外展推移截骨术治疗先天性髋内翻

目的：分析转子间外展推移截骨术治疗先天性髋内翻的远期疗效。方法：用转子间外展推移截骨术治疗先天性髋内翻２０例（２７髋），平均随访１４年。结果：所有股骨头骺板早期闭合，大转子上移，大部分髋臼发育不良，股骨头球形适应改变，肢体短缩。结论：手术可以使股骨头骺板处在正常位置上，但不能使股骨头骺板内部异常生长得到控制。股骨头骺板的早期闭合是骺板损伤后修复的结局，它维持头干角的稳定并引发周围形态的畸形     

改良Chiari滑膜切除术治疗Perthes病手术前后股骨头及髋…

对５３例Ｐｅｒｔｈｅｓ病患儿手术前后髋关节Ｘ线片进行测量，以同期自身健侧股骨头及髋臼大小，形状为对照进行比较分析。结果显示：术前患侧股骨头直径增大，骺高降低，形状变扁，且有半脱位倾向。术后新形态的髋臼能完好覆盖增大的股骨头，并且术后股骨头变圆，骺高增加，逐渐接近健侧股骨头大小和形状。     

改良Chiari滑膜切除术治疗Perthes病手术前后股骨头及髋臼演变

对５３例Ｐｅｒｔｈｅｓ病患儿手术前后髋关节Ｘ线片进行测量，以同期自身健侧股骨头及髋臼大小、形状为对照进行比较分析。结果显示：术前患侧股骨头直径增大，骺高降低，形状变扁，且有半脱位倾向。术后新形成的髋臼能完好覆盖增大的股骨头，并且术后股骨头变圆，骺高增加，逐渐接近健侧股骨头大小和形状。     

骨盆三联截骨术治疗儿童股骨头缺血性坏死的疗效分析

目的评价骨盆三联截骨手术对儿童股骨头缺血性坏死的中远期疗效。方法回顾性分析2005年至2012年在我科采取骨盆三联截骨术治疗的儿童股骨头缺血性坏死(Legg-Calve-Perthes Disease,LCPD)患者临床资料,共39例,男29例,女10例。年龄6~12岁,平均8.2岁。术前进行Herring分型,术后3年进行Stulberg分型以及测量CE角、Sharp角、AI值、股骨头挤压指数、双下肢长度差值来评价手术疗效。结果患者手术后随访3~10年,平均5.3年。术前Herring分型:B型18例,B/C型13例,C型8例。术后38例CE角、Sharp角、AI值、股骨头挤压指数显示股骨头获得了良好的包容。Herring分型为B型、B/C型、C型的患者术后Stulberg分型优良率分别为94.44%、76.92%、50%,各Herring型患者术后Stulberg分型差异有统计学意义(P0.05)。术后双下肢长度差值:Herring B型(6.94±2.69)mm,B/C型(10.46±3.45)mm,C型(14.87±4.94)mm,各型(包括2例C型术后长度差20 mm)术后双下肢长度差值有统计学意义(P0.05)。结论骨盆三联截骨术治疗Herring B、B/C与C型LCPD患者,可使股骨头获得良好包容,有利于髋关节功能的恢复,并能减少术后跛行的发生,是改善儿童股骨头缺血性坏死的中远期功能以及减少残留畸形的有效治疗方式。     

牵引加原位穿针(钉)治疗儿童股骨头骺滑脱

目的 研究改良Russell牵引加原位穿针(钉)在治疗儿童股骨头骺滑脱中的应用.方法 回顾1995年8月至2006年8月,我科收治并获得随访的SCFE病例 42 例,其中男 30 例,女12例.所有患儿术前均行改良Russell牵引,评价该牵引方法 对股骨头骺复位的作用,然后行原位穿针(钉)术,术后对患儿进行随访,观察该治疗方法 对患儿髋关节功能的影响情况.结果 Russell牵引可明显改善股骨头骺脱位情况,42 例SCFE患儿中,采用牵引加原位穿针(钉)固定方法 治疗的共 33例,其中除 2 例患儿牵引无效及术后髋关节功能恢复差之外,其余患儿均得到满意效果.结论 改良Russell牵引加原位穿针(钉)治疗可最大限度保留SCFE患儿髋关节功能,临床疗效满意.     

先天性髋关节脱位闭合复位的治疗体会

目的总结1994年1月￣2004年12月采用闭合复位治疗的先天性髋关节脱位262例(316髋)的疗效,探讨其影响因素。方法于全麻下行内收肌松解,手法复位,蛙式位或人体位支架固定,复位后定期作X线检查。结果262例患儿平均随访5年3个月(2￣8.5年),优良率为93%。复位后因髋臼发育不良仍有半脱位的13髋经再次手术痊愈。26髋发生股骨头骨骺发育不良(即无菌性坏死),占8%,其中14个髋术前股骨头骺未出现。结论先天性髋关节脱位早期治疗是成功的关键,闭合复位后支架固定是减少股骨头坏死的重要因素。     

梯形外展截骨术治疗儿童严重髋内翻

目的介绍一种自行设计的梯形外展截骨术治疗儿童严重髋内翻.方法1985～2000年收治19例患儿,共23髋采用此术式治疗.结果19例患儿获随访,平均为3年5个月,畸形基本矫正,HE角接近正常,骺板线趋近水平,平均8°;颈干角平均为129°,髋关节活动自如,未见复发病例.结论梯形外展截骨术是治疗儿童严重髋内翻的有效方法,具有操作简单、手术打击小、时间短、避免了内植入物的弊病等优点,符合股骨头部的生物力学规律,并强调了早期手术和应用HE角评价术后效果的重要性.     

3DCT辅助Dega截骨术治疗脑瘫患儿髋关节脱位的短期疗效分析

目的 通过3DCT模拟手术预测治疗效果,探讨Dega截骨术在脑瘫患儿髋关节脱位治疗中的应用和短期疗效.方法 收治8例发生髋关节脱位的脑瘫患儿共11髋,其中4髋半脱位,7髋完全脱位;手术时平均年龄为7岁8个月,术前均行骨盆-双股骨全长3DCT扫描并模拟手术,据此行Dega截骨术;评价手术前后肢体功能、步态的改善和髋关节发育状况;评价术后髋臼覆盖的变化和头臼的形态学匹配.结果 术后平均随访24个月.手术时Y形软骨未闭的7例9髋,髋臼指数(AI)术前(38±7)°,术后(12±6)°,平均减少26°;手术时Y形软骨闭合的1例2髋,Sharp角术前平均(51±1)°,术后(45±2)°,平均减少6°;所有患儿中心边缘角(CEA)术前(-9±18)°,术后(20±6)°,平均增加29°;股骨头偏移百分比(MP)术前(63±22)％,术后(19±6)％,平均改善44％;手术前后AI、CEA和MP的差异有统计学意义(P＜0.01).术前所有患儿Shenton线均不连续,术后均恢复连续性.根据改良Severin影像学分类,Ⅰ型4髋(36％)为优,Ⅱ型5髋(45％)为良,Ⅲ型2髋(18％)为可,优良率82％.手术时Y形软骨未闭合的7例9髋头臼形态匹配良好,关节包容满意;手术时Y形软骨已闭合的1例2髋头臼匹配良好无半脱位,但残留关节包容不良.术前GMFCS分级Ⅱ、Ⅲ、Ⅳ级各2、3、3例,术后评级Ⅰ、Ⅱ、Ⅲ级各1、4、3例.其中1例手术前后均为Ⅲ级,但其髋关节术前伸直、外展受限明显,术后活动范围已接近正常;1例手术前后均为Ⅱ级,但髋关节活动较前好转,跛行改善明显,步态趋于稳定.全部8例11髋术后髋关节功能改善满意,步态均较术前稳定.结论 3DCT可以直接观察脑瘫患儿髋关节脱位的骨性病理改变并模拟手术以指导截骨操作,以之辅助Dega截骨术治疗Y形软骨闭合前的脑瘫患儿髋关节脱位,短期效果良好,影像学及关节功能均改善满意;但用于Y形软骨闭合后的病例,尚需充足的数据以评价其疗效.     

儿童移位型股骨颈骨折16例临床分析

目的 评估16例采用切开复位、髋关节减压及内固定治疗的移位股骨颈骨折患儿的中期临床效果.方法 本院近期采用减压、切开复位及内固定治疗移位股骨颈骨折患儿16例,其中男10例,女6例,平均年龄9.5（4～15）岁.按照Delbet分型原则,Ⅰ型（股骨头骺滑脱）2例,Ⅱ型（经颈型骨折）5例,Ⅲ型（颈基底型骨折）9例;受伤原因：3例摩托车车祸伤,8例高处坠落伤,2例滑雪伤,1例自行车摔伤,1例卡车车祸伤,1例滑冰伤.方法 为切开复位、髋关节囊小切口减压,直视下解剖复位,应用加压螺钉或克氏针内固定治疗.结果 16例患儿均获随访,平均随访时间3.6年,髋关节X线片评估复位效果：8例为优,5例为良,3例为一般;14例应用加压螺钉固定,2例行克氏针固定.最后1次髋关节X线片随访提示1例术后股骨头骺早闭,3例合并股骨头坏死;13例髋关节功能良好,日常生活无明显影响.结论 移位股骨颈骨折患儿采取小切口髋关节内减压、解剖复位及坚强内固定,可减少股骨头坏死等并发症的发生,中期随访疗效满意.但Delbet分型Ⅰ型骨折的患儿术后股骨头坏死的发生率较高,预后差.     

Salter骨盆截骨术治疗学步期发育性髋关节脱位

目的介绍Salter骨盆截骨术在学步期发育性髋关节脱位（DDH）患儿中的治疗指征和手术要点，探讨Salter骨盆截骨术早期干预“学步期”DDH患儿的重要意义。方法2002年12月-2005年12月，采用Salter骨盆截骨术治疗DDH患儿29例，3例为多关节挛缩。男8例，女21例；年龄12～18个月，平均16．59个月；双侧12例，左侧11例，右侧6例。共计手术31髋，全脱位25髋，半脱位6髋。所有病例完善术前检查后不作牵引直接行Salter骨盆截骨术。术后髋人字石膏固定2个月，双下肢皮肤牵引，床上关节活动1个月后下地负重行走。结果所有病例术后2个月，6个月，1年，之后每年1次连续随访。随访6442个月，平均21．76个月。X线疗效按照Severin分级：Ⅰ级28髋，Ⅱ级3髋，Ⅲ级0髋。临床疗效优28髋，良3髋，可0髋，差0髋。结论根据国外的报道和我们的经验，对12～18个月处于“学步期”年龄段的全脱位和严重半脱位类型的DDH患儿采用Salter骨盆截骨术与保守治疗相比，可以在确保髋关节有效复位的同时，降低股骨头缺血坏死的发生率，避免二次手术，缩短疗程，对多关节挛缩髋脱位也具有良好的疗效，是一种安全、有效的手术方法。     

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.     

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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     

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.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.