应用Ilizarov技术治疗儿童复发性僵硬型先天性马蹄内翻足

目的探讨应用Ilizarov技术治疗复发性僵硬型先天性马蹄内翻足(Congenital Clubfoot,CCF)的方法与临床疗效。方法自2008年1月至2015年12月,应用Ilizarov技术治疗29例45足复发性僵硬型CCF,依据Dimeglio分型,均为Ⅲ、Ⅳ型,将连接于胫骨、跟骨,跖骨的Ilizarov外固定环互相连接、组合成复杂的三维外固定器,通过四维调节(三维空间加一维时间),逐渐矫正畸形,从而使患足达到或接近正常足的外形和功能。其中,31足单纯安装Ilizarov外固定牵伸器,9足结合经皮跟腱延长,7足结合中跗骨截骨,3足结合后足的V形截骨。术后7天开始矫正,速度1 mm/d,分4次完成,踝关节矫正至背伸约10°,后足轻度外翻后,停止矫形。矫正位带外固定器负重或保护下行走4周,拆除外固定器短腿管型石膏固定6~8周,拆石膏后夜间支具维持矫形3年。结果所有患者均获随访,随访时间11个月至6年,平均39个月。根据国际马蹄足畸形研究会(ICFSG)的评分系统,优23足,良18足,可3足,差1足,优良率91%。差的1足为DimeglioⅣ型,单纯应用Ilizarov外固定牵伸器治疗,矫形后步态改善,但遗留部分跟骨内翻畸形,2年后畸形明显复发,行三关节融合术治愈。结论应用llizarov外固定器三维矫正马蹄内翻足畸形,安全、微创、疗效确实,尤其适用于大年龄儿童之复发性僵硬型马蹄内翻足,有一定的临床应用价值。     

距下完全松解术治疗重度先天性马蹄内翻足的体会

目的：推荐治疗重度先天性马蹄内翻足的新的手术方法，以提高疗效。方法：距下完全松解术治疗重度先天性马蹄内翻足１４例２４足。１４例均为男性，平均年龄２２个月，最小年龄６个月，最大４岁。１０例为双侧足畸形，４例为单足，其中３例为左侧，１例为右侧。术后平均随访３年９个月，最短为１３个月，最长为５年。结果：畸形完全纠正为１９足占７９．１％，畸形复发４足占１６．６％，１足并发足外翻畸形占４．１％，１足局部皮肤坏死占４．１％。结论：距下完全松解术，跟骨与距骨之间得到充分旋转复位，而使畸形得到较好的纠正。     

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     

小儿严重外伤性马蹄内翻足治疗的探讨

介绍Ｍｃｋａｙ手术加隐动脉皮瓣治疗小儿严重外伤性马蹄内翻足的临床应用情况，本组按Ｍｃｋａｙ原则矫正马蹄内翻足畸形，同时采用带血管蒂岛状隐动脉皮瓣，交腿移植修复踝跟部皮肤缺损创面，共治疗５例。术后随访，踝跟部外观满意，无片状疲痕或溃疡，踝关节背伸１０～２０，跖屈１５～２５，内、外踝连线与足底纵轴线夹角为８０～９０。评分优４例，良１例。该术式对矫正小儿严重外伤性马蹄内翻足踝部畸形，恢复其功能具有重要作用。     

早期肌力平衡手术治疗先天性马蹄内翻足的远期疗效

报告并评价早期肌力平衡手术治疗先天性马蹄内翻足的远期疗效。１９５７～１９９４年采用早期矫正畸形建立肌力平衡手术治疗３个月～１２岁儿童先天性马蹄内翻足１０８８例１５２１足。采用自行制定的疗效评定标准，对术后６～３６年（平均１１年６个月）、随访时年龄１３～４０岁的１２８例１８８足（其中年龄在１８岁以上５２例７４足）的远期疗效进行评价。总优良率８９．４％，骨骼完全成熟患儿的优良率８９．２％。手术疗效与足踝部主要肌肉功能、术前足畸形程度和手术时年龄均有明显关系（Ｐ＜０．０５）。手术操作不当也可影响手术疗效。早期矫正畸形并建立动态肌力平衡手术是治疗儿童先天性马蹄内翻足的有效方法，能取得外观及功能满意的远期疗效。     

张应力原理及四维相矫治重症马蹄内翻足畸形

张应力原理及四维相矫治重症马蹄内翻足畸形赵黎陈拱治黄耀添综述对于严重的马蹄内翻足畸形，主张手术治疗［１～３］。但经手术治疗的患儿中有约２５％（１３％～５０％）效果不佳［４］，因其在６～７岁之前易复发。当前的主要难题是难治性、复发性和延误处理的马蹄内翻...     

先天性马蹄内翻足足骨发育异常X线表现

目的:研究马蹄足足骨生长发育特点，为手术治疗和临床研究提供解剖学依据，探讨治疗原则。方法:对我院1997年以前收治的单侧先天性马蹄内翻足患儿双足摄X线片和CT三维重建图像，了解其畸形组成、足骨发育，与健侧比较，从而探讨维持畸形的解剖学、力学因素。结果:患足中足及跟距骨均有生长发育异常，骨关节改变，力线改变，促进或参与了畸形发生、畸形保留。结论:了解先天性马蹄内翻足病理解剖结构，分析畸形维持原因，为尽早手术治疗提供依据，为临床研究提供足骨生长发育的特点。规范手术治疗原则。     

儿童复发性马蹄内翻足的定位诊断与外科治疗的选择

目的 探讨复发性马蹄内翻足的定位诊断问题,为选择针对性的矫形手术方法提供依据.方法 回顾性分析自2002年以来治疗的复发性马蹄内翻足18例26足,按Davidson方法分成三组:A组为单纯性前足内收畸形(8例10足);B组为前足内收并中足旋后复合畸形(蚕豆足畸形,8例13足);C组为后足内翻合并前足内收畸形(2例3足).A组畸形采用胫前肌腱外移术或胫前肌腱外移术+骰骨外侧闭合性楔形截骨术矫形,B组畸形采用中跗骨联合截骨矫形,C组畸形采用跟骨外移截骨+软组织松解+胫前肌腱外移,或加Ilizarov外固定器逐渐矫形.结果 本组18例26足术后平均随访时间为30个月(18～70个月),其中随访时间超过2年为16例23足.按照国际马蹄内翻足研究会评定标准(ICFSG)评价手术效果,优8例10足,良6例10足,可1例2足,差1例1足.其中评定为可和差的2例3足均为C组病例,虽然其后足内翻合并前足内收畸形获得比较满意的矫正,但距骨滑车畸形引起的胫距、距下关节僵硬并未解决,是导致评分过低的主要因素.结论 儿童复发性马蹄内翻足的术前定位诊断与矫形结果有较强的相关性.依照术前定位诊断确定的畸形类型,选择针对性的矫形手术方法,才能实现满意的矫形效果.     

跟腱延长前移术治疗脑性瘫痪痉挛型马蹄足的疗效

目的评价跟腱延长前移术治疗儿童脑性瘫痪痉挛型马蹄足的疗效。方法收集1998年5月-2011年6月应用跟腱延长前移术治疗脑性瘫痪痉挛型马蹄足儿童53例85足。男28例45足,女25例40足;年龄2.5～14.0岁,平均6.8岁。痉挛程度按Ashworth 5级法评定:3级12足,4级38足,5级35足。患儿均能行走,智力发育及下肢肌力基本正常,伴不同程度肌张力增高,腱反射亢进,踝阵挛和Babinski征阳性,无明显内翻、外翻及平足畸形,X线片未提示明显骨性畸形。合并双髋内收畸形5例,双膝屈曲畸形2例,均在术前或同期行手术矫正。结果患儿均获随访,随访时间0.5～11.2 a,平均2.3 a。1例于术后2周出现切口裂开、跟腱断裂,再次行跟腱吻合术,术后恢复良好;患儿术后均未发现小腿三头肌肌力较术前下降。53例85足均获满意疗效,优55足,良30足,优良率达100%。结论跟腱延长前移术利用生物力学原理,在跟腱延长的基础上将跟腱止点前移至跟距关节后缘,缩短了跟腱至踝关节的力臂,平衡了踝关节背伸与跖屈肌肌力,远期效果良好,是儿童脑性瘫痪痉挛型马蹄足较好的治疗方法。     

Ilizarov技术双段截骨矫治小儿肱内翻的研究

目的 通过手术矫正小儿肱骨内翻及短缩畸形以改善患儿上肢不等长和肩关节外展功能受限。方法 本组５例,１０－１７岁患儿采用一次多平面肱骨截骨,即在肱骨不同平面交叉穿过几组克氏针与预组装好的伊氏外固定器相连接,在肱骨外科颈下做外翻截骨后,使上端Ω环于矢状面向外下翻转纠正肱骨头干角。     

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.     

�¶�֢��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     

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.     

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   

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.     

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.     

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.