儿童急性白血病继发丙型病毒性肝炎

目的 探讨儿童急性白血病继发输血后丙型病毒性肝炎的发生、发展及转归。方法 用ＲＴ－ＰＣＲ法检测血清ＨＣＶ－ＲＮＡ,并用ＥＬＩＳＡ方法测ＨＣＶ核心区多肽抗体（ＨＣＶ－ＣＰ）。结果 １０７例儿童白血病中ＨＣＶ感染率达７０％（７５／１０７）;ＨＣＶ－ＣＰ检测阳性率明显低于ＨＣＶ－ＲＮＡ（４０％ｖｓ７４％）;ＨＣＶ感染者化疗期间曾有转氨酶（ＡＬＴ）明显升高,但停止化疗后５０例中仅１例ＡＬＴ持续偏高,余均恢     

三种不同类型抗心磷脂抗体在小儿病毒性心肌炎中的价值

采用酶联免疫吸附试验（ＥＬＩＳＡ）法测定６２例ＣｏｘＢ组病毒引起的病毒性心肌炎（ＶＭ）患儿和４０名健康儿童的血清抗心磷脂抗体（ＡＣＡ）。结果：ＶＭ组ＡＣＡ－ＩｇＧ、ＡＣＡ－ＩｇＡ、ＡＣＡ－ＩｇＭ阳性率（分别为４５．２％、３０．６％、３３．９％）均显著高于正常对照组（分别为２．５％、０、０）;ＶＭ发病早期ＡＣＡ－ｉｇＡ、ＡＣＡ－ＩｇＭ阳性显著多于ＡＣＡ－ｉｇＧ阳性恩赐 ＡＣＡ－ＩｇＧ阳性者的ＣＫ－Ｍ     

逆转录—多聚酶链反应检测柯萨奇B组病毒

研究目的用分子生物学方法对柯萨奇Ｂ组病毒（ＣＢＶ）进行检测并探讨与临床症病的关系。研究方法对我院２９８例疑似ＣＢＶ感染的患儿，采用特异性引物进行逆转录－多聚酶链反应（ＲＴ－ＰＣＲ）检测ＣＢＶ。结果检出ＣＢＶ阳性１２７人，阳性率４２．６％。感染与年龄有关，夏秋季多见。８０例健康儿童中，阳性５人，占６．２５％。２０例ＲＴ－ＰＣＲ检测阳性标本用ＥＬＩＳＡ法检测，１７例阳性。结论ＲＴ－ＰＣＲ可快速、敏感、准确地检出标本中的ＣＢＶ。此病毒可侵犯多种器官，引起各种临床症病，有较强的传染性。     

柯萨奇B组病毒感染与小儿哮喘发作关系的探讨

目的　探讨柯萨奇 Ｂ 组病毒（ Ｃ Ｖ Ｂ） 感染与哮喘发作的关系。方法　观察急性发作期哮喘患儿１０２ 例、上呼吸道感染１８２ 例、非感染性疾病患儿７１ 例、健康儿童３０ 例。采用 Ｅ Ｌ Ｉ Ｓ Ａ 法测定静脉血 Ｃ Ｖ Ｂ 抗原（ Ｃ Ｖ Ｂ－ Ａｇ）和 Ｉｇ Ｍ 抗体（ Ｃ Ｖ Ｂ－ Ｉｇ Ｍ） 、免疫组化法测定 Ｔ 细胞亚群和［３ Ｈ］ － Ｔｄ Ｒ 标记法测定 Ｎ Ｋ 细胞活性。结果　①哮喘患儿 Ｃ Ｖ Ｂ 感染率为５７８ ％ （５９／１０２） ;②哮喘患儿 Ｃ Ｄ８ 显著升高; Ｃ Ｄ３ 、 Ｃ Ｄ４／ Ｃ Ｄ８ 及 Ｎ Ｋ 细胞活性均明显下降（ Ｐ ＜００１） ;而 Ｃ Ｖ Ｂ 阳性哮喘患儿 Ｎ Ｋ 细胞活性又低于非 Ｃ Ｖ Ｂ 阳性哮喘儿（ Ｐ ＜ ００５） , Ｃ Ｄ８ 明显高于非 Ｃ Ｖ Ｂ 阳性哮喘儿（ Ｐ ＜ ００１） 。结论　 Ｃ Ｖ Ｂ 感染与哮喘发作有密切关系。     

逆转录多聚酶链反应对肠道病毒性脑膜炎、脑炎诊断价值探讨

目的探讨中枢神经系统感染的病原体及其检测方法。方法应用逆转录多聚酶链反应（ＲＴ－ＰＣＲ）检测６４例无菌性脑膜炎及２７例无菌性脑炎患儿脑脊液（ＣＳＦ）中肠道病毒（ＥＶ）ＲＮＡ。结果急性期ＣＳＦ中ＥＶＲＮＡ阳性检出率为：无菌性脑膜炎６０９％,脑炎４８１％;阳性患儿恢复期ＣＳＦ仅个别阳性;对照组全部阴性。起病５天以内阳性率明显高于５天以后（Ｐ＜００５）;５～１０月份较１１～４月份发病率高（Ｐ＜００５）。２５例病毒分离阳性者中２４例ＲＴ－ＰＣＲ阳性,５３例病毒分离阴性者中１９例ＲＴ－ＰＣＲ阳性,ＲＴ－ＰＣＲ敏感性显著高于病毒分离的敏感性（Ｐ＜００１）。结论ＥＶ是引起中枢神经系统感染的重要病原体,ＲＴ－ＰＣＲ技术是诊断ＥＶ感染的有效方法。     

CMV感染婴儿肝炎综合征患儿的肾脏损害

为了解ＣＭＶ感染所致婴儿肝炎综合征患儿的肾脏损害,用ＥＬＩＳＡ法检测了ＣＭＶ感染、非ＣＭＶ感染婴儿 肝炎综合征患儿和正常婴儿尿视黄醇结合蛋白（ＲＢＰ）,以及β２微球蛋白（β２ＭＧ）、Ｎ－乙酰－β－Ｄ氨基葡萄糖苷酶（ＮＡＧ）、Ｃｃｒ血β２ＭＧ等。结果表明,尿ＲＢＰ和尿β２ＭＧ均呈ＣＭＶ感染患儿组〉非ＣＭＶ感染患儿组〈对照组,ＣＭＶ感染组７１．４％、非ＣＭＶ感染组４４．４％尿ＲＢＰ超过对照组９５％参考     

抗庚型肝炎病毒抗体与庚型肝炎病毒RNA检测的对比研究

目的：正确认识抗庚型肝炎病毒（ＨＧＶ）抗体和ＨＧＶ－ＲＮＡ的临床诊断价值。方法：用ＥＬＩＳＡ和逆转录ＰＣＲ方法同时检测９０例儿童抗ＨＧＶ抗体和ＨＧＶ－ＲＮＡ。结果：抗ＨＧＶ阳性率为１５６％（１４／９０），其中慢性丙型肝炎中（丙肝）占１０／２８，慢性乙型肝炎中（乙肝）占４／１２；ＨＧＶ－ＲＮＡ阳性率占２４４％（２２／９０），其中丙肝中占１２／２８，乙肝中占４／１２，非甲－戊肝炎中占２／４，急性黄疸型肝炎中占２／４，肾炎或肾病中占２／２０。２２例健康儿童均阴性。抗ＨＧＶ阳性者中ＨＧＶ－ＲＮＡ阳性率为１４３％（２／１４），丙氨酸转氨酶（ＡＬＴ）异常率为５７１％；ＨＧＶ－ＲＮＡ阳性者中的抗ＨＧＶ阳性率为９１％（２／２２），ＡＬＴ异常率为４５５％，两组比较Ｐ＞００５。结论：要想全面了解ＨＧＶ感染率，此两种方法都应采用；抗ＨＧＶ阳性可能预示病毒复制停止；病毒血症与肝功能损害的临床联系具有多样性，需动态观察。     

对《肠道病毒感染与儿童1型糖尿病关系的研究》一文的质疑

读了《肠道病毒感染与儿童 1型糖尿病关系的研究》一文 (刊于中华儿科杂志 2 0 0 0年 38卷 2期 92页 )后 ,对该文摘要中提出的“儿童 1型糖尿病发病与肠道病毒 (ＥＶ)感染 ,特别是柯萨奇Ｂ组病毒感染有关”的结论 ,认为是不能接受的。该文结论的取得主要基于下列 2项实验结果 ,即 :(1)糖尿病组 (下称病组 ) 4 5例患儿中ＥＶＲＮＡ阳性 17例 (39%) ,对照组 5 1例儿童中阳性 3名 (6 %) ;(2 )病组 35例ＣＶＢ ＩｇＭ阳性14例 (4 0 %) ,对照组 40名中阳性 2名 (5 %)。让我们来看看该文的上述 2项实验方法及其临床意义。 (1)ＥＶＲＮＡ检测 ,作者…     

小儿庚型肝炎的探讨

为了探讨一种新型肝炎病毒，即庚型肝炎病毒（ｈｅｐａｔｉｔｉｓＧｖｉｒｕｓ，ＨＧＶ）在小儿中的感染特点，检测了３６例肝炎患儿及１６例健康体检儿童血清中ＨＧＶ－ＲＮＡ（套式逆转录ＰＣＲ法）。结果表明，３６例肝炎中有１１例ＨＧＶ感染者，其中６例合并慢性ＨＣＶ感染（３例接受过干扰素治疗），２例合并慢性ＨＢＶ感染，２例为慢性非Ａ－Ｅ肝炎，１例合并ＨＢＶ＋ＨＡＶ感染。１６例健康儿童均阴性。ＨＧＶ感染率在血制品输入者１０例中７例阳性，在未输入者２２例中３例阳性（两者比较，Ｐ＜０．０１），在血制品使用情况不明者２０例中１例阳性。提示输入血制品是小儿ＨＧＶ感染的主要途径，但不排除还有其他途径，感染者主要为慢性肝炎患儿，干扰素的疗效有待进一步研究     

聚合酶链反应诊断病毒性心肌炎

用聚合酶链反应（ＰＣＲ）检测３０例小儿病毒性心肌炎血清肠道病毒（ＥＶ）ＲＮＡ，６例阳性。检出率２０％，并对阳性病例作临床分析。用ＥＬＩＳＡ法混合反应对照检测柯萨奇Ｂ组病毒（ＣＢＶ）ＩｇＭ、ＩｇＧ，阳性率分别为７７％和３０％。认为ＰＣＲ对ＥＶ感染早期检出率高，对病原学诊断有意义。     

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.     

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.     

MAINTENANCE OF DIFFERENTIATED FUNCTION OF THE SURFACTANT SYSTEM IN HUMAN FETAL LUNG TYPE II EPITHELIAL CELLS CULTURED ON PLASTIC

We report a simplified culture system for human fetal lung type II cells that maintains surfactant expression. Type II cells isolated from explant cultures of hormone-treated lungs (18-22 wk gestation) by collagenase + trypsin digestion were cultured on plastic for 4 days in serum-free medium containing dexamethasone (Dex, 10 nM) + 8-bromo-cAMP (0.1 mM) + isobutylmethylxanthine (0.1 mM) or were untreated (control). Surfactant protein (SP) mRNAs decreased markedly in control cells between days 1 and 4 of culture, but mRNA levels were high in treated cells on day 4 (SP-A, SP-B, SP-C, SP-D; 600%, 100%, 85%, 130% of day 0 content, respectively) . Dex or cAMP alone increased SP-B, SP-C, and SP-D mRNAs and together had additive effects. The greatest increase in SP-A mRNA occurred with cAMP alone. Treated cells processed pro-SP-B and pro-SP-C proteins to mature forms and had a higher rate of phosphatidylcholine (PC) synthesis (2-fold) and higher saturation of PC (~34% versus 27%) than controls. Only treated cells maintained secretagogue-responsive phospholipid synthesis. By electron microscopy, the treated cells retained lamellar bodies and extensive microvilli. We conclude that Dex and cAMP additively stimulate expression of surfactant components in isolated fetal type II cells, providing a simplified culture system for investigation of surfactant-related, and perhaps other, type II cell functions.