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目的探讨心脏疾患及合并充血性心力衰竭(CHF)患儿心衰时,血清甲状腺激素(TH)水平的变化规律及临床意义。方法对2003-09—2006-09山西省阳泉市第一人民医院收治的116例心脏疾病患儿分为2组,心脏病合并CHF组82例,心脏病未合并CHF组34例,以28例健康体检儿童为对照组,均做如下检测及分析:(1)检测CHF患儿血清TH水平,包括三碘甲状腺原氨酸(T3)、游离T3(FT3)、甲状腺素(T4)、游离T4(FT4)、反T3(rT3)及促甲状腺激素(TSH),三组间进行比较。(2)以血清TH水平与心功能进行相关分析。(3)动态观察CHF患儿的血清TH水平,探索其变化规律。结果(1)CHF组与N-CHF组及正常对照组比,血清T3、FT3及FT4均显著降低(P<0.01),rT3显著升高(P<0.01)。(2)随心功能的下降,T3、FT3、T4、FT4渐降低,rT3渐升高。TSH与心功能变化无相关性。(3)CHF组经治疗后血清TH多恢复(P<0.01),顽固性心衰组则无恢复(P>0.05)。结论(1)小儿CHF伴有血清TH的改变,以T3、FT3及FT4的降低,rT3的升高为主,其改变程度与心功能状态具有相关性。(2)随着心功能的改善,TH的改变多渐恢复,顽固性心衰患儿则无恢复,提示TH水平持续不恢复者预后较差。     

慢性肾脏病患儿血清甲状腺素水平变化的意义

目的观察慢性肾脏病（CKD）患儿血清甲状腺激素水平变化及其临床意义。方法对78例CKD患儿进行分期（入院治疗前，患儿达到完全缓解后，住院治疗8周确定患儿未缓解时口服小剂量L－甲状腺素片4～6周），于清晨采取其空腹静脉血2～3mL，血标本均采用放射免疫分析法检测其总甲状腺素（T4）、三碘甲状腺原氨酸（T3）、促甲状腺激素（TSH）等指标，观察其变化。采用SPSS11．0软件进行统计学分析。结果CKD患儿治疗前T3明显低于健康对照组（P〈0．01），经治疗达到完全缓解时，其T3水平与治疗前及未缓解前比较，均明显升高（Pa〈0．01）；病情未缓解组T1明显低于健康对照组及缓解组（Pa〈0．01），且出现L下降，明显低于健康对照组及缓解组（Pa〈0．01）；各组TSH无明显改变（Pa〉0．05）；治疗无缓解患儿加用小剂量L-甲状腺素片后，T3、T4逐渐升高，与治疗前比较有显著性差异（Pa〈0．05），并有部分患儿病情缓解。结论动态检测甲状腺激素水平可作为观察CKD患儿病情、判断疗效及评估预后的有效指标之一。     

充血性心力衰竭患儿血清白细胞介素-18和淋巴细胞CD54变化

目的探讨IL-18和CDs4在小儿充血性心力衰竭（CFIF）发病中的作用，及其对z],JLCFIF的诊断价值。方法CFIF患儿52例，心功能按修订的Ross和Reithman评分系统。心功能Ⅱ级（3～6分）18例、Ⅲ级（7～9分）17例，Ⅳ级（10～12分）17例。原发疾病为扩张性心肌病、心内膜弹力纤维增生症及先天性心脏病等。对照组15例为上呼吸道感染患儿。血清IL-18水平用ELISA法进行测定，采用流式细胞术检测外周血淋巴细胞CDs4表达。结果CFIF患儿血清IL-18及CDs4均显著增高（P均〈0．001），且随CFIF加重渐增加，治疗后两者均较治疗前明显降低（P均〈0．05）；且两者在心肌病组、先天性心脏病组及其他病组3组间比较差异无显著性（P均〉0．05），但均显著高于对照组（P均〈0．01）。结论IL-18和CDs4可能参与小儿CFIF发生发展。可以作为评价CHF严重程度的生物化学标记；CFIF作为一种临床综合征，无论最初病因是否相同，免疫反应可能都具有某些共同特征。     

慢性充血性心力衰竭患儿血浆儿茶酚胺、心钠素水平的意义

目的探讨慢性充血性心力衰竭（CHF）患儿血浆儿茶酚胺（CA）[包括肾上腺素（E）、去甲肾上腺素（NE）]、心钠素（ANP）水平变化及其与心脏左室功能的关系。方法选择CHF患儿及正常对照组各35例，测定其血浆NE、E及ANP水平，检测左室射血分数（LVEF）。结果1．CHF组治疗前血浆NE、E、ANP水平显著高于正常对照组，二组比较差异有显著性（Pa〈0．01）。2．CHF组治疗前显著高于抗心力衰竭综合治疗心力衰竭好转后血浆NE、E、ANP水平，二组比较差异有显著性（Pa〈0．01）。3．CHF组治疗后血浆NE、E、ANP水平显著高于正常对照组，二组比较差异有统计学意义（Pa〈0．05）。4．CHF组心功能越差，血浆NE、E、ANP升高越明显，差异有显著性（P〈0．01）。5．以35例CHF患儿血浆NE、E、ANP为自变量，LVEF为应变量作直线相关分析，显示CHF组血浆NE、E、ANP与LVEF呈显著负相关。结论CA、ANP水平与CHF程度密切相关，心功能下降可能与CA、ANP水平过度生成有关，其可作为评价CHF的指标之一。     

肾病综合征血清游离甲状腺素与肿瘤坏死因子、清蛋白及尿蛋白的关系

目的探讨肾病综合征(NS)患儿血清游离甲状腺素(FT3、FT4)变化及其与肿瘤坏死因子(TNF)、清蛋白(Alb)及尿蛋白的关系。方法检测活动期NS 60例患儿血清FT3、FT4、TNF、Alb及尿蛋白,25例正常儿童作为对照组。结果肾病组血清FT3、FT4降低,与正常对照组比较有显著性差异(P<0 01),NS组血FT3、FT4与Alb呈正相关,与TNF,尿蛋白呈负相关。结论NS患儿活动期存在甲状腺功能低下,为小剂量甲状腺素(TH)佐治NS提供理论依据。     

脂肪酸结合蛋白在小儿急性心力衰竭诊断中的应用研究

目的 通过测定急性充血性心力衰竭（CHF）患儿血浆脂肪酸结合蛋白（FABP）水平的变化，进一步探讨其在临床应用中的价值。方法 选择31例健康儿童作为对照组，CHF住院患儿48例作为观察组，其中肺炎合并CHF20例，先天性心脏病合并CHF18例，心肌炎合并CHF10例。FABP测定方法采用酶联免疫吸附法，比较CHF前后FABP水平；根据NYHA小儿CHF分级，对不同心功能级别患儿FABP水平间进行比较。结果 患儿CHF期FABP水平显著高于恢复期（P〈0．01）；同时CHF患儿心功能分级不同，FABP浓度明显不同，CHF程度越重，FABP浓度愈高，差异有非常显著性（P〈0．01）。结论 CHF时FABP浓度明显增高，FABP浓度与心功能密切相关；CHF程度越重，血FABP浓度越高。FABP可作为评价心功能程度及预后的指标。     

苯巴比妥、苯妥英、卡马西平对癫痫患儿甲状腺激素的影响

观察常用抗癫痫药物对癫痫患儿血清甲状腺激素的影响。对无甲状腺功能减退临床表现的癫痫患儿(各组均20例)共80例,应用RIA法测定血清TT4、TT3、FT4、FT3、rT3、TSH浓度。结果未经治疗癫痫患儿所有激素水平与正常对照组比较无显著差异,苯巴比妥组FT4值低于正常对照组(P<0.01),卡马西平组TT4、FT4值也明显降低(P<0.01),苯妥英组TT4、FT4、FT3值均显著降低(P<0.01),所有各组rT3、TSH无改变。资料表明,抗癫痫药物对甲状腺激素影响强度依次为苯妥英钠、卡马西平、苯巴比妥。     

先天性甲状腺功能减退症新生儿心电图改变的意义

目的评价先天性甲状腺功能减退症(CH)新生儿的心电图(ECG)改变,及与血清甲状腺激素(TH)水平的相关性,探讨TH减少对新生儿心脏电生理活动的影响。方法对50例CH新生儿(日龄17～28d)和35例健康新生儿(健康对照组)进行常规十二导联ECG检查,分别检测心率(HR)、PR间期(PR)、QT间期(QT)、QRS波电轴(QRSa)、QRS波时限(QRS)、校正QT间期(QTC)等,同时用化学发光法测定血清游离三碘甲状腺原氨酸(FT3)、游离甲状腺素(FT4)、总三碘甲状腺原氨酸(TT3)、总甲状腺素(TT4)和促甲状腺素(FSH)水平,对心电图指标和血TH水平行相关性分析。结果与健康对照组相比,CH组血清FT3、FT4、TT3、TT4水平显著降低,TSH水平显著升高(Pa<0.001);CH组HR显著低于健康对照组,PR及QT较健康对照组显著延长(Pa<0.05),但二组QRSa、QRS及QTC差异均无统计学意义(Pa>0.05)。HR与FT3、FT4呈显著正相关,与TSH呈显著负相关(Pa<0.05);PR间期与FT3、FT4、TT4呈显著负相关,与TSH呈显著正相关(Pa<0.05);但QT、QRS、QRSa及QTC与血TH水平均无明显相关(Pa>0.05)。结论CH可对新生儿窦房结起搏产生显著影响,引起心脏自律性改变,而心肌动作电位、房室传导等电生理活动则尚未受影响。     

支气管哮喘患儿诱导痰液中白细胞介素-4及γ-干扰素水平的研究

目的研究支气管哮喘患儿诱导痰液中白细胞介素（IL）-4及γ-干扰素（IFN-γ）水平变化，探讨其在哮喘发病机制中的作用。方法2004年2月～2006年6月儿科住院哮喘患儿36例，用ELISA法测定诱导痰中急性期及缓解期IL-4及IFN-γ水平。结果哮喘患儿诱导痰IL-4水平急性期明显高于缓解期和正常对照组（P〈0．01）；IFN-γ水平急性期低于缓解期及对照组（P〈0．05）；IL-4／IFN-γ比值急性期高于缓解期及对照组（P〈0．05）。急性期患儿诱导痰IL-4水平重症组明显高于轻症组（P〈0．01），IFN-γ水平重症组明显低于轻症组（P〈0．05），IL-4／IFN-γ比值重症组高于轻症组（P〈0．05）。结论哮喘患儿存在Thl／Th2功能紊乱，IL-4及IFN-γ参与哮喘患儿的免疫状态改变，在哮喘发病机制中发挥一定作用。     

小儿慢性病贫血红系祖细胞与肿瘤坏死因子α、白介素6的关系研究

目的 研究检测肿瘤坏死因子α（TNF-α）、IL-6（白介素-6）两种慢性病发生有关的细胞因子水平，以及红系造血祖细胞集落培养生长探讨它们在慢性病贫血（ACD）发病中的作用。方法 检测病例组为慢性病贫血组（ACD组）患儿，对照组为慢性病无贫血组（NA组）和缺铁性贫血组（IDA组）患儿的血清肿瘤坏死因子α（TNFα）、白介素-6（IL-6）水平，以及骨髓的红系祖细胞水平（BFU-E、CFU-E）。结果 三组BFU-E有差异（F=3．56，P〈0．05；三组CFU-E有显著差异（F=8．87，P〈0．01）。ACD组与NA组相比，血清TNF-α前者高于后者（t=2．25，P〈0．05），两组血清IL-6无差异（t=1．91，P〉0．05）。在20例行骨髓培养的慢性病患儿中，血清TNFα与骨髓BFU-E之间无显著相关性（r=-0．37，P〉0．05）；血清TNF-α与CFU-E之间有负相关（r=-0．57，P〈0．05）；IL-6与BFU-E、CFU-E无相关性。结论 ACD患儿骨髓红系祖细胞BFU-E、CFU-E生成受抑制；血清TNF-α、IL-6的升高参与贫血发生；TNFα可能抑制慢性病患儿骨髓CFU-E形成而导致贫血。     

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.