肱骨远端倒“V”形截骨治疗儿童肘内翻的疗效评价

目的介绍肱骨远端倒“V”形截骨治疗儿童肘内翻畸形的手术操作技术,并评价其近期疗效。方法2009年1月至2011年7月作者收治10例儿童肘内翻患儿,术前依照x线片测量肘内翻的严重程度,确定需要外翻截骨的角度,在肱骨远端实施倒“V”形截骨,实现所需矫正的角度为内翻角＋7°的正常提携角后,采取交叉克氏针固定,屈肘60°位石膏固定,6周后拆石膏并拔除克氏针,开始主动肘关节功能训练。采用临床外观、肘关节伸屈功能,x线片测量提携角的方法评价治疗结果。结果10例均获得随访,平均随访时间1．8年（1—2．5年）。患肢提携角接近正常,肘关节伸屈活动达到术前水平,无切口感染、血管神经损伤、矫形丢失、骨不愈合等并发症。术后平均10周截骨处均达骨性愈合。临床评价和x线评价结果均达到优良的标准。结论肱骨远端倒“V”形截骨治疗儿童肘内翻畸形,手术操作相对简单,矫形效果好,能有效恢复肱骨与尺骨的解剖轴线。     

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.     

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     

足中跗骨联合截骨治疗儿童蚕豆足畸形

目的 介绍足中跗骨联合截骨(外侧骰骨闭合性楔形截骨和内侧楔状骨开放性楔形截骨)治疗儿童复发性马蹄内翻足之蚕豆足畸形的手术方法,并初步评价其临床疗效.方法 自2004年至今,采用该方法治疗儿童复发性马蹄内翻足之蚕豆足畸形共24例28足,具体操作方法:足中跗骨联合截骨分别在足内、外侧作两个手术切口,Ⅰ期进行骰骨的闭合性楔形截骨(基底位于背外侧)与内侧楔状骨冠状面开放性楔形截骨(截骨点位于其中点).在骰骨截除楔形骨块后,将该楔形骨块嵌入内侧楔状骨截骨间隙内,产生前足外展、中足内旋和骰骨截骨间隙闭合的作用,并使用自制门形钉固定两处截骨.并对随访两年以上的14例16足从临床外观和放射学的角度测量评价该手术的临床疗效.结果 随访超过2年者总计为14例16足,其临床外观和放射学改变均有明显改善,跟骨-第四跖骨角从术前平均-32°改善到术后平均-16°,距骨-第一跖骨角从术前平均-18°改善到术后平均-4°,但有1足发生内侧楔状骨移植骨块移位,导致矫形丢失.结论 足中跗骨联合截骨是治疗儿童复发性马蹄内翻足(或称之为先天性马蹄内翻足治疗后的遗留畸形)之"蚕豆足"畸形的有效方法,其操作简单、安全,疗效肯定,且不需要更多的软组织松解,适用于4岁以上或内侧楔状骨已经良好骨化的患儿.     

肱骨远端截骨Ilizarov矫形与单纯楔形截骨克氏针固定矫治术治疗儿童肘内翻的对比研究

目的比较肱骨远端截骨Ilizarov矫形与单纯楔形截骨克氏针固定矫治术治疗儿童肘内翻的疗效及优缺点。方法收集2008年5月至2017年5月收治的34例肘内翻患儿作为研究对象,其中经肱骨远端截骨Ilizarov矫形术治疗儿童肘内翻16例(A组),男童13例,女童3例,左侧9例,右侧7例,年龄(7. 3±2. 1)岁;单纯楔形截骨克氏针固定矫治术治疗儿童肘内翻18例(B组),男童12例,女童6例,左侧7例,右侧11例,年龄(8. 2±1. 9)岁。术前两组患儿一般资料具有可比性,术后对两组患儿进行了15个月至8年2个月的随访,平均随访39个月。对两组患儿术中各项指标、术后并发症及术后疗效进行比较,肘关节功能按改良的Flynn标准进行评价。结果与肱骨远端截骨Ilizarov外固定术比较,单纯楔形截骨克氏针固定矫治术治疗肘内翻手术时间更短[(0. 9±0. 2) h vs.(1. 8±0. 3) h],完全愈合时间更短[(28±4. 5) d vs.(48. 5±3. 9) d],拆内、外固定耗时更短[(31. 4±5. 7) min vs.(1±0. 14) min)],但手术切口更大[(3. 8±1. 1) cm vs.(1. 5±0. 9) cm],术中出血量更多[(25. 4±2. 5) m L vs.(10. 4±1. 5) m L],差异均有统计学意义(P 0. 05)。肱骨远端截骨Ilizarov矫形组中有3例出现术后并发症,其中2例为神经挫伤,1例为针道感染,单纯楔形截骨克氏针固定矫治组中有4例术后出现尺神经挫伤,差异无统计学意义(χ2=0. 062,P 0. 05);两组预后优良率分别为93. 75%和94. 4%,差异无统计学意义(χ2=0. 007,P 0. 05)。经肱骨远端截骨Ilizarov矫形术治疗,术前提携角肘内翻(15. 2±2. 4)°,术后提携角外翻(11. 2±1. 5)°。经单纯楔形截骨克氏针固定治疗,术前提携角为内翻(16. 3±1. 9)°,术后为提携角外翻(10. 8±1. 7)°。两组手术前后提携角的纠正有统计学意义(P 0. 05)。结论肱骨远端截骨Ilizarov矫形术和单纯楔形截骨克氏针固定矫治术治疗儿童肘内翻的临床疗效相同;Ilizarov外固定在外观瘢痕、外侧髁异常膨大方面有优势;但单纯截骨克氏针固定矫治术在手术时间、截骨完全愈合时间、取内固定耗时、术后护理等方面有优势。     

足跖侧松解和跗骨V形截骨治疗儿童高弓足畸形

目的　介绍一种软组织和骨性联合手术治疗儿童高弓足畸形的方法 ,并评价其临床效果。方法　对因神经肌肉性疾病所致的高弓足 /高弓内翻足畸形 15例 19足和先天性马蹄内翻足术后遗留高弓足畸形 1例 1足 ,采取足跖侧软组织松解和足跗骨V形截骨联合手术的治疗方法 ,术后用小腿管型石膏固定 6周。主要从足外形的改善、足负重及行走功能和X线测量评价手术效果。结果　本组平均获得随访 3年 6个月。跗骨截骨均于术后 6～ 8周愈合。术后 10周开始负重和行走。除 3例高弓足畸形复发并后足内翻加重 ,17足外形、足负重及行走功能均明显改善 ,X线测量Meary角由术前平均 2 5°减少至 5°以下。本组优者 8足、良者 9足、差者 3足 ,优良率为 85 .0 %。结论　足跖侧软组织松解和足跗骨V形截骨联合手术是治疗儿童高弓足 /高弓内翻足畸形确实有效的方法 ,对大于 6岁儿童足的生长发育无明显的不良影响。但是 ,对进行性神经肌肉性病变所致的高弓足 /高弓内翻足畸形 ,则术后容易复发或畸形加重     

骨盆股骨短缩去旋转内翻截骨治疗先天性髋脱位

目的 探讨骨盆截骨结合股骨短缩去旋转内翻截骨治疗先天性髋关节脱位的疗效。方法 手术治疗先天性髋关节脱位15例16髋，全部均为女性，年龄平均4岁6个月(3岁～6岁6个月)，采用髋关节切开复位、骨盆截骨及股骨短缩去旋转内翻截骨术。结果 优13个髋，良3个髋，没有再脱位。X线片结果优12个髋，良4个髋，没有发生股骨头缺血性坏死。结论 骨盆截骨结合股骨短缩去旋转内翻截骨术治疗先天性髋关节脱位能够获得满意的结果。     

杵臼形截骨术治疗先天性髋内翻

杵臼形截骨术治疗先天性髋内翻胡嘉彦，隋承峰，金城壁先天性髓内翻均采用楔形截骨术治疗，术后需取出内固定物。笔者通过实践，提出股骨粗隆下柠臼形截骨术，无需再手术取钢板。手术经股骨上端外侧入路，显露股骨上端，分别经大粗隆至股骨干和小粗隆下股骨干至对侧皮质各...     

改良Pauwels'股骨转子间截骨术治疗儿童创伤后髋内翻

目的 评估改良Pauwels'股骨转子间截骨术在儿童创伤性髋内翻治疗中的作用.方法 作者近年来收治10例儿童创伤性髋内翻患儿,其中男7例、女3例,平均年龄11.2岁,采用改良Pauwels'股骨转子间截骨解剖钢板内固定术,比较术前术后颈干角、双侧肢体长度的变化.结果 10例患儿均获随访,平均随访时间3.5(3~5)年,...     

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.     

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.     

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.     

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.