一期后路半椎体切除短节段内固定治疗先天性脊柱侧凸

目的 探讨一期后路半椎体切除、短节段内固定手术治疗小儿先天性脊柱侧凸的临床疗效.方法 2004年9月至2009年6月,作者采用一期后路半椎体切除,短节段经椎弓根钉棒系统或椎板钩棒系统内固定手术治疗因半椎体所致的先大性脊柱侧凸患儿31例,男19例,女12例,年龄1～8岁,平均年龄3.6岁,术后支具固定4～6个月.术前站立位X线片显示单个半椎体28例,2个以上3例;半椎体位于胸段4例,胸腰段21例,腰段6例;Cobb's角侧凸平均42°(36°～64°),后凸39°(31°～58°).结果 经平均2.8年(6个月至4年)随访,无一例出现神经系统并发症,术中出现2例椎弓根螺钉切割椎体现象,采取更换固定上或下一椎体处理.术后冠状面矫正18°～37°,平均矫正25°,平均矫正率69.4%;矢状面矫正16°～38°,平均矫正24.5°,平均矫正率62.8%.未出现矫正度丢失或"曲轴现象".结论 对小年龄脊柱半椎体畸形患儿进行早期后路切除并短节段内固定,不仅可以矫正和控制脊柱畸形的发展,而且还能最大限度地保留脊柱的生长潜能和运动节段,疗效可靠.     

青少年伴发脊柱侧凸的Chiari畸形的治疗策略

目的　探讨伴发脊柱侧凸的Chiari畸形的临床评估及对其治疗效果进行初步分析。方法　对 32例伴发脊柱侧凸的Chiari畸形的青少年患儿进行枕大孔扩大减压成形手术治疗 ,同时 12例患儿施行了枕颈融合 ,后期 2 0例患儿未行枕颈融合。本组中 2 2例伴发的脊柱侧凸有矫形手术指征 ,在枕颈部手术后 6个月再行脊柱侧凸矫形术。结果　本组随访 6个月～ 5年 ,患儿的感觉障碍及腹壁浅反射减弱或消失的症状改善不明显 ;6例获得MRI复查患儿中 4例脊髓空洞明显缩小。 2 2例行脊柱侧凸矫形术患儿 ,术后平均Cobb角矫形率为 6 2 % ,终末随访时矫正率丢失 5 % ,10例行脊柱侧凸Milwaukee支具的患儿中有 2例脊柱侧凸有改善。结论　作为脊柱侧凸治疗的准备性手术 ,对Chiari畸形需行枕大孔扩大成形术 ,减少脊柱侧凸矫形时可能出现的神经损害。在脊髓空洞的处理及枕颈部融合上 ,在脊髓空洞与脊髓之比 <5 0 %时 ,不需行空洞引流术。对于患儿 ,如仅行枕大孔扩大减压 ,C1后弓切除 ,可以不行枕颈融合。     

前路Halm-Zielke器械治疗脊柱侧凸

目的　应用Halm Zielke双棒系统前路手术矫正脊柱侧凸。方法　 1999年 6月～ 2 0 0 0年 11月应用Halm Zielke系统治疗胸腰段和腰段特发性脊柱侧凸 10例 ,皆为女性 ,年龄 12 .5～ 18.4岁 ,平均 15 .7岁 ,全部病例均获随访。结果　术前侧凸Cobb’s角平均 5 7.1°,术后 14 .2° ,矫正率为75 .1%。旋转畸形 (Nash Moe法 )术前平均 2 .3° ,术后 0 .6° ,平均矫正 1.7°。除 1例轻度泌尿道感染外 ,无脊髓神经等其他并发症。结论　前路Halm Zielke手术矫正脊柱侧凸疗效确切 ,植入物稳定性好 ,并发症少 ,术后无须支架外固定     

脊柱前后入路治疗先天性脊柱侧凸

为了提高手术治疗小儿先天性脊柱侧凸的矫正率，减少术后畸形复发和曲轴现象的发生，采用脊柱前后入路手术治疗小儿先天性脊柱侧凸１５例。平均手术年龄９岁，Ｃｏｂｂ角平均８０°，平均随访４年。与同期２５例因先天性脊柱侧凸行单纯脊柱后路矫形术患儿进行术后平均矫正率、矫正丢失度／年、有无曲轴现象等临床指标比较。结果显示脊柱前后入路组治疗效果明显优于单纯脊柱后路组。对患有严重脊柱侧凸、弧度僵硬或并发后凸的患儿，宜行脊柱前后入路手术，对已行单纯脊柱后路矫形的患儿，应定期随访，如手术失败或畸形复发，有必要再次行脊柱前后入路矫形融合术。     

后路交叉置钉技术在青少年脊柱侧凸治疗中的应用

目的探讨后路交叉置钉技术对于治疗青少年脊柱侧凸的临床疗效。方法 2010年1月至2014年1月,68例青少年脊柱侧凸病例,男10例,女58例,年龄11~16岁,平均13.5岁;其中4例为神经肌肉型脊柱侧凸,其余为特发性脊柱侧凸;术前冠状面主弯Cobb角平均56.2°,胸椎(T5~T12)后凸角度平均17.7°,手术方法为后路交叉置入椎弓根螺钉对脊柱侧凸进行矫正,对术后Cobb角、主弯Cobb角矫正率结果进行评价。结果术后随访12~40个月,平均22.5个月。术后主弯Cobb角矫正到18.5°,与术前比较有差异(t=3.705,P0.01),末次随访时平均20.2°,与术后比无差异(t=1.053,P0.05)。胸椎(T5~T12)后凸角术后平均22.8°,末次随访时平均23.2°,术前与术后、术后与末次随访相比较均无差异(P0.05)。术后主弯Cobb角矫正率为70.5%,而末次随访时虽然主弯矫正率(69.4%)有一定丢失,但和术后比较无差异(t=0.126,P0.05)。术后无患者出现脊髓或神经根损伤。末次随访时无内固定松动及断钉断棒,植骨融合牢固,均未出现明显的矫正丢失。结论对于青少年脊柱侧凸后路手术矫正,采用交叉置钉技术是一项值得推广的低费用、高安全的脊柱侧凸矫正技术。     

先天性脊柱侧凸的外科治疗

目的　探讨先天性脊柱侧凸外科治疗的时机和方法。方法　 1993年 2月至 2 0 0 0年 12月 ,共手术治疗先天性脊柱侧凸 16例 ,其中男 4例 ,女 12例 ,年龄 2 .5～ 15岁 ,平均 12 .5岁。其中先天性半椎体 8例 ,分节不良 5例 ,混合型 3例。术前Cobb’s角 35°～ 80° ,平均 6 0 .5°。治疗方法有Ⅰ期前后路半椎体切除并后路内固定脊柱融合术 8例 ,单纯后路内固定脊柱融合术 4例 ,分次后路撑开不融合 4例。结果　平均随访时间 3年 8个月 ,术后Cobb’s角 15°～ 4 0° ,平均 30 .2°,平均矫正率 5 0 .1%。并发症包括椎板、椎弓骨折 2例、脱钩 2例、假关节 1例。无神经系统并发症。结论　应根据不同年龄和不同类型对先天性脊柱侧凸进行不同的早期手术治疗     

改良Cheneau支具治疗青少年特发性脊柱侧凸的疗效分析

目的　评价改良Cheneau支具治疗青少年特发性脊柱侧凸的临床效果。方法　观察自1996年 5月至 2 0 0 1年 6月使用改良Cheneau支具治疗并得到随访的 15 9例AIS患儿 ,其中男 6 8例 ,女 91例 ,年龄 7.2～ 16 .4岁 ,平均 10 .3岁 ,随访时间 2 4～ 4 8个月 ,平均 2 8个月。分别记录支具治疗前、治疗后 6个月、治疗后 1年、治疗后 2年的Cobb角、顶椎偏离中线距离、顶椎旋转程度和躯干位移等观察指标 ,评价支具治疗效果。结果　治疗前脊柱侧凸的Cobb角平均为 39.7° ,支具治疗 2年后Cobb角平均为 2 6 .8° ,矫正率为 35 .0 % ,二者差异有显著性意义 (P <0 .0 1)。支具治疗后顶椎偏离中线距离、顶椎旋转程度和躯干位移等观察指标亦有明显改善 (P <0 .0 1)。除 2例治疗失败外 ,其他患儿侧凸畸形均得到显著改善 ,无明显并发症。结论　改良Cheneau支具治疗青少年特发性脊柱侧凸可以取得满意疗效 ,正确的临床应用能有效避免手术治疗。     

应用C－D器械治疗34例脊柱侧凸初步报告

为了提高脊柱侧凸的疗效，应用ＣＤ器械治疗３４例脊柱侧凸。３４例中男１１例，女２３例。特发性脊柱侧凸２５例，先天性７例，小儿麻痹后遗症２例。术前Ｃｏｂｂ角为４０～１３０°，平均７２．８°。手术矫正后Ｃｏｂｂ角平均２８．９°，下降了４３．９°，矫正率６０．３％。严重并发症１例为术后瘫痪，６个月后基本康复；另１例为术后１周出现急性呼吸道感染合并衰竭，经抢救痊愈；余患儿术后１２天内均下床活动。ＣＤ器械能提供三维矫正，具有优良的反旋转作用和可靠的固定，脊柱融合节段少，患儿能早期下床等优点，值得推广应用。     

儿童腰骶部半椎体一期后路切除短节段固定治疗效果分析

目的评价儿童先天性腰骶部畸形一期后路半椎体切除、短节段固定治疗的手术效果。方法回顾性分析2014年1月至2017年12月首都医科大学附属北京儿童医院诊治的21例腰骶部半椎体患儿的临床资料,均行腰骶部一期后路半椎体切除术以及短节段固定融合术,随访至少24个月。术前、术后及末次随访时患儿均行站立位全脊柱正侧位X线检查。对比术前、术后以及末次随访时结构性侧凸、近端代偿弯、胸椎后凸、腰椎前凸的Cobb角、躯干偏移、矢状面平衡以及骨盆入射角、骨盆倾斜角、骶骨倾斜角。结果 21例中男12例,女9例;手术年龄2.17～13.00岁,平均(6.50±3.22)岁;术后随访24～84个月,平均(48.10±17.72)个月;融合固定2～4个椎体,平均(2.67±0.91)个椎体;手术时间120～300 min,平均(168.57±46.18) min;术中出血量100～1 000 m L,平均(368.04±234.58)m L;侧凸Cobb角由术前的(28.9±6.3)°降至术后的(8.5±3.0)°,末次随访时为(7.0±3.4)°,矫正率为75.8%。冠状面近端代偿弯Cobb角由术前(25.8±11.7)°自发性矫正为末次随访时(13.7±8.3)°,矫正率为46.9%。术后躯干偏移较术前均有明显改善,末次随访冠状面及矢状面的矫正率分别为53.1%和56.3%,且随访过程中躯干趋于平衡和稳定。所有病例术前、术后脊柱-骨盆序列均保持平衡,术后无脊髓神经功能损伤、感染等并发症。结论儿童腰骶部半椎体畸形一期后路半椎体切除、短节段固定可获得较好的侧凸矫正并改善躯干偏移,同时可保留一定的活动节段,有效控制畸形的加重和近端代偿弯的进展,避免腰椎长节段的固定融合。     

ISOLA器械治疗小儿脊柱侧凸

目的定期随访评价ISOLA器械治疗小儿脊柱侧凸.方法41例小儿脊柱侧凸患儿,特发性脊柱侧凸32例,神经肌肉性脊柱侧凸5例,其他类型的脊柱侧凸4例,术前、术中、术后均摄X线片,记录Cobb角,顶椎的移位及旋转,躯干偏离中线程度.结果Cobb角术前平均为66°,术后1年为36°,术后2年为36°.1年后,所有患儿的脊柱均完全融合.顶椎的偏离改善了14mm.躯干偏离改善了5mm.特发性脊柱侧凸患儿1年后有基本的正常活动.术后4例出现并发症,1例深部感染,1例骶尾部褥疮,1例横钩脱落,1例L3椎弓根螺丝钉脱落.结论目前有多种后路器械治疗小儿脊柱侧凸.严格掌握手术指征及ISOLA的原理,该术式能获得较为满意的疗效.     

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.     

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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.