狼疮脑病患儿脑脊液中抗神经节甙抗体的研究

神经精神性红斑狼疮（ｎｅｕｒｏｐｓｙｃｈｉａｔｒｉｃｌｕｐｕｓｅｒｙｔｈｅｍａｔｏｓｕｓ，ＮＰＬＥ）又称狼疮脑病，是系统性红斑狼疮（ｓｙｓｔｅｍｉｃｌｕｐｕｓｅｒｙｔｈｅｍａｔｏｓｕｓ，ＳＬＥ）的一种内脏合并症。在儿童，ＮＰＬＥ的患病率约为狼疮患儿...     

脑脊液肿瘤坏死因子测定在中枢神经系统白血病的临床意义

本文检测１００例正常人，急性白血病患儿脑脊液中肿瘤坏死因子（ＴＮＦ），并对已发生中枢神经系统白血病（ＣＮＳＬ）的１０例患儿进行动态监测，测得正常儿童脑脊液（ＣＳＦ）中ＴＮＦ为８．８６±０．８０ｎｇ／ｍｌ，无发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ为９．３２±１．５２ｎｇ／ｍｌ，而发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ显著升高达２０．６１±２．２１ｎｇ／ｍｌ，治疗缓解后下降为９．１６±０．７６ｎｇ／ｍｌ。提示ＴＮＦ检测对临床观察ＣＮＳＬ的发生发展与转归有一定价值。     

脑脊液肿瘤坏死因子测定在中枢神经系统白血病的临床意义

本文检测１００例正常人，急性白血病患儿脑脊液中肿瘤坏死因子（ＴＮＦ），并对已发生中枢神经系统白血病（ＣＮＳＬ）的１０例患儿进行动态监测，测得正常儿童脑脊液（ＣＳＦ）中ＴＮＦ为８．８６±０．８０ｎｇ／ｍｌ，无发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ为９．３２±１．５２ｎｇ／ｍｌ，而发生ＣＮＳＬ儿童ＣＳＦ中ＴＮＴ显著升高达２０．６１±２．２１ｎｇ／ｍｌ，治疗缓解后下降为９．１６±０．７６ｎｇ／ｍｌ。提示ＴＮＦ检     

儿童系统性红斑狼疮肺损伤致呼吸窘迫综合征二例

儿童系统性红斑狼疮肺损伤致呼吸窘迫综合征二例耿荣陈贤楠何晓琥儿童系统性红斑狼疮（ＳＬＥ）肺部损害发生率约为３９％～８９％，以胸膜和肺间质病变为主，合并呼吸窘迫综合征（ＡＲＤＳ）较少见。现报道２例。例１女，７岁，间断发热、四肢关节疼伴皮疹１年半，呼吸困...     

新生儿缺氧缺血性脑病脑脊液神经元特异性烯醇化酶测定的临床意义

目的探讨新生儿缺氧缺血性脑病脑脊液中神经元特异性烯醇化酶水平的变化及其临床意义，方法新生儿缺氧缺血性脑病（ＮＨＩＥ）患儿４７例，轻、中、重度分别为８、２５、１４例，选择无窒息史、无神经系统疾病的新生儿１０例为对照组。出生后２～４天抽取患儿及对照组脑脊液，用双抗体夹心酶联免疫法测定脑脊液中神经元特异性烯酸化酶（ＮＳＥ），并对测定结果进行统计学分析。结果对照组及轻、中、重度ＮＨＩＥ患儿脑脊液中ＮＳＥ水平分别为１０．１９６±３．２３７μｇ／Ｌ，１３．９４２±４．６７３μｇ／Ｌ，４５．６５８±１４．５４６μｇ／Ｌ，１０５．５１５±３９．６５２μｇ／Ｌ。对照组与轻度ＮＨＩＥ比较，差异不显著（Ｐ＞０．０５），与中、重度ＮＨＩＥ，比较差异极为显著（Ｐ＜０．０１）。轻度与中、重度ＮＨＩＥ及中度与重度ＮＨＩＥ比较有极显著性差异（Ｐ＜０．０１）。结论脑脊液中ＮＳＥ含量与ＮＨＩＥ患儿脑损伤的程度有关，随着ＮＨＩＥ患儿脑损伤程度的加重，其脑脊液中ＮＳＥ含量逐渐升高。因此，脑脊液中ＮＳＥ含量可作为早期判断ＮＨＩＥ脑损伤程度的生化指标。     

神经节甙抗体在儿童系统性红斑狼疮脑病诊断中的意义

为了解儿童系统性红斑狼疮（ＳＬＥ）及狼疮脑病（ＮＰＬＥ）时抗神经节甙抗体（ＡＧＡ）的存在及血清或脑脊液中ＡＧＡ的临床相关性。采用酶标记免疫测定法检测了４７例ＳＬＥ患儿（其中１３例临床诊断为ＮＰＬＥ）的７１份血清标本、８份脑脊液标本中的ＡＧＡ水平。结果脑脊液中的ＡＧＡ与ＮＰＬＥ有明显相关性，５例ＮＰＬＥ脑脊液中ＩｇＧ－ＡＧＡ阳性、４例ＩｇＭ－ＡＧＡ阳性。２例的纵向观察显示脑脊液中的ＡＧＡ变化与临床病程相关。提示ＡＧＡ有可能作为儿童ＮＰＬＥ诊断的重要指标之一。     

新生儿胃食管反流发病机理的研究

为探讨新生儿胃食管反流（ＧＥＲ）的发病机理，对３８例经钡餐造影诊为ＧＥＲ的患儿进行食管ｐＨ值动态监测和食管动力功能检查，１５例无症状儿作对照组。结果：ＧＥＲ组各项反流指标均显著大于对照组。３８例中１８例为生理性ＧＥＲ，２０例为病理性ＧＥＲ。病理性反流组下食管括约肌压力（ＬＥＳＰ）和屏障压（ＢＰ）均显著低于对照组，而食管功能的其他指标则差异无显著意义。以总ｐＨ值＜４百分时间２．７７％和综合评分８．９２为９５％参考值上限，则ＧＥＲ组病理性反流的检出率为５５．３％（２１／３８），高于对照组的６．７％（１／１５）（Ｐ＜０．０１）。ＬＥＳＰ和ＢＰ的９５％参考值下限分别为８．３９ｋＰａ、８．１５ｋＰａ，对照组无一例ＬＥＳＰ低下，ＧＥＲ组ＬＥＳＰ降低占１５．７％（６／３８），二组差异无显著意义（Ｐ＞０．０５）。提示：新生儿期食管功能已成熟，新生儿ＧＥＲ的发生不单是ＬＥＳＰ降低这一因素，还可能与短暂下食管括约肌松驰有关。     

急性淋巴细胞性白血病患儿脑脊液腺苷脱氨酶活性的测定

为了探讨脑脊液腺苷脱氨酶（ＡＤＡ）对中枢神经系统白血病（ＣＮＳＬ）的诊断价值，用比色法对７例急性淋巴细胞性白血病（ＡＬＬ）伴ＣＮＳＬ和１４例无ＣＮＳＬ的ＡＬＬ患儿进行了脑脊液ＡＤＡ活性的测定并与１５例对照儿的测定值比较。结果，无ＣＮＳＬ的ＡＬＬ患儿的测定值与对照组比较无明显差别（Ｐ〉０．０５），而伴ＣＮＳＬ的ＡＬＬ患儿的测定值明显高于无ＣＮＳＬ的ＡＬＬ患儿和对照儿的测定值（Ｐ〈０．０５），并随治疗     

脑脊液纤维结合蛋白和铁蛋白在CNSL的临床意义

本文观察了急性白血病患者ＣＳＦ－ＦＮ和Ｆ含量，结果ＣＮＳＬ患者ＣＳＦ－ＦＮ和Ｆ含量全部升高，与对照组及无ＣＮＳＬ组比较有极显著性差异（Ｐ〈０．０１）。１０例ＣＮＳＬ患者治疗后８例缓解的ＣＳＦ－ＦＮ和Ｆ明显下降；一例虽缓解但下降不明显，半月后复发。另一例病情恶性，ＣＳＦ－ＦＮ和Ｆ反较治疗前升高。无ＣＮＳＬ组３０例患者９例ＣＳＦ－ＦＮ增高，８／２３例ＣＳＦ－Ｆ增高，其中二项同时增高的有７例，随访１５－     

急性淋巴细胞性白血病患儿脑脊液中肿瘤坏死因子及可溶性白细？…

用ＲＡＩ和ＲＬＩＳＡ法对２５例急性淋巴细胞白血病患儿脑脊液肿瘤坏死因子（ＴＮＦ）和可溶性白细胞介素２受体（ＳＩＬ－２Ｒ）进行测定，１０例中枢神经系统白血病（ＣＮＳＬ）患儿脑脊液中ＴＮＦ及ＳＩＬ－２Ｒ测定值均明显高于无ＣＮＳＬ的白血病患儿及对照儿（Ｐ〈０．００１），并且二者在ＣＮＳＬ患儿脑脊液中的增高程度呈正相关（ｒ＝０．６４３，Ｐ〈０．０５）。无ＣＮＳＬ组与对照组脑脊液ＴＮＦ、ＳＩＬ－２Ｒ的测定值     

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.