张力—应力定律在肛门直肠狭窄治疗中的应用：一种非手术…

利用张利－应力原理，采用球囊持续扩张方法，治疗肛门直肠狭窄狭窄患儿１３例，其中先天性肛门狭窄２例，肛门成形术后肛门狭窄５例，先天性直肠狭窄１例，术扣或外伤性直肠狭窄４例，先天性肛门直肠狭窄１例。狭窄段长度０．６－１．０ｃｍ例，１．２－５．０ｃｍ９例。     

哮喘患儿口服长效缓释茶碱代谢动力学及用药方案探讨

研究哮喘患儿口服长效缓释茶碱８．８ｍｇ／ｋｇ的代谢动力学特点及合理用药方案。代谢动力学参数：达峰时（Ｔｍａｘ）为（５．９１±０．３２）ｈ，峰浓度（Ｃｍａｘ）为（１０．４１±０．８８）ｍｇ／Ｌ，血药半衰期（Ｔ１／２）为（６．８４±１．３４）ｈ，总消除率（Ｔｃｌ）为（０．７８８±０．０９５）ｍｌ／（ｍｉｎ·ｋｇ）。比较了患儿相同日剂量而间隔１２小时及间隔８小时服药的经时血茶碱浓度。两种服药方案的血药峰浓度（Ｃｍａｘ）没有显著差异，而间隔８小时服药时的血药谷浓度（Ｃｍｉｎ）较间隔１２小时服药时明显升高，血药波动度明显下降，血茶碱浓度更为平稳。     

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     

应用生玉米淀粉治疗肝糖原贮积症的临床研究

用生玉米淀粉悬液，每次１．７５ｇ／ｋｇ餐间和夜间分次口服治疗肝糖原贮积症患儿１７例取较好的效果。结果表明：服生玉米淀粉后低血糖症状明显好转，空腹血糖治疗前平均为２．２ｍｍｏｌ／Ｌ（３９．６ｍｇ／ｄｌ），治疗后为４．２ｍｍｏｌ／Ｌ（７５．６ｍｇ／ｋｌ，Ｐ＜０．０５）。随防３个月至３年半的病例，其肝脏平均缩小３ｃｍ）（Ｐ＜０．０５）。身高平均半年增长５ｃｍ。提示本法对肝糖原贮积症积症的低血糖、肝大及生     

同胞姐妹铁粒幼细胞性贫血二例

同胞姐妹铁粒幼细胞性贫血二例范玉金杜庆寿汪桂荣例１女，３岁。因进行性贫血３个月伴发热３天入院。体检：体温３９．６℃，重度贫血貌。肝肋下１．５ｃｍ，脾肋下１．０ｃｍ。实验室检查：血红蛋白４３ｇ／Ｌ，红细胞１．５×１０１２／Ｌ；白细胞１２．４×１０９／Ｌ...     

改良Okada手术治疗女婴肛门直肠畸形

改良Ｏｋａｄａ手术治疗女婴肛门直肠畸形褚先秋用改良Ｏｋａｄａ手术治疗女婴肛门直肠畸形８例。年龄８个月～３岁。７例为肛门闭锁合并直肠前庭瘘，１例合并直肠阴道瘘。经Ｘ线瘘管造影显示直肠盲端距离肛门窝为２．０～２．５ｃｍ者６例，３．０ｃｍ者２例；瘘管长１．...     

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）。     

张力－应力定律在肛门直肠狭窄治疗中的应用──一种非手术治疗的新方法

利用张力－应力原理，采用球囊持续扩张方法，治疗肛门直肠狭窄患儿１３例，其中先天性肛门狭窄２例，肛门成形术后肛门狭窄５例，先天性直肠狭窄１例，术后或外伤性直肠狭窄４例，先天性肛门直肠狭窄１例。狭窄段长度０．６～１．０ｃｍ４例，１．２～５．０ｃｍ９例。８例曾经扩肛器扩肛失败。持续球囊扩肛每天２０小时，扩张压力在狭窄部静息压力曲线的迅速上升期范围。１３例中１２例成功，即排便通畅，肛检及球囊造影示狭窄段消失。所需时间５～１５天，平均６．７天。１例无效。随访６～１８个月无复发者。球囊持续扩张为治疗小儿肛门直肠狭窄的可靠方法，特别适用于狭窄段长和常规扩肛器扩张治疗失败的患儿。     

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     

支气管肺炎合并心力衰竭的发病机理研究

目的探讨支气管肺炎合并心力衰竭（简称心衰）的发病机理。方法对１５例肺炎合并心力衰竭患儿和１７例肺炎患儿及１７例正常健康小儿应用超声心动图检查测定左室收缩末室壁应力、射血分数（ＥＦ）、缩短分数（ＦＳ）和心率矫正之左室周径平均缩短速度（ｍＶｃｆｃ）；测定血管紧张素Ⅱ（ＡⅡ）和红细胞内游离钙水平。结果左室收缩末室壁应力：支气管肺炎合并心衰组（４９±６ｇ／ｃｍ２）较肺炎组（４２±９ｇ／ｃｍ２）和正常对照组（４１±８ｇ／ｃｍ２）增加；ＡⅡ：支气管肺炎合并心衰组（３６６±１６０ｎｇ／Ｌ）较肺炎组（５６±１６ｎｇ／Ｌ）和正常组（３８±８ｎｇ／Ｌ）增高；红细胞内游离钙水平：支气管肺炎合并心衰组Ｆ３３５／Ｆ３８５（１．９０±０．２８）和肺炎组Ｆ３３５／Ｆ３８５（１．８６±０．２６）较正常组Ｆ３３５／Ｆ３８５（１．６６±０．２４）增加；ＥＦ、ＦＳ和ｍＶｃｆｃ无改变。结论支气管肺炎合并心衰无心肌收缩力下降，而ＡⅡ增加、心脏后负荷增加。它们在支气管肺炎合并心衰的发病中起到重要作用。     

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.