儿童特发性血小板减少性紫癜的临床观察

观察了８０例特发性血小板减小性紫（ＩＴＰ），治疗前血小板数均１９×１０＾９／Ｌ并伴有明显出血症状，选择口服醋酸泼尼松组１９例，剂量为１ｍｇ／ｋｇ，疗程６－８周。静脉丙球蛋白组２８例，剂量为０．２５－０．４０／ｋｇ，时间为２－５天，用前给地塞米松３－５ｍｇ预防过敏反应。免疫抑制组１１例为西艾克３ｍｇ／ｍ＾２／周或ＶＣＲ１．５ｍｇ／ｍ＾２／周，静脉缓慢滴注，用４－６次。切脾组１９例均选择激素无效或依赖     

每周一次补铁方案对学前儿童铁营养与体格发育作用的研究

２５５名３～５岁学龄前儿童随机会为每周或每日补铁组，补铁剂量为每周一次或每日４ｍｇ／ｋｇ。经两个月补铁试验，观察比较正常与贫血儿童对两种不同补铁方案，在改善儿童铁营养状况和促进体格发育方面的作用。结果显示，每周一次补铁方案使贫血患病率由２４．４％下降到５．７％，血红蛋白含量增加１２．８ｇ／Ｌ。每周一次补铁儿童补铁后身高、体重、上臂围净增长值分别为１．３～１．５ｃｍ，０．５～０．７ｋｇ、０．１５～０．１３ｃｍ，显著高于正常对照儿童。每周一次补铁方案在改善儿童铁营养，促进体格发育方面的作用与每日补铁方案无显著性差异（Ｐ＞０．０５）。     

肺表面活性物质治疗新生儿RDS呼吸衰竭的疗效

用肺表面活性物质（ＰＳ）抢救１５例新生儿呼吸窘迫综合征（ＮＲＤＳ）时的呼吸衰竭，ＰＳ首次剂量２００ｍｇ／ｋｇ．重复剂量１００ｍｇ／ｋｇ，用１～３次，经气管插管注入肺内，给ＰＳ后５～１５分钟ＰａＯ２和ＰａＯ２／ＦｉＯ２显著上升，随后ＦｉＯ２和平均气道压（ＭＡＰ）下调，５．５±０．８小时胸片即见改善，４０．２±１６．５小时胸片恢复，治疗组病死率比对照组低。结果表明ＰＳ对抢救ＮＲＤＳ呼吸衰竭有明显疗效。     

婴幼儿动脉导管未闭术后急性高血压的临床治疗

本文报告了自１９９３年１月至１９９６年９月期间，４８例婴幼儿动脉导管未闭术后高血压的临床治疗，用硝普钠和卡托普利治疗，硝普钠剂量为１～５μｇ／ｋｇ．ｍｉｎ，连续静脉推注，手术后１２小时开始口服卡托普利，剂量为１～２ｍｇ／ｋｇ．ｄ疗程为２～４周，有效率为９７．９％。     

每周一次补铁方案对学前儿童铁营养与体格发育作用的研究

２５５名３￣５岁学龄前儿童随机会为每周或每日补铁组，补铁剂量为每周一次或每日４ｍｇ／ｋｇ。经两个月补铁试验，观察比较正常与贫血儿童对两种不同补铁方案，在改善儿童铁营养状况和促进体格发育方面的作用。结果显示，每周一次补铁方案使贫血患病率由２４．４％下降到５．７％，血红蛋白含量增加１２．８ｇ／Ｌ。每周一次补铁儿童补铁后身高、体重、上臂围净增长值分别为１．３￣１．５ｃｍ，０．５￣０．７ｋｇ、０．１５￣０     

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     

抗心律失常药物在儿科急救中的应用

抗心律失常药物的应用是许多高危心律失常的重要抢救措施。现就儿科急救医学领域中常用的抗心律失常药简述如下。１　利多卡因ａ．　适应证：室性心律失常。为室性心动过速及室颤、频发室性早搏（多源性或连续３次以上室性早搏）首选药物，属Ⅰｂ类抗心律失常药。ｂ．　剂量：每次１ｍｇ／ｋｇ静脉注射，静注后１５～３０秒即起效，５分钟达高峰，维持１０～３０分钟。如无效，可每５～１０ｍｉｎ重复１次至有效，总剂量不超过３～５ｍｇ／ｋｇ。根据病情，可用７～１０天。如室性心动过速反复发作则静滴：２０～５０μｇ／（ｋｇ·ｍｉｎ）…     

酚妥拉明致严重鼻塞、呼吸困难、心衰2例

酚妥拉明致严重鼻塞、呼吸困难、心衰２例海南三亚市医院儿科（５７２０００）陈绿林，林毅例１，男，２０天，Ｇ２Ｐ２，因新生儿肺炎并心力衰竭入院。用酚妥拉明（０．５～１）ｍｇ／（ｋｇ·次）与半量阿拉明同置于１０％葡萄糖液（１０～２０）毫升内缓慢静脉滴注，当...     

小儿肾病综合征的冲击疗法

研究目的探讨小儿肾病综合征的治疗方法。研究方法小儿肾病综合征患儿共６６例，随机分为两组：冲击组（３４例）和对照组（３２例）。冲击组用地塞米松（１．５－３）ｍｇ／（ｋｇ．ｄ）加入１０％ＧＳ溶液（１００－１５０）ｍｌ静滴，每日１次，连用３ｄ，第４日停用，第５日开始隔日１次，共用６次。次日开始口服强的松（１．５－２）ｍｇ／（ｋｇ．ｄ），共４周，后渐减量。对照组仅用强的松，用法用量同冲击组。结果冲击完全缓     

氯高铁血红素抗贫血疗效及追踪调查报告

本文报告用氯高铁血红素每天３００ｍｇ／ｋｇ、１５０ｍｇ／ｋｇ、５０ｍｇ／ｋｇ治疗失血性小鼠。治疗后Ｈｂ明显高于阴性对照组；大、中剂量组疗效优于葡萄糖酸亚铁组。对１２０例缺铁性贫血小儿予氯高铁血红素口服液治疗（１－２ｍｇ／ｋｇ·ｄ），治疗后Ｈｂ明显升高，ＦＥＰ明显下降；治愈率达８７．５％，总有效率１００％；治疗结束后３月，６月及１年追踪随访示有８７．５％、７５％、６０％小儿Ｈｂ≥１１０ｇ／Ｌ。提示：     

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.