小儿特发性血小板减少性紫瘢T细胞亚群的观察

小儿特发性血小板减少性紫瘢Ｔ细胞亚群的观察田根全，袁文菊，程岩青海省儿童医院ＴＨＥＴ－ＣＥＬＬＳＵＢ－ＧＲＯＵＰＭＥＡＳＵＲＥＭＥＮＴＯＦＩＤＩＯＰＡＴＨＩＣＴＨＲＯＭＢＯＣＹＴＯＰＥＮＩＣＰＵＲＰＵＲＡ（ＩＴＰ）ＩＮＰＥＤＩＡＴＲＩＣＰＡＴＩＥＮＴ...     

小儿血液病骨髓活检的临床意义

小儿血液病骨髓活检的临床意义屠立明，沈亦逵，王若洁广东省人民医院儿科ＣＬＩＮＩＣＳＩＧＮＩＦＩＣＡＮＣＥＯＦＢＯＮＥＭＡＲＲＯＷＢＩＯＰＳＹＩＮＣＨＬＬＤＲＥＮＨＥＭＡＴＯＬＯＧＩＣＤＩＳＥＡＳＥＳ￥ＴｕＬｉｍｉｎｇ，ｅｔａｌ．（ＧｕａｎｇｄｏｎｇＰ...     

CD40L在川崎病发病机制中的作用探讨

为探讨ＣＤ４０Ｌ在儿童川崎病发病机制中的作用,用流式细胞技术及ＥＬＩＳＡ法检测急性期川崎病（ＫＤ）患儿外周血单个核细胞（ＰＢＭＣ）体外表达ＣＤ４０Ｌ水平、细胞凋亡率及可生ＣＤ４０Ｌ（ｓＣＤ４０Ｌ）对单核细胞株ＴＨＰ－１产生ＴＮＦ－α的影响。结果：ＫＤ表达ＣＤ４０Ｌ明显增高;存在淋巴细胞凋亡延迟;患儿ＰＢＭＣ培养上清（可能含有ｓＣＤ４０Ｌ）可诱导ＴＨＰ－１产生高浓度ＴＮＦ－α。抗ＣＤ４０Ｌ单抗可纠正     

血小板质控初探

血小板质控初探袁光孚，张玉芳，保菊英，秦振庭西宁市第一人民医院ＣＡＭＰＡＲＩＳＩＯＮＯＦＤＩＦＦＥＲＥＮＴＭＥＴＨＯＤＳＦＯＲＰＬＡＴＥＬＥＴＣＯＵＮＴＩＮＧＡＴＨＩＧＨＡＬＴＩＴＵＤＥＱＩＮＧＨＡＩ￥Ｋｕａｎｇ－ＦｕＹｕａｎ．ｅｔａｌ（Ｘｉ－Ｎｉｎ...     

HGFs联合对体外人脐血造血干细胞及SCID小鼠体内扩增作用的研究

本实验应用外源性重组人多种造血生长因子（ｒｈＨＧＦｓ）处理人ＨＳＣ／ＳＣＩＤ小鼠模型，以体外半固体培养法及ＦＣＡＳ检测ＣＦＵ－ＧＭ、ＣＦＵ－ＧＥＭＭ及ＣＤ３４＾＋细胞，从而评价ＨＧＦｓ对受鼠体内人造血干细胞的扩增作用。结果：人脐血及骨髓ＭＮＣ经孵育２４小时后ＣＤ３４＾＋细胞含量，ＣＦＵ－ＧＭ、ＣＦＵ－ＧＥＭＭ产率皆得到明显扩增（Ｐ〈０．０１）；且脐血组优于骨髓组（Ｐ〈０．０１）。输脐血ＭＮＣ＋ＨＧ     

哮喘患儿外周血白细胞介素10水平及其对白细胞介素2等的调节作用

目的 探讨白细胞介素２、１０（ＩＬ－２、１０），一氧化氮（ＮＯ）和肿瘤坏死因子α（ＴＮＦ－α）在小儿哮喘发病机制中的作用。方法 采用双抗夹心酶联免疫吸附试验（ＥＬＩＳＡ）技术检测了２８例哮喘患儿血浆及外周血单个核细胞（ＰＢＭＣ）在植物血凝素（ＰＨＡ）刺激下产生ＩＬ－１０的水平，同时检测了ＮＯ、ＩＬ－２和ＴＮＦ－α的水平，并研究了重组人ＩＬ－１０（ｒｈＩＬ－１０）对ＰＢＭＣ体外诱生ＮＯ、ＩＬ－２和Ｔ     

早搏与心功能的关系及影响因素的研究

为探讨小儿早搏对心功能的影响，采用多普勒超声心动图测定４０例早搏患儿的单个早搏射血分数（ＰＢＥＦ）、心脏指数（ＰＢＣＩ）；单个非早搏的射血分数（ＮＰＢＥＦ）、心脏指数（ＮＰＢＣＩ）及实际的射血分数（ＡＥＦ）、心脏指数（ＡＣＩ）。同时研究这些指标与心电图和心肌酶的关系。结果显示：（１）早搏患儿ＰＢＥＦ、ＰＢＣＩ均小于ＮＰＢＥＦ、ＮＰＢＣＩ；病程长者ＰＢＥＦ、ＡＥＦ及ＡＣＩ均下降；室性早搏者的ＡＣＩ减少；早搏＞１０次／分者的ＡＥＦ及ＡＣＩ下降；Ｒ－Ｒ′／Ｒ－Ｒ比值小者的ＰＢＥＦ、ＡＥＦ及ＰＢＣＩ亦小；早搏ＱＲＳ－Ｔ综合波长者的ＰＢＥＦ及ＰＢＣＩ减少。（２）肌酸磷酸激酶同功酶（ＣＫ－ＭＢ）升高者的ＰＢＥＦ、ＡＥＦ、ＰＢＣＩ、ＡＣＩ均值的下降比ＣＫ－ＭＢ不升高者显著。研究提示，早搏＞１０次／分、Ｒ－Ｒ′／Ｒ－Ｒ＜０．６、ＱＲＳ－Ｔ综合波＞０．４秒和ＣＫ－ＭＢ＞１６ＩＵ／Ｌ者的心功能多受到影响，应予以积极治疗。     

一氧化氮与Ⅰ型辅助细胞因子在过敏性紫癜中的作用

目的观察过敏性紫癜（ＨＳＰ）患儿急性期的一氧化氮（ＮＯ）及Ⅰ型辅助细胞（ＴＨ１）因子水平,以探讨其在该病全身血管炎发病机制中的作用。方法用镉还原法测定血浆ＮＯ水平,用ＥＬＩＳＡ法检测血浆及外周血单个核细胞（ＰＢＭＣ）培养上清中白细胞介素－２（ＩＬ－２）及干扰素－γ（ＩＦＮ－γ）水平。结果ＨＳＰ急性期血浆ＮＯ水平较对照组明显增高（Ｐ＜００５）,血浆ＩＬ－２及ＩＦＮ－γ与正常对照组比较无显著性差异（Ｐ＞００５）,但患儿的ＰＢＭＣ培养上清的ＩＬ－２及ＩＦＮ－γ明显低于正常对照组（Ｐ＜００５）。结论ＮＯ及ＴＨ１细胞因子在ＨＳＰ全身血管炎的发生、发展中起作用     

合胞病毒感染儿童淋巴细胞多种表面标记及细胞因子的初步研究

呼吸道合胞病毒（ＲＳＶ）感染发病机理的研究一直受到重视，研究者发现ＲＳＶ感染患儿存在着一系列免疫功能紊乱。本文用ＡＰＡＡＰ技术对ＲＳＶ感染患儿淋巴细胞ＣＤ３、ＣＤ４、ＣＤ８、ＣＤ２３、ＣＤ２５、ＣＤ５７、ＩＦＮｒ、ＨＬＡ－ＡＢＣ、ＨＬＡ－ＤＰ和ＨＬＡ－ＤＲ等表面标记进行检测，并用ＥＬＩＳＡ技术检测血浆中白细胞介素２（ＩＬ－２）、可溶性白细胞介素２受体（ｓＩＬ－２Ｒ）和呼吸道合胞病毒特异性ＩｇＥ（Ｒ     

小儿全身性癫痫与髓鞘碱性蛋白关系的研究

目的 通过检测脑脊液（ＣＳＦ）和血清髓鞘碱性蛋白（ＭＢＰ）的含量，了解小儿全身性癫痫是否引致髓鞘和血脑屏障（ＢＢＢ）的破坏，方法 采用简易ＭＢＰＥＬＩＳＡ法对４７ 小儿全身性癫痫病患儿脑脊液和血清标本作ＭＢＰ定量测定。结果 在一个月内有全身性癫痫发作的患儿其ＣＳＦ和血清ＭＢＰ含量均值明显高于正常对照组（Ｐ〈０．０１）。结论 小儿全身性癫痫发作可导致ＣＳＦ和血清ＭＢＰ升高，提示大脑器质性损伤和ＢＢＢ     

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.