓解期均下降 ,与正常组比较无显著差别 (P均 >0 0 5 ) ;两组急性期比较无差别 (P >0 0 5 )。与血浆ET一样 ,血清NO在毛支组和哮喘组 ,急性期均显著高于正常组 (P <0 0 1和P <0 0 5 ) ;缓解期则下降 ,与对照组比无显著差别 ,(P均 >0 0 5 ) ;两组急性期相比 ,差异无显著性 (P >0 0 5 )。两组急性期血浆ET与血清NO呈明显正相关 (P <0 0 5 )。 结论 　在毛支和哮喘中 ,血ET和NO均升高 ,二者关系密切 ,均可能参与毛支及哮喘的发病     

先天性心脏病肺动脉高压患儿左室舒张功能与血浆脑利钠水平的相关性研究

目的：探讨先天性心脏病 (CHD)合并肺动脉高压 (PAH) 患儿脑利钠肽（BNP）水平及与左室舒张功能的关系。方法：对95例CHD继发有PAH的患儿和42例无PHA的CHD患儿（对照组）的多普勒超声心动图资料与其血浆BNP水平进行对比分析。结果：与对照组相比, PAH组的左室舒张末内径（LVDd ）、右室舒张末内径（RVDd） 和肺动脉内径（PAd）明显增大(P<0.05),三尖瓣返流（VTR）速度增快及肺动脉收缩压（PASP）升高(P<0.05)。与对照组比较,PASP组患儿二尖瓣口多普勒血流频谱A峰流速（AV）、A峰流速积分（AVI）和E峰流速积分（EVI）及AV/EV和AVI/EVI比均逐渐明显增大(P<0.01);左室等容舒张时间明显延长(LIVRT)(P<0.05)。血浆BNP水平随着PASP增高而升高,与对照组相比差异有显著性（P<0.01）。PAH组先心病患儿其肺动脉压与二尖瓣口血流频谱AV/EV比值呈正相关（P<0.01）,二尖瓣口血流参数与血浆BNP水平亦呈正相关（P<0.01）。结论：CHD合并PAH患儿左室舒张功能与血浆BNP水平呈正相关;BNP在PAH引起左室舒张功能障碍的发生发展过程中发挥了重要作用。［中国当代儿科杂志,2010,12（1）：13-16］     

左向右分流型先天性心脏病患儿心脏结构和功能改变与骨龄指数的相关性

目的探讨左向右分流型先天性心脏病(CHD),包括房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)患儿的骨龄变化以及心脏结构和功能改变与骨龄指数(BAI)的相关性。方法收集确诊左向右分流型CHD共130例,其中ASD 52例、VSD 46例、PDA 32例,超声检测各项心脏结构和功能指标,摄左手及腕关节正位片,应用最新《中国儿童骨龄评分法》图谱进行骨龄评定并计算BAI,对比分析各组骨龄以及CHD各项心脏结构和功能指标与BAI的直线相关性。结果 ASD、VSD与PDA患儿BAI的差异有统计学意义(P0.05);PDA组的BAI分别高于ASD组和VSD组,差异均有统计学意义(P均0.05)。无PAH、轻度PAH以及中重度PAH三组的BAI分别为(0.84±0.09)、(0.75±0.07)和(0.65±0.08),差异有统计学意义(F=27.77,P=0.000);小缺损组的BAI为(0.82±0.09),高于中大缺损组的(0.73±0.10),差异有统计学意义(t=3.54,P=0.002)。ASD、VSD患儿缺损大小(DS)与BAI均呈负相关(r=-0.48、-0.54,P均0.05);ASD、VSD、PDA患儿肺动脉收缩压(PASP)以及肺循环血流量/体循环血流量比值(Qp/Qs)均与BAI呈负相关(r=-0.64~-0.38,P均0.05)。结论左向右分流型CHD患儿的骨龄及BAI均显著落后于正常同龄儿童;左向右分流型CHD患儿骨龄落后程度与PASP、DS及分流量大小相关。     

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目的 探讨先天性心脏病(CHD)患儿肺动脉高压(PH)形成的影响因素.方法 研究对象均为2003-06-2005-02于北京大学深圳医院收集病例,以健康者30名为对照组(A组),以肺动脉压正常和并发PH的左向右分流的CHD患儿各30例为观察组(B组、C组).以高效液相色谱法、硝酸还原法及放射免疫法测定其血清精氨酸(L-Arg)、一氧化氮(NO)、血浆内皮素(ET-1)的浓度.结果 血清L-Arg浓度对照组(A组)为(72.00±18.01)nmol/mL,肺动脉压正常的患儿(B组)为(30.74±8.97)nmol/mL,伴PH的患儿(C组)为(23.51±12.37)nmol/mL.血清NO浓度A组为(76.10±17.10)nmol/mL,B组(90.55±26.57)nmol/mL,C组(60.05±17.60)nmol/mL.血浆ET-1浓度A组(50.82±7.58)pg/mL,B组(64.90±16.28)pg/mL,C组(69.64±10.66)pg/mL.结论 血清NO浓度和血浆ET-1浓度及其之间的平衡关系共同影响PH的形成及其程度.血浆ET-1浓度的升高是肺动脉压升高的直接因素,血清NO浓度的降低是间接因素,而血清NO浓度降低是由血清L-Arg浓度的降低引起.     

先天性心脏病患儿血清儿茶酚胺 醛固酮测定临床意义研究

目的研究小儿左向右分流型先天性心脏病(CHD)血清儿茶酚胺(CA)[肾上腺素(E)、去甲肾上腺素(NE)、多巴胺(DA)]和醛固酮(ALD)的变化,分析其与心功能、容量负荷、肺动脉压力及心肌重塑的相关性。方法 2007年10月至2008年10月天津市儿童医院心脏科住院经彩色多普勒超声心动图检查确诊为左向右分流性CHD患儿53例,对照组20例,检测血清CA、ALD。采用实时三维超声心动图检测心脏房室腔径及心功能指标。结果 (1)CHD组血清NE、ALD高于对照组(P0.01)。(2)心衰组、大分流组、肺动脉高压组血清CA、ALD高于无心衰组、小分流组、无肺动脉高压组(P0.01)。(3)心衰组患儿左室舒张末期内径指数、左室舒张末期容积指数、左室质量指数均高于无心衰组(P0.01)。(4)血清CA与左房内径指数、左室舒张末期内径指数、左室舒张末期容积指数呈正相关(P0.01)。结论血清CA、ALD可作为早期诊断心力衰竭及肺动脉高压的血清学指标之一,CA与心肌重塑密切相关。     

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.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.