An Upgrade of the International Hip Dysplasia Institute Classification for Developmental Dysplasia of the Hip

BackgroundThe purpose of the current study was to upgrade the International Hip Dysplasia Institute (IHDI) classification of developmental dysplasia of the hip (DDH).MethodsThe upgrading was suggested by adding the state of the acetabulum (type A for the normal acetabulum and type B for the dysplastic one). The pelvic radiographic films of 110 children suspected to have DDH were used by three observers to sort out the hips into grades according to the original form and the suggested upgraded one subsequently.ResultsThe interobserver reliability between the observers improved from a good level (intraclass correlation coefficient [ICC], 0.885; 95% confidence interval [CI], 0.856–0.909) with the original form to an excellent level (ICC, 0.919; 95% CI, 0.898–0.936) with the upgraded form. When the upgraded form was used, only the grade 1 hips were divided into types A and B, while those classified as grades 2, 3, and 4 were all graded as type B only.ConclusionsThe IHDI classification of DDH can be upgraded into grade 1A, grade 1B, grade 2, grade 3, and grade 4.     

发育性髋脱位闭合复位术前磁共振预判因素分析

目的探讨2岁以内发育性髋关节脱位(developmental dislocation of the hip,DDH)患儿术前磁共振相关因素对手术方式的预判。方法回顾性分析2013年7月至2017年2月收治的57例(60髋)符合条件的发育性髋关节脱位患儿资料,其中男9例,女48例。术中根据Bowen造影标准将患儿分为3组,闭合复位成功为A组(26例),闭合复位失败为B组(24例),无法复位为C组(7例)。在磁共振上测量三组患儿术前关节囊前入口角(anterior access angle,AAA)、下入口角(inferior access angle,IAA)、冠状位最大入口直径(coronal maximal access diameter,CMAD)、轴位最大入口直径(axial maximal access diameter,AMAD)、轴位股骨头直径(axial femoral head diameter,AFHD)、冠状位股骨头直径(coronal femoral head diameter,CFHD)、冠状位口径比(CMAD/AFHD)及轴位口径比(AMAD/CFHD)等指标,比较三组数据的差异有无统计学意义。Logistic多因素回归分析性别、侧别、年龄、脱位程度、负重时间等和治疗方式的相关性,受试者工作特征曲线(Receiver operating characteristic curve,ROC曲线)评估闭合复位组指标的灵敏度、特异度及拐点。结果3组数据的AAA、CFHD、AFHD等方面的差异无统计学意义(P>0.05)。A组IAA角为(108.2±9.8)°,显著大于B组(98.8±11.2)°和C组(91.7±6.9)°,三组差异具有统计学意义(P<0.05);A组与B组,A组与C组在CMAD、AMAD、CMAD/AFHD等的差异具有统计学意义(P<0.05)。A、B两组AMAD/CFHD的差异有统计学意义,A、C两组差异无统计学意义。B、C两组CMAD、AMAD、CMAD/AFHD、AMAD/CFHD的差异均无统计学意义(P>0.05)。根据Logistic多因素回归分析结果,性别、侧别、脱位程度、负重时间等和治疗方式的差异无统计学意义(P>0.05),年龄有相关性。A、B两组差异具有统计学意义的5组数据ROC曲线下最大面积为AMAD/CFHD(0.848),确定AMAD/CFHD的敏感度(74.1%)、特异度(92.3%)及拐点(0.515)。结论术前磁共振指标IAA、CMAD、AMAD、CMAD/AFHD、AMAD/CFHD可作为术前预判因素。当AMAD/CFHD比值>0.515时,建议行闭合复位治疗。     

Tracheostomy,respiratory support,and developmental outcomes in neonates with severe lung diseases: Retrospective study in one center

ObjectivesPediatric tracheostomy has evolved significantly in the past few decades and the optimal timing to perform it in children with respiratory assistance is still debated. The objective of this study was to describe the indications, timing, complications, and outcomes of infants on respiratory support who had a tracheostomy in a tertiary pediatric intensive care unit (PICU).MethodsAll children younger than 18 months of corrected age requiring respiratory support for at least 1 week and who had a tracheostomy between January 2005 and December 2015 were included. Their demographic and clinical data and their outcomes at 24 months of corrected age were collected and analyzed after approval from the CHU Sainte-Justine ethics committee.ResultsDuring the study period, 18 children (14 preterm infants, 4 polymalformative syndromes, and 2 diaphragmatic hernias) were included. The median corrected age at tracheostomy was 97 days (0–289 days) and 94.4% were elective. The indications for tracheostomy were ventilation for more than 7 days with (61.1%) or without (38.9%) orolaryngotracheal anomaly. The median number of consultants involved per patient was 16 consultants (10–23 consultants). The median hospital length of stay was 122 days (8–365 days) before tracheostomy and 235 days (22–891 days) after tracheostomy. The median invasive ventilation time was 68 days (8–168 days) before tracheostomy and 64 days (5–982 days) after tracheostomy. In terms of complications, there were nine cases of tracheitis and five cases of tracheal granulomas. At 24 months of corrected age, 17 of 18 children survived, one of/17 was still hospitalized, three of 17 were decannulated, three of 17 received respiratory support via their tracheostomy, 11 of 17 were fed with a gastrostomy, and all had neurodevelopmental delay.ConclusionTracheostomy in infants requiring at least 1 week of ventilation is performed for complex cases and is favored for orolaryngotracheal anomalies. Clinicians should anticipate the need for developmental care in this population.     

Clinical manifestations and epilepsy treatment in Japanese patients with pathogenic CDKL5 variants

ObjectivePatients with pathogenic cyclin-dependent kinase-like-5 gene (CDKL5) variants are designated CDKL5 deficiency disorder (CDD). This study aimed to delineate the clinical characteristics of Japanese patients with CDD and elucidate possible appropriate treatments.MethodsWe recruited patients with pathogenic or likely pathogenic CDKL5 variants from a cohort of approximately 1,100 Japanese patients with developmental and epileptic encephalopathies, who underwent genetic analysis. We retrospectively reviewed clinical, electroencephalogram, neuroimaging, and genetic information.ResultsWe identified 29 patients (21 females, eight males). All patients showed severe developmental delay, especially in males. Involuntary movements were observed in 15 patients. No antiepileptic drugs (AEDs) achieved seizure freedom by monotherapy. AEDs achieving ≥ 50% reduction in seizure frequency were sodium valproate in two patients, vigabatrin in one, and lamotrigine in one. Seizure aggravation was observed during the use of lamotrigine, potassium bromide, and levetiracetam. Adrenocorticotrophic hormone (ACTH) was the most effective treatment. The ketogenic diet (KD), corpus callosotomy and vagus nerve stimulation did not improve seizure frequency in most patients, but KD was remarkably effective in one. The degree of brain atrophy on magnetic resonance imaging (MRI) reflected disease severity. Compared with females, males had lower levels of attained motor development and more severe cerebral atrophy on MRI.ConclusionOur patients showed more severe global developmental delay than those in previous studies and had intractable epilepsy, likely because previous studies had lower numbers of males. Further studies are needed to investigate appropriate therapy for CDD, such as AED polytherapy or combination treatment involving ACTH, KD, and AEDs.     

Cell types and cell-substrate interactions in serum-free dissociated cultures of rat hypothalamus

We have developed a system for the long-term cultivation of hypothalamic neurons at low density in defined serum-free medium, and have identified the major classes of cells in these cultures. Cells prepared from the dissociation of embryonic rat hypothalami are plated, in serum-free medium, onto monolayers of cortical astrocytes. Neurons adhere and begin to extend neurites soon after plating, and survive for up to several weeks in culture. Cell-type-specific immunological markers were utilized to identify neurons and the major classes of glial cells in these cultures. The culture of hypothalamic neurons in serum-free conditions provides a valuable system for the study of the cellular basis of hypothalamic neuronal heterogeneity and functional diversity.     

成人髋关节发育不良髋臼解剖学特征及临床意义

目的观察成人髋关节发育不良（DDH）终末期骨性关节炎患者的髋臼解剖形态特征,探讨在人工全髋关节置换术（THR）中各型髋臼缺损发生的特点和重建方法。方法回顾性分析2003年3月—2012年9月60例72髋成人DDH终末期骨性关节炎患者行THR患者的临床资料,观察髋臼解剖形态特征;并以髋臼中心为同心圆,Harris窝底为深度磨削髋臼塑形后,观察髋臼缺损的特点。根据缺损大小和对髋臼假体初始稳定性的影响分别行不植骨、骨泥植骨和结构性植骨治疗。结果72髋中,20髋呈浅杯状,其中CroweI型14例、CroweⅡ型4例、CroweⅣ型2例,髋臼缺损〈10％者15髋、10％～30％者5髋;22髋呈浅盘状,其中CmweI型2例、CmweⅡ型16例、CroweⅢ型4例,髋臼缺损〈10％者2髋、10％-30％者16髋、〉30％者4髋;20髋呈贝壳状,其中CroweⅡ型5例、CroweⅢ型17例,髋臼缺损〈10％者1髋、10％-30％者5髋、〉30％者14髋;10髋呈三角状,均为CroweⅣ型,髋臼缺损〈10％者8髋、10％～50％者2髋。髋臼缺损〈10％者,髋臼假体稳定,未植骨;10％-30％者,髋臼假体较稳定,骨泥植骨;〉30％者髋臼假体不稳定,行结构性植骨后假体稳定。结论成人DDH终末期骨性关节炎的髋臼具有浅杯状、浅盘状、贝壳状和三角状4种解剖形态,这些异常解剖形态的发生除与先天性发育缺陷和后天长期的异常力学环境有关,还与股骨头脱位程度有关;在人工THR中,应根据不同形态髋臼缺损发生的特点和对髋臼假体稳定性的影响确定髋臼重建方法。     

唐山市区重点高中学生焦虑心理及影响因素

目的调查唐山市区重点高中生焦虑症发生现况,分析相关因素,为进行相关的心理干预及治疗提供依据。方法分层整群抽取唐山市区4所重点高中的1338名高中生,应用焦虑自评量表(SAS)进行测试,以SPSS13.0进行统计分析。结果①1338名高中生焦虑检出率为17.9%,其中处于经常口角争执的家庭、家长粗暴的教育,女生及高三年级学生的焦虑检出率偏高,差别有统计学意义(χ2=33.17,25.31,9.50,12.46;P0.001);②SAS测评得分以处于经常口角争执的家庭、家长粗暴的教育及高三年级学生偏高,差异有统计学意义(F=21.08,18.97,7.49;P=0.001);③性别、家庭氛围和受教育方式是重点高中学生焦虑的重要影响因素(OR=0.62,0.60,0.68;P0.05)。结论高中生是焦虑症的高危人群,高中女生较男生更易出现焦虑症状,高年级焦虑症检出率高于低年级,家庭成员间关系冷漠,教育方式粗暴会明显导致焦虑的产生。     

Gesell 问卷用于评价小儿神经发育的信度及效度分析

目的：检验 Gesell 问卷用于评价小儿神经发育的信度及效度。方法对1岁以内小儿按照入选标准筛选出50例,由专一检查者对患儿进行 Gesell 发育评估,同一患儿家长于1小时内填写 Gesell 问卷;利用内部一致性分析检验 Gesell 问卷的信度并使用相关性效度分析用来评价问卷的效度,同时评价 Gesell 问卷的敏感性、特异性,及阳性预测值、阴性预测值。结果对 Gesell 问卷按5个能区分别检验内部一致性,五个功能区的 Cronbachˊs Alpha 范围在0.479~0.743之间,除语言能区外 Gesell 问卷各个功能区的分半信度较好。Gesell 问卷与 Gesell 发育量表五个功能区间的相关性较高,相关系数 r 均为正值且范围为0.376~0.636（ P <0.01）。除适应性能区的敏感性较差外,其他四区的敏感性及特异性均较好。Gesell 问卷五个功能区的阳性预测值范围为42.9％~75.0％,阴性预测值范围为69.7％~92.3％。结论 Gesell 问卷的信度及效度较好,特异性和阴性预测值较高,对于初级筛查神经发育异常小儿具有实用价值。