桡骨杵臼截骨及尺骨头复位治疗MadeLung畸形

目的 探讨桡骨杵臼截骨及尺骨头复位治疗MadeLung畸形的初步疗效.方法 2002～2006年间应用桡骨远端杵臼截骨及尺骨头复位治疗MadeLung畸形4例,均为女性,左侧1例,右侧1例,双侧2例.术前腕关节疼痛,X线片显示桡骨远端关节面向掌侧倾斜75°～85°(平均80°).手术年龄12～13岁(平均12.5岁).结果 术后随诊1～4年(平均2年),腕关节疼痛全部消失,腕关节外观及活动均得到改善.1例出现尺骨截骨部位延期愈合.结论 桡骨杵臼截骨及尺骨头复位是治疗MadeLung畸形的有效方法之一.     

先天性桡骨发育不良的初步治疗体会

目的 介绍治疗先天性桡骨发育不良的初步体会.方法 2006年7月至2008年12月我院收治先天性桡骨发育不良的患儿7例,共8侧(其中一例为双侧累及).男6例,女1例,年龄最小5个月,最大5.5岁,平均2.5岁.除2例为Ⅲ型,其余均为Ⅳ型.此外1例伴发颈椎畸形,1例伴有马蹄足畸形,1例伴有髋关节发育不良.首先采用手法牵拉减缓软组织的挛缩,到1岁时进行手术,手术方法主要是采用尺骨腕关节中置术,即暴露腕关节,于近侧腕骨中心切一小槽,修剪尺骨远端,然后将其插入腕骨小槽中并用克氏针固定,同时对尺侧松弛的组织进行紧缩.在进行该项手术前先要对患儿前臂桡侧挛缩程度进行评估,若桡侧挛缩程度严重,则用ILIZAROV支架牵拉软组织后再行手术.手术后石膏同定4周,术后8周取出克氏针,术后需佩戴矫形支具.随访时间3～30个月,平均12个月.结果 所有患儿在手术后畸形立即得到明显纠正,外观得到明显改善,手术后患肢功能较前也有增进,肘关节活动增多.结论 尺骨腕关节中置术结合桡侧软组织牵拉,术后再辅以矫形支具的佩戴能达到比较满意的治疗效果.     

尺骨延长治疗儿童多发性骨软骨瘤所致前臂畸形26例

目的总结26例因多发性骨软骨瘤导致前臂畸形的手术治疗经验,探讨手术的安全性、疗效及注意事项。方法采用尺骨截骨Ilizarov技术和骨外固定器逐渐延长尺骨,矫正弯曲,同时牵引桡骨小头复位。在治疗过程中,始终进行腕关节、掌指关节和指间关节的屈伸功能锻炼,尤其是伸直功能锻炼。结果8例前臂短缩、弯曲得到矫正,肘内翻、腕关节尺偏纠正,外观满意。前臂旋转功能较术前改善,旋前增加10°-16°,旋后增加11°-14°。1例发生暂时性桡神经麻痹,1例发生肘关节功能障碍,2例低龄患儿出现指间关节屈曲,并发症的发生率为15％。结论采用尺骨延长术治疗多发性骨软骨瘤所致的前臂畸形具有改善外观和功能的作用。治疗期间应注意肘关节、腕关节、掌指关节和指问关节的康复训练,尤其是低龄儿童。     

先天性双侧桡侧球棒手畸形一例

患儿男,7天,因“先天性双侧前臂畸形伴双手拇指缺失,左手食指、中指融合”入院。患儿系第2胎第2产,足月剖宫产分娩,家中无类似病史。查体：双侧前臂短小弯曲,桡骨缺失,腕关节向尺骨桡侧完全脱位,手指与尺骨纵轴成角畸形,     

旋转截骨治疗先天性上桡尺关书连接

对８例１１个肢体先天性上桡尺关节连接畸形进行了桡尺骨旋转截骨和旋前肌松解术，获得满意的疗效。对手术适应证、手术方法进行了讨论，认为此手术方法尤其适用于严重前臂旋前畸形的病例。     

旋转截骨治疗先天性上桡尺关节连接

对８例１１个肢体先天性上桡尺关节连接畸形进行了桡尺骨旋转截骨和旋前肌松解术，获得满意的疗效。对手术适应证、手术方法进行了讨论，认为此手术方法尤其适用于严重前臂旋前畸形的病例。     

软组织重建治疗Wassel Ⅳ-D型复拇畸形

目的 探讨软组织重建治疗儿童Wassel Ⅳ-D复拇畸形的方法.方法 2007年10月至2011年3月对8例9手Wassel Ⅳ-D型复拇畸形通过软组织重建进行手术治疗,其中男5例6手,女3例3手.术中切除桡侧拇指,保留尺侧拇指,末节桡偏严重者,行桡侧关节囊松解,尺侧关节囊紧缩,指间关节和掌指关节用克氏针固定,掌骨头桡侧修整.A2滑车重建,屈拇长肌腱桡侧止点居中.随访时间4～33个月,平均18个月.结果 随访结果采用Tada等制定标准评价:优5例6指；良2例；差0例.家属对治疗效果均表示满意.结论 采用软组织重建治疗Wassel Ⅳ-D型复拇畸形,效果满意.     

桡骨栅栏状截骨及尺骨中段截骨治疗儿童先天性尺桡骨近端连接

目的 探讨桡骨远端栅栏状截骨及尺骨中段截骨对先天性尺桡骨近端连接的治疗效果.方法 2010年1月至2012年6月我们收治28例先天性尺桡骨近端骨性连接患儿,对其中18例实施尺骨中段截骨、克氏针内固定、桡骨远端栅栏状截骨手术治疗,术后予石膏固定6周,拔除克氏针,定期复查,随访11个月至3年.结果 先天性尺桡骨近端连接患儿经尺骨中段截骨、桡骨远端栅栏状截骨治疗,左侧前臂可达旋后10°～20°、右侧前臂可达旋前10°～20°,无矫形丧失现象,日常生活达到功能要求.结论 先天性尺桡骨近端连接采取桡骨远端栅栏状截骨及尺骨中段截骨治疗效果确实,操作可重复性强.     

先天性钩状拇指的手术治疗

目的 探讨先天性钩状拇指不同类型的手术治疗方法.方法 回顾分析山东大学齐鲁儿童医院小儿外科手术治疗的17例先天性钩状拇指的临床资料.17例均为双侧,共34手,年龄3～9岁(平均7岁9月);参照Weckesser分型.本组同一患儿双手分型相同,其中Ⅰ型9例(52.9%),Ⅱ型5例(29.4%),Ⅲ型3例(17.6%);Ⅰ型采用示指固有伸肌转移或桡侧腕长伸肌和掌长肌腱移植重建拇指伸肌功能;Ⅱ、Ⅲ型通过松解指蹼挛缩、掌指关节关节囊、背侧骨问筋膜及拇收肌横头第三掌骨止点.并采用示指同有伸肌或桡侧腕长伸肌、掌长肌腱转移重建伸肌功能等方法治疗.结果 随访7个月至15年,17例中11例(64.7%)优,3例(17.6%)良,2例(11.8%)中,1例(5.9%)差.结论 针对先天性钩状拇指的不同类型采用相应不同的手术方法治疗,治疗效果显著.     

手术治疗先天性三节指骨拇畸形

目的探讨先天性三节指骨拇畸形的手术治疗。方法本组患儿4例5指,男1例,女3例,手术年龄5个月至2岁。按Wood分型：I型2指,Ⅱ型3例。合并多指4指。I型采用直接切除多余指骨,Ⅱ型采用多余指骨截骨内固定,合并肌腱畸形者重建肌腱。结果4例患儿在门诊获得8个月至3年的随访,效果满意。结论根据分型,I型采用直接切除多余指骨,Ⅱ型采用多余指骨截骨内固定的方法是治疗儿童先天性三节指骨拇畸形的有效方法。     

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.     

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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.     

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.