股髓牵拉延长术治疗新生儿化脓性关节炎后严重短肢畸形

介绍一种治疗急性化脓性髋关节炎后合并下肢不等长超过５．０ｃｍ患儿的方法。本组共治疗４例蔡氏ⅣＢ型新生儿急性化脓性髋关节炎后遗症患儿，年龄７１３岁，     

一次性加压延长治疗先天性胫骨假关节

采用自制长骨外固定器及Ｉｌｉｚａｒｏｖ器械治疗先天性胫骨假关节，使假关节愈合及肢体短缩问题同时得到解决。本组４例先天性胫骨假关节，术前肢体短缩４．０～１０．５ｃｍ，通过一次性手术对假关节施行切除，加压固定，井应用Ｉｌｉｚａｒｏｖ器械对短缩肢体进行延长。假关节全部愈合，患肢延长４．０～１２．０ｃｍ。结论：该手术可使假关节的肢体短缩及愈合两大矛盾同时得到解决，恢复患儿行走功能。     

直肠灌注安定溶液的药代动力学研究

为了解安定直肠灌注后在儿童体内的代谢过程以及确定适宜的直肠给药部位，对８例５岁半～１３岁患儿分别在肛门内５ｃｍ和１０ｃｍ处灌注安定溶液，并观察血药浓度的变化及进行药代动力学分析。结果表明，安定的代谢符合二室开放模型，半衰期平均为３８±１１ｈ，峰浓度为６．５±０．３ｎｇ／Ｌ，清除率是０．０２４±０．００６Ｌ·ｋｇ－１·ｈ－１，表观分布容积为０．７２±０．０６Ｌ／ｋｇ。在肛门内５ｃｍ给药的４例，其血药浓度在３分钟时平均为３．４±２．３ｎｇ／Ｌ，７分钟时已达６．３±０．５ｎｇ／Ｌ，达峰浓度时间平均为０．３２±０．０９ｈ；而在肛门内１０ｃｍ处给药的另４例，在７分钟时的血药浓度平均为３．２±１．８ｎｇ／Ｌ，至３０分钟左右达５．０ｎｇ／Ｌ以上，其达峰浓度时间平均为１．１３±０．１１ｈ。提示造成两组安定吸收速率快慢差异的原因与直肠肛门血液循环的解剖生理特点有关，直肠灌注安定溶液时采用５ｃｍ左右的深度较为适宜。     

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。     

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。     

电视胸腔镜下手术治疗小儿动脉导管未闭

电视胸腔镜手术是９０年代兴起的新技术，本院１９９２年底开展此项手术，并取得良好效果，其中治疗先天性心脏病动脉导管未闭（ＰＤＡ）８例，占治疗总数的２０％，现报告如下。临床资料：患儿年龄２～１２岁，平均４岁。男５例，女３例。术前经彩超诊断为ＰＤＡ，直径０．３～１．０ｃｍ。管状５例，漏斗状３例。采用Ｗｕｌｆ电视内窥镜系统，进口钛钉及国产钽钉。手术要点：单腔静脉复合麻醉，右侧卧位，于腋中线、第三或四肋间为中点，分别做２～３个０．５～１．０ｃｍ皮肤切口，分别送人套针，应先注入２００～１０００ｍＩＣＯ２将肺…     

Pna手术及盆底肌肉折叠术在肛门闭锁术后大便失禁中的应用

目的　了解Ｐｅ～ｎａ 手术及盆底肌折叠术治疗无肛术后大便失禁的效果。方法　采用再次Ｐｅ～ｎａ 手术和盆底肌折叠术治疗高中位无肛畸形术后大便失禁患儿９ 例（ 平均年龄６ ．３ 岁）。术中见９ 例患儿肛门和直肠均未穿过横纹肌复合体的中心,其中４ 例新生儿期行腹会阴肛门成形术的患儿,直肠偏于复合体的左侧或右侧,另外５ 例直肠偏于复合体的前方。术中将直肠重新固定在横纹肌复合体之中心。结果　术后随访半年到两年,显示患儿的排便控制功能均有改善,３ 例术前临床评分为０ 者,术后增加到４ ～５ 分,２ 例术前１ 分者,术后增加到５ 分和６ 分,３ 例２ 分者增加到５ ～６ 分,另１ 例３ 分者增加到６ 分。肛门直肠测压显示：术前和术后肛管的静息压力变化不明显,而收缩压力增加显著,由术前的（３０ ．８±１６ ．４）ｍ ｍ Ｈｇ 增加到（５２ ．７ ±１１ ．１）ｍ ｍ Ｈｇ。结论　直肠错过横纹肌复合体中心是高中位肛门畸形患儿术后大便失禁的病因之一。再次行Ｐｅ～ｎａ 手术和盆底肌折叠术可以改善患儿的排便控制功能     

小儿贲门痉挛的外科治疗

手术治疗小儿贲门痉挛１３例。年龄５个月～１４岁。贲门狭窄最短２ｃｍ，最长４ｃｍ。行Ｈｅｌｌｅｒ术，切开贲门肌层４～５ｃｍ，胃底浆肌层１．５～２．０ｃｍ，３例加胃底折叠术。１２例术后恢复顺利，１例并发食管胸膜瘘。均治愈。术后近期吞咽困难和呕吐消失。获随访１１例，随访时间６个月～８年，９例症状消失，无复发，２例症状改善，进生冷食物或进食快时仍有呕吐。食管吞钡Ｘ线检查９例，７例通过顺利，无返流，１例有返流，１例贲门口仍有狭窄。Ｈｅｌｌｅｒ术治疗小儿贲门痉挛有较好的效果。     

胼胝体脂肪瘤合并脑膜膨出1例

胼胝体脂肪瘤合并脑膜膨出１例邵彤，刘学宽，李洛患儿：男，５岁。额部长一肿物增大半年。体检：智力低下，头围４９．８ｃｍ，额部中线皮下一软性肿物７．０ｃｍ×６．０ｃｍ×２．０ｃｍ，哭闹时稍增大，透光试验阴性。鼻根部增宽。Ｘ线片示额骨裂４．０ｃｍ×３．８ｃ...     

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）。     

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

�¶�֢��ϵ�ϰ���ע��ȱ�ݶද�ϰ������ڵ�ת��

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     

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.     

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.     

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.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.     

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.