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     

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     

新生儿感染性疾病白细胞介素2受体和IgG_4水平的测定

应用三抗体和双抗体夹心ＥＬＩＳＡ法，测住院新生儿感染性疾病和对照组各２０例的血清可溶性白细胞介素２受体（ＳＩＬ－２Ｒ）和血清、唾液ＩｇＧ_４水平。结果患儿组ＳＩＬ－２Ｒ为３０７±３６ｋＵ／Ｌ，对照组为１９８±３２ｋＵ／Ｌ（Ｐ＜０．０５）；患儿组血清ＩｇＧ_４为３９．２±６．５ｍｇ／Ｌ，对照组为１８．２±２．０ｍｇ／Ｌ（Ｐ＜０．０１）；患儿组唾液ＩｇＧ_４为０．１６７＋０．０３６ｍｇ／Ｌ，对照组０．０５５±０．０１４ｍｇ／Ｌ（Ｐ＜０．０１），患儿组三项指标均高于对照组。提示新生儿时期对感染有一定的免疫反应能力。     

小儿髓外白血病防治方法的研究

为降低中枢神经系统白血病（ＣＮＳＬ）和睾丸白血病（ＴＬ）发病率，对１４０例急性白血病恩儿分别采用￣（６０）Ｃｏ头颅照射（￣（６０）Ｃｏ）、大剂量氨甲蝶呤静注（ＨＤＭＴＸ）、￣（６０）Ｃｏ＋ＨＤＭＴＸ、ＨＤＭＴＸ＋￣（６０）Ｃｏ四种方法进行ＣＮＳＬ和ＴＬ防治研究。结果表明：四组防治ＣＮＳＬ的疗效相似，ＣＮＳＬ发生率无显著差异（Ｐ＞０．０５）；急性淋巴细胞白血病早期采用ＨＤＭＴＸ预防ＴＬ效果满意，而￣（６０）Ｃｏ组ＴＬ发生率高。从整体防冶效果评价，ＨＤＭＴＸ静注优于￣（６０）Ｃｏ头颅照射。１２例接受双侧睾丸放疗配合全身化疗，ＴＬ均未发生，３例只接受患侧睾丸放疗配合全身化疗者均发生ＴＬ。提示早期使用ＨＤＭＴＸ，必要时配合￣（６０）Ｃｏ放疗可防止中枢神经系统白血病和睾丸白血病的发生。     

G—CSF在白血病骨髓抑制期的应用

报告Ｇ－ＣＳＦ在小儿白血病骨髓抑制期的临床应用，共２３例（４３例次），并设对照组比较。在２３例急性白血病患儿，２０例为急性淋巴细胞白血病，３例为髓系白血病。结果：骨髓抑制期应用Ｇ－ＣＳＦ组与对照组比较，中性粒细胞绝对性（ＡＮＣ）恢复到ＡＮＣ〉１×１０＾９／Ｌ及血小板（ＰＬＴ）恢复到ＰＬＴ〉１００×１０＾９／Ｌ的时间明显缩短（Ｐ〈０．０５）及感染率明显降低（Ｐ〈０．０５），且副反应轻。此结果提示，Ｇ     

低出生体重儿远期听力、视力与智力随访

目的 探讨低出生体重（ＬＢＷ）儿远期听力、视力及智力发育情况。方法 对４６例ＬＢＷ儿在４￣８．５ａ时进行随访，以４０例同龄正常出生体重（ＮＢＷ０儿作对照，检测吸力、视力与智商（ＩＱ）。结果 ＬＢＷ儿听力、视力及ＩＱ异常率均高于对照组（Ｐ均〈０．０５），平均听阈高于对照组（Ｐ〈０．０１），而平均视力与ＩＱ分别低于对照组（Ｐ均〈０．０１），ＬＢＷ儿出生体重分别与ＩＱ、双眼平均视力呈正相关（ｒ＝０．３９     

抗DNA酶B对小儿A组溶血性链球菌感染的诊断价值

目的探索抗ＤＮＡ酶Ｂ测定Ａ组溶血性链球菌（ＧＡＳ）感染血清学方法的诊断价值。方法用抗ＤＮＡ酶Ｂ微量法和自制试剂，测定５４８例ＧＡＳ感染所致疾病的小儿血清抗ＤＮＡ酶Ｂ抗体，同时测定抗链球菌溶血素Ｏ（ＡＳＯ）。结果（１）急性风湿热组（ＡＲＦ）抗ＤＮＡ酶Ｂ阳性率（７２．５％）与ＡＳＯ阳性率（７６．５％）比较，差异无显著意义（Ｐ＞０．０５）；但活动期风湿性心脏病（简称风心病）组病人的抗ＤＮＡ酶Ｂ阳性率（８２．６％）明显超过ＡＳＯ阳性率（５５．４％）（Ｐ＜０．００１）；抗ＤＮＡ酶Ｂ联合ＡＳＯ测定在两组的阳性率均在９０％左右。（２）急性肾小球肾炎（ＡＧＮ）皮损组抗ＤＮＡ酶Ｂ阳性率９２．６％，明显超过ＡＳＯ的５９．２％（Ｐ＜０．０５）。（３）ＡＧＮ上感组抗ＤＮＡ酶Ｂ与ＡＳＯ的阳性率分别为７６．７％和７３．９％（Ｐ＞０．０５）。抗ＤＮＡ酶Ｂ联合ＡＳＯ的阳性率在上感组和皮损组均超过９０％。结论抗ＤＮＡ酶Ｂ在诊断ＡＲＦ和ＡＧＮ方面具有重要的临床价值，特别与ＡＳＯ联合应用价值更大；对活动期风心病和ＧＡＳ皮肤感染致ＡＧＮ的诊断价值明显优于ＡＳＯ。     

小儿急性白血病骨髓细胞糖皮质激素受体表达及其临床意义

本文应用放射配体结合法测定了３０例ＡＬＬ骨髓细胞ＧＣＲ为７８７３±３００２位点／细胞；１７例ＡＮＬＬ为６１１３±１６２２位点／细胞（Ｐ＜０．０５）。形态学分型Ｌ１型１７例，ＧＣＲ为７２６７±３１７８位点／细胞；Ｌ２型１３例，ＧＣＲ为７６５０±３３６２位点／细胞（Ｐ＞０．０５）。临床分型标危型ＡＬＬ１４例，ＧＣＲ为８９９８±１９６３位点／细胞；高危型９例为５８４７±３８８５位点／细胞（Ｐ＜０．０５）。对１８例ＡＬＬ经联合化疗完全缓解前后比较发现，ＧＣＲ分别为８１１５±３２５８位／细胞和４６６９±２１０６位点／细胞（Ｐ＜０．０１）。本实验同时测定了ＡＬＬ和ＡＮＬＬ患儿血浆皮质醇浓度，平均分别为１１２．２０±４１．１４ｎｇ／ｍｉ和１０４．６５±４３．４９ｎｇ／ｍｌ，相关系数分别为ｒ＝０．１５和０．１１，说明ＧＣＲ表达水平不受皮质醇含量的影响。     

急性白血病患儿血清肿瘤坏死因子a的活性变化及临床意义

用放射免疫方法测定了２０例正常儿童及４０例急性白血病（ＡＬ）患儿化疗前后血清肿瘤坏死因子（ＴＮＦ－ａ）浓度。结果：在ＡＬ诊断时，ＴＮＦ－ａ水平显著升高；当患儿获得部分缓解至完全缓解时，ＴＮＦ－ａ水平呈梯形降低，其最低值仍高于健康儿童（Ｐ〈０．０１￣０．０５）；２２例急性淋巴细胞白血病（ＡＬＬ）患儿ＴＮＦ－ａ水平在任何阶段都比１８例急性非淋巴细胞白血病（ＡＮＬＬ）患儿低（Ｐ〈０．０１）；ＴＮＦ－ａ水     

流式细胞仪分析儿童急性淋巴细胞白血病免疫表型63例

为了了解儿童急性淋巴细胞白血病的免疫表型,应用一组单克隆抗体及流式细胞仪间接免疫荧光法对６３例初治儿童急性淋巴细胞白血病(ＡＬＬ）免疫分型：Ｔ淋巴细胞型、Ｂ淋巴细胞型、Ｔ／Ｂ混合型及裸核（Ｎｕｌｌ型）ＡＬＬ分别为４１．３％、４６％、９．５％、３．２％,髓系抗原（Ｍｙ）及ＣＤ(３４)表达率为１７．４％和２８．５％,Ｍｙ＋ＡＬＬ中ＣＤ(３４)表达为５４．５％,显著高于Ｍｙ－ＡＬＬ（２３．３％）（Ｐ＜０．０１）,Ｔ／Ｂ混合型ＡＬＬ对Ｍｙ及ＣＤ(３４)均有高表达,与Ｔ、Ｂ－ＡＬＬ相比均有显著差异（Ｐ＜０．０１－０．０５）     

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.