rhG—CSF在小儿ANLL强烈化疗中的应用

为探讨ｒｈＧ－ＣＳＦ对小儿ＡＮＬＬ强烈化疗后粒细胞缺乏的疗效，采用ＡＡＥ方案（ＡＤＭ、Ａｒａ－Ｃ、ＶＰ１６或ＶＭ２６），化疗后当ＷＢＣ〈１×１０＾９／Ｌ或ＡＮＣ〈０．５×１０＾９／Ｌ时，给予ｒｈＧ－ＣＳＦ２００μｇ／ｍ＾２·ｄ（５￣１０μｇ／ｋｇ·ｄ），皮下注射，一般连续５￣１０天。本文１５例ＡＮＬＬ，用ｒｈＧ－ＣＳＦ３０例次。用ｒｈＧ－ＣＳＦ前，ＷＢＣ平均０．７８×１０＾９／Ｌ、ＡＮＣ０．１５×     

应激状态下新生儿对不同剂量外源性脂肪代谢的研究

为了探讨应激状态下新生儿应用两种不同剂量脂肪乳剂时的脂肪代谢情况，以指导临床合理应用。将１７例胎龄＞３５周，手术后需ＴＰＮ＞１周的新生儿，随机分成低剂量脂肪乳剂组（１ｇ·ｋｇ~（－１）／ｄ）８例，普通剂量对照组（２ｇ·ｋｇ~（－１）／ｄ）９例。在ＴＰＮ前、ＴＰＮ第３天和第７天分别观察ＴＧ、ＴＣ、ＰＬ、ＦＦＡ、ＨＤＬ－Ｃ、ＬＤＬ－Ｃ、Ａｐｏ－Ａ_１和Ａｐｏ－Ｂ变化，结果低剂量组ＴＰＮ１周期间，ＴＧ、ＴＣ、ＰＬ变化不明显，而对照组ＴＧ、ＴＣ在ＴＰＮ第３天升高（但＜１．５０ｇ／Ｌ），第７天回到基值；ＰＬ持续升高，Ａｐｏ－Ａ_１／Ａｐｏ－Ｂ的比值１周变化为：低剂量组１．４７→１．６０→１．４１，对照组１．２０→０．８５→０．９６，它的变化与ＴＧ、ＴＣ等血脂变化相一致。从研究中得出，足月新生儿ＴＰＮ时选用１ｇ·ｋｇ~（－１）／ｄ对血脂几乎没有影响，用２ｇ·ｋｇ~（－１）／ｄ虽然有一过性升高，但仍＜１．５０ｇ／Ｌ，是安全的。     

干扰素剂量和给药方法对婴幼儿呼吸道合胞病毒肺炎疗效的影响

研究目的探讨干扰素剂量和给药方法对婴幼儿呼吸道合胞病毒肺炎疗效的影响。研究方法婴幼儿呼吸道合胞病毒（ＲＳＶ）肺炎４８例，随机分为Ａ、Ｂ、Ｃ、Ｄ４组，每组１２例。各组基础治疗相同，Ａ组肌注大剂量α干扰素［α－ＩＦＮ，５×１０４ＩＵ／（ｋｇ·ｄ）］。Ｂ组肌注小剂量α－ＩＦＮ［２×１０４ＩＵ／（ｋｇ·ｄ）］，Ｃ组肌注小剂量α－ＩＦＮ加雾化吸入小剂量γ－ＩＦＮ（３×１０３ＩＵ／次），Ｄ组雾化吸入小剂量γ－ＩＦＮ．结果Ｄ组患者退热时间比Ａ、Ｂ、Ｃ３组明显延长（Ｐ＜０．０１），其他症状（如咳嗽、呼吸困难、喘鸣、干、湿罗音等）消失时间Ａ与Ｃ组、Ｂ与Ｄ组相比均无显著差异（Ｐ＞０．０５），Ａ、Ｃ组与Ｂ、Ｄ组相比，有显著性差异，Ａ、Ｃ组显效较早。结论ＩＥＮ治疗ＲＳＶ肺炎的疗效与给药剂量和方法密切相关，小剂量ＩＦＮ治疗效果较差。小剂量α－ＩＦＮ肌注配合小剂量γ－ＩＦＮ雾化吸入治疗ＲＳＶ肺炎效果最好。     

环磷酰胺冲击治疗儿童系统性红斑狼疮（附6例报告）

探讨儿童系统性红斑狼疮（ＳＬＥ）的治疗方法及环磷酰胺（ＣＴＸ）的疗效。ＳＬＥ患者共１４例，ＣＴＸ冲击治疗组６例，ＣＴＸ每疗程（２０～３０）ｍｇ／ｋｇ，分２ｄ静滴，疗程间隔１５ｄ，同时加用强的构１ｍｇ／ｋｇ。强的松治疗组８例，强的松２ｍｇ／ｋｇ．ｄ）连服８周后渐减量维持。ＣＴＸ冲击治疗组８周内显效５例，有效１例，总有效率为１００％，强的松治疗组仅１例完全缓解，５例有效，２例无效，总有效率为７５％（Ｐ     

大剂量维生素C在新生儿再灌注损伤中的应用

观察了大剂量维生素Ｃ（ＶｉｔＣ）用于新生儿再灌注损伤的疗效。结果ＶｉｔＣ１ｇ／（ｋｇ·ｄ）的疗效明显优于０．５ｇ／（ｋｇ·ｄ）。前者首次用药后患儿血清丙二醛（ＭＤＡ）明显减少，总超氧化物歧化酶（ＳＯＤ）明显增加（Ｐ＜０．００１），血液酸度无明显变化（ｐＨ：Ｐ＞０．１，ＨＣＯ３－：Ｐ＞０．０５，ＢＥ；Ｐ＞０．１）。而后者首次用药后患儿血清ＭＤＡ无明显减少（Ｐ＞０．０５），ＳＯＤ虽明显增加（Ｐ＜０．０１），但增加的幅度明显低于前者，提示ＶｉｔＣ作为自由基清除剂治疗新生儿再灌注损伤时，剂量以１ｇ／（ｋｇ·ｄ）为宜。     

碱性成纤维细胞生长因子对新生大鼠缺氧缺血性脑损伤的保护作用

目的 探讨碱性成纤维细胞生长因子（ ｂＦＧＦ）对新生大鼠缺氧缺血性脑损伤( ＨＩＢＤ）的保护作用。方法 将 ３２只 ７日龄Ｗｉｓｔａｒ大鼠分为４组：假手术组、ＨＩＢＤ组、 ｂＦＧＦ治疗组（分大剂量组１７．５ μｇ／ｋｇ和小剂量组１０ μｇ／ｋｇ）,每组 ８只。后３组动物制成 ＨＩＢＤ模型,给予治疗组大鼠连续 ７ ｄ腹腔注射 ｂＦＧＦ, ＨＩＢＤ组腹腔注射等体积生理盐水作为对照。全部大鼠于术后１４ ｄ处死,对脑纹状体、皮质的光镜下结构、乙酰胆碱酯酶（ＡｃｈＥ）、酸性磷酸酶（ＡＣＰ）活性变化进行观察和比较。结果 新生大鼠 ＨＩＢＤ后皮质、纹状体有选择性神经元坏死及胶质细胞增生, ｂＦＧＦ治疗组上述病变明显减轻; ＨＩＢＤ后纹状体、皮质神经元 ＡｃｈＥ活性明显下降(－～＋）, ｂＦＧＦ组 ＡｃｈＥ活性（＋～＋＋）较ＨＩＢＤ组恢复快; ＨＩＢＤ后纹状体神经元 ＡＣＰ活性明显增高（＋＋＋）, ｂＦＧＦ组 ＡＣＰ活性（＋＋）变化程度小于ＨＩＢＤ组。但大、小剂量ｂＦＧＦ治疗组未见明显差异。结论 ｂＦＧＦ能通过影响受损神经元的酶物质代谢而加快ＨＩＢＤ新生大鼠神经元的修复。     

选择性头部亚低温对新生儿缺氧缺血性脑损伤的治疗作用

目的 探讨选择性头部亚低温对足月新生儿窒息后缺氧缺血性脑损伤（ＨＩＢＤ）的神经保护作用及其安全性。方法 将２２例重度窒息足月新生儿随机分为治疗组（１１例）和对照组（１１例）。治疗组采用选择性头部降温方法,维持鼻咽温度为（３４．０±０．２）℃,持续７２ ｈ;对照组不进行降温治疗。两组均于治疗后６４～７２ ｈ测脑脊液神经元特异性烯醇化酶（ＮＳＥ）,血ＣＫ－ＭＢ,尿β２微球蛋白（β２－ＭＧ）等。于治疗前、生后１０ ｄ和３个月进行常规１６导ＥＥＧ检测,并采用新生儿神经行为评分（ＮＢＮＡ）、婴幼儿发育量表（ＣＤＣＣ）进行神经行为发育评价。结果 治疗组脑脊液ＮＳＥ为（１９．５±２．２）μｇ／Ｌ,明显低于对照组[（２４．６±５．３）μｇ／Ｌ］（Ｐ ＜ ０．０１）;生后２８ ｄ治疗组ＮＢＮＡ评分为（３６±３）分,低于对照组[（３２±２）分］（Ｐ＜０．０１）。治疗前两组ＥＥＧ均异常,生后１０ｄ,３个月治疗组ＥＥＧ均正常,对照组２例持续重度异常。两组患儿血ＣＫ－ＭＢ及尿β２－ＭＧ差异无显著性（Ｐ＞０．０５）。结论 选择性头部亚低温对足月新生儿窒息后ＨＩＢＤ可能具有神经保护作用,临床上具有安全性。     

血清C-反应蛋白和唾液酸联合测定在新生儿细菌感染性疾病中的意义

目的 探讨血清Ｃ－反应蛋白（ＣＲＰ）和唾液酸（ＳＡ）联合测定在新生儿早期细菌感染诊断和疗效观察中的意义。方法 同时测定５２例起病在１周内的细菌感染新生儿血清ＣＲＰ和ＳＡ水平。结果 ＣＲＰ在起病２４ｈ内即增加到（６８．３±３２．８）ｍｇ／Ｌ,与正常对照组[（０．７８±０．２５）ｍｇ／Ｌ］相比差异具有显著性（Ｐ＜０．０１）,当炎症得到一定控制后则明显下降（Ｐ＜ ０．０１）。ＳＡ在起病２４ ｈ内逐渐增加,至１～３ ｄ和４～７ ｄ时分别升至（１．８８±０．８５）,（２．９５±０．８７）ｍｍｏｌ／Ｌ,与对照组[（０．９１±０．４０）ｍｍｏｌ／Ｌ］比较差异具有显著性意义（Ｐ＜ ０．０１）,当感染得到完全控制时可降至正常对照水平。结论 血清ＣＲＰ和ＳＡ的联合测定,有助于提高新生儿早期细菌感染性疾病的诊疗水平。     

手术前抗肿瘤药物VCA联合化疗对小鼠伤口拉力的影响

目的 探讨手术前抗肿瘤药物联合化疗对小鼠伤口愈合的影响，以寻找联合化疗后最佳手术时机。方法 实验小鼠设４组，每组４０只，Ａ组为对照经且，Ｂ、Ｃ、Ｄ组为化疗后不同时间的实验组，给予ＶＣＡ联合化疗，分别于化疗后０ｄ、７ｄ和１４ｄ在背部做相同切口并缝合，所有小鼠术后７处死，愈合伤口做拉力实验。结果 Ｂ组与其他３组比较差异有显著意义（Ｐ〈０．０５），而Ａ、Ｃ、Ｄ组之间有显著意义（Ｐ〈０．０５），Ｂ组与Ａ组     

与系统性红斑狼疮发病相关的遗传因素研究进展

系统性红斑狼疮（ＳＬＥ）是一种典型的自身免疫性疾病,发病与遗传因素有关。目前已知多种遗传基因影响该病的易感性,其中主要为人类白细胞抗原（ＨＬＡ）区域基因。ＨＬＡ区域基因分为三类,对Ⅰ类区域基因（Ａ．Ｂ、Ｃ位点）的研究较早,近年主要集中在对Ⅱ类区域基因的研究。该区域基因包括ＤＲＢ位点及ＤＱ、ＤＰ、ＤＯ、ＤＭ等基因位点,其中对ＤＲＢ、ＤＱ位点的研究较多。Ⅲ类区域基因主要为补体成分Ｃ２、Ｃ４、ＢＦ又肿瘤坏死因子、热休克蛋白７０等基因位点,它们与ＳＬＥ相关性研究的报道也较多见。除此而外,ＨＬＡ区域以外的基因尚有 Ｆａｓ基因、 ｂｃｌ－２基因、 Ｐ５３基因及 ｃ－ｍｙｃ基因,这些基因均通过影响细胞凋亡而影响ＳＬＥ的发病。本文对上述基因与ＳＬＥ的疾病相关性进行综述。     

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.