Paediatric rehabilitation

More and more researchers are questioning the theoretical and scientific foundations as well as the efficacy and effects of many physiotherapy interventions. The same applies for many of the neurophysiological based interventions that are being used in paediatric rehabilitation. Opinions and views regarding the development of motor behaviour of infants and children are significantly changing. Paediatric interventionists should consider bringing their interventions and focus of treatment into agreement with changed scientific knowledge. Moreover, for almost all other medical problems in childhood, paediatric rehabilitation has little to offer but mostly miniaturized forms of adult treatment. It not only means that we have to make a paradigm shift, but also are in the need of a broader view on paediatric rehabilitation as a specialized professional activity.     

Diagnostic strategies for the evaluation of asymmetry in infancy—a review

Asymmetry in infancy is a diagnosis with a large spectrum of features, expressing an abnormal shape of parts of the body or unequal postures and movements. Symptoms may be structural and/or functional, with localised or generalised expression. Within the last decade, many professionals have focused on the adverse consequences on motor performance of infants, associated with the recommendations on the prevention of sudden infant death. The purpose of the present study was to highlight different aspects of asymmetry in infancy based on best available evidence in the current literature and to present a flow chart illustrating different diagnostic pathways of asymmetry in infancy. Conclusion:we conclude that asymmetry in infancy is a diagnosis with a large spectrum of features and a multifactorial aetiology without consensus in definition, nomenclature and classification. Systematic diagnostic management of asymmetry in infancy is indicated. The presented diagnostic flow chart might serve as a basis.Abbreviations CMT congenital muscular torticollis - DP deformational plagiocephaly - POI pseudotumour of infancy - PT positional torticollis - SCM sternocleidomastoid muscle     

Osteogenesis imperfecta in childhood: impairment and disability. A prospective study with 4-year follow-up

OBJECTIVES: To study (1). changes in anthropometrics, joint range of motion (ROM), muscle strength, functional ability, caregiver assistance, and level of ambulation in children with osteogenesis imperfecta (OI) and (2). the prediction of clinical characteristics at the level of ambulation at follow-up and the prediction of clinical characteristics on progression or regression at the level of ambulation over time. DESIGN: Prospective study with follow-up of 4 years. SETTING: A children's hospital that serves a nationwide center for treatment and research in children with OI in the Netherlands. PARTICIPANTS: At follow-up, 49 children (24 boys, 25 girls; mean age +/- standard deviation, 11.3+/-3.8y; range, 5.2-19.4y) participated. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Anthropometry, joint ROM, muscle strength, fracture frequency, intramedullary rodding, level of ambulation, functional ability, and caregiver assistance. RESULTS: In type I OI, total joint ROM decreased significantly over time, especially in the lower extremities, with a significant decrease in generalized joint hypermobility according to Bulbena (median start, 7.5; interquartile range [IQR], 4-9; median end, 6; IQR, 2-7; P<.001). In types III and IV, a severe decrease in total joint ROM was present without significant changes over time. No significant changes in total muscle strength (upper or lower extremities) in the different types of OI were measured at follow-up. In OI type I, a significant increase in self-care (P=.003) and social function (P=.008) was measured; in type III, a significant increase in self-care (P=.003), mobility (P=.004), and social function (P=.005) was measured, with a significant decrease in parental assistance in self-care (P=.02) and mobility (P=.005). In type IV, a significant increase was observed in the self-care (P=.01) and social function domains (P=.02). Type of OI (regression coefficient=-1.0; 95% confidence interval [CI], -1.64 to -0.47) and total muscle strength were the only significant predictors for level of ambulation (regression coefficient=.01; 95% CI,.17-.32). Body weight was significantly lower in the group that progressed in level of ambulation (P=.03), whereas children with a decline in level of ambulation had significantly higher body weight (P=.05). CONCLUSIONS: Ours is the first study with a long-term follow-up that provides information concerning the natural course of developmental outcome parameters of OI in childhood. Joint ROM and muscle strength did not change significantly over time, possibly because of the biomechanical skeletal properties of the different OI types. Functional ability improved significantly over time, but, especially in types III and IV, did not reach normative values, possibly because of a plateau phase in functional ability. Knowledge of the natural course of the disease is essential to interpret the results from intervention studies.     

织构复合水凝胶体系的假说

生物软组织可视为具有多层次结构的织构复合水凝胶体系(TCHS)、以水凝胶复合元件(HCE)为基本的构件(CP),通过一定的组合、排列方式构筑一系列多层次结构的不同软组织。软组织中任何层次的结构单元既可视为织构复合水凝胶体系又可视为构件。任何层次的TCHS中,构件的结构及其组合排列方式决定着该层次单元的功能。以织构复合水凝胶体系的观点考察了真核细胞、角膜和骨骼肌的多层次结构。双层网络水凝胶、皮芯复合水凝胶纤维人工肌肉模型、时空匹配可降解细胞支架等研究成果初步地证明了提出织构复合水凝胶体系观点的合理性。     

大鼠移植心脏组织中血小板衍生生长因子A mRNA表达及雷帕霉素的干预效应

目的:血小板衍生生长因子在平滑肌细胞的表型转化过程中起重要作用。观察大鼠移植心脏组织中血小板衍生生长因子AmRNA表达的变化及雷帕霉素的干预效应。方法:实验于2005-10/2006-01在中南大学湘雅二医院胸心外科实验室完成。将60只SD大鼠、24只Wistar大鼠按随机数字表法分为3组:①同系移植组:供、受体各12只,均为SD大鼠。②异系移植组:供体为Wistar大鼠(n=24),受体为SD大鼠(n=24),受体大鼠随机分为雷帕霉素组(n=12)和环孢霉素组(n=12),术后分别给予雷帕霉素1.25mg/(kg·d)灌胃及环孢霉素A10mg/(kg·d)皮下注射,给药60d,给药结束后留取移植心脏待检。③另12只SD大鼠直接取心脏组织作为正常对照组。指标检测:①对移植心脏组织行VanGieson染色后采用Miassystem4.1医学图像分析管理系统分析血管狭窄程度。②应用反转录-聚合酶链反应检测血小板衍生生长因子AmRNA在移植心脏组织中的表达情况。结果:36只受体SD大鼠及12只正常SD大鼠全部进入结果分析,无脱失。①同系移植组、环孢霉素组及雷帕霉素组大鼠的冠状动脉狭窄指数均显著高于正常对照组[(13.12±0.72)%,(62.45±8.12)%,(28.91±3.24)%,(0.09±0.02)%(P<0.01)],环孢霉素组及雷帕霉素组高于同系移植组(P<0.05),环孢霉素组高于雷帕霉素组(P<0.01)。②正常对照组、同系移植组、环孢霉素组及雷帕霉素组大鼠的血小板衍生生长因子AmRNA相对含量分别为0.19±0.06,0.21±0.08,1.12±0.22及0.47±0.11,环孢霉素组、雷帕霉素组显著高于同系移植组(P<0.01),环孢霉素组高于雷帕霉素组(P<0.05)。结论:血小板衍生生长因子AmRNA的高表达与移植心脏的血管硬化有关;雷帕霉素具有预防大鼠心脏移植物血管病变的作用,其作用可能与抑制心脏组织中血小板衍生生长因子AmRNA的表达有关。     

骨髓基质干细胞移植对正常和心肌梗死大鼠心电图及心功能的影响

目的:骨髓基质干细胞移植到心肌梗死的瘢痕心肌组织中可以改善心功能,但以心电图为观察指标的研究不多。实验观察骨髓基质干细胞移植对正常和心肌梗死大鼠心电图及心功能的影响。方法:实验于2004-01/2005-03在哈尔滨医科大学完成。①实验动物:选取4周龄雄性Wistar大鼠80只,随机数字表法分为梗死移植组、正常移植组、梗死非移植组、正常非移植组,20只/组。另选取7d龄Wistar雄鼠30只作为骨髓基质干细胞的来源。②实验方法:采用密度梯度离心法获取鼠骨髓基质干细胞,配成1×109L-1的细胞悬液,使用5-氮胞苷体外诱导培养3～4周,移植前24～48h行Brdu标记。取载有细胞的盖玻片,测定钙释放时将20mmol/L的caffeine快速加在细胞表面。梗死移植组、梗死非移植组大鼠建立心肌梗死模型。造模4周后,梗死移植组将0.25mL诱导的骨髓基质干细胞悬液注射至大鼠心肌梗死后的瘢痕组织,正常移植组同法将骨髓基质干细胞悬液注射至正常心肌组织,梗死非移植组、正常非移植组注射等量不含骨髓基质干细胞的培养液基质。③实验评估:观察骨髓基质干细胞的诱导分化情况及其植入后在瘢痕心肌组织中的生存状态。测定细胞内钙离子浓度。记录术前、冠脉结扎后即刻/细胞移植即刻、术后4周的心电图变化。检测术后4周的超声和血流动力学指标变化。结果:80只大鼠均进入结果分析。①骨髓基质干细胞的诱导分化及其植入后的生存状态:5-氮胞苷诱导3周后,骨髓基质干细胞表达肌钙蛋白Ⅰ和肌凝蛋白重链,细胞内有丰富的肌丝和Z线,细胞器较多。植入4周后在心肌瘢痕组织中分化为心肌细胞。②细胞内钙离子浓度:两组细胞在caffeine刺激下钙离子的释放均呈波峰状,但诱导组应用caffeine后钙离子浓度降低且低于基础状态,钙释放受到抑制,未诱导组不受影响。③心电图观察:与术前比较,梗死移植组QRS波变窄,R波降支出现正常顿挫波,未见显著心律失常。④超声检测及血流动力学分析:术后4周,与梗死非移植组比较,梗死移植组左室收缩末压、左室射血分数和压力变化速率最大值均显著升高(P<0.05或0.01)。结论:骨髓基质干细胞体外诱导后能分化为心肌样细胞,植入到瘢痕心肌组织中生存、增殖良好,可改善心电图及心肌弹性,从而改善心肌梗死大鼠的心功能。     

The Dutch version of the self-report Child Activity and Limitations Interview in adolescents with chronic pain

Purpose: To assess the factor structure, related constructs and internal consistency of the Child Activity Limitation Interview 21-Child version for use in Dutch-language countries.

Methods: Cross-sectional validation study: After forward and back translation of the Dutch version of the Child Activity Limitation Interview 21-Child adolescents (11–21 years old) with chronic musculoskeletal pain completed an assessment. The assessment contained the Dutch Child Activity Limitation Interview, and questionnaires about demographics, pain intensity, functional disability, anxiety and depression. Internal consistency and construct validity were evaluated through exploratory factor analysis (principal axis factoring with oblique rotation) and hypotheses testing using pain intensity, activity limitations, anxiety and depression as comparative constructs.

Results: Seventy-four adolescents completed the assessment. Exploratory factor analysis resulted in a two-factor structure, explaining 50% of the variance. Internal consistency was good (Cronbach’s α?=?0.91 total scale, α?=?0.90 Factor 1, α?=?0.80 Factor 2). All nine hypotheses were confirmed.

Conclusion: The Dutch version can be used to assess pain-related disability in Dutch-speaking adolescents comparable to the study sample. Scores on both subscales provide insight into the severity of the pain-related disability in both daily routine and more physically vigorous activities.

• Implications for Rehabilitation

• Chronic pain is a disabling disorder which not only impacts physically but restricts quality of life.

• This study provides clinicians a questionnaire to measure pain-related disability and quantify the impact of pain on the daily living of adolescents.

• The advantage of the Dutch version of the Child Activity and Limitations Interview over other measurements is that it can distinguish limitations in daily activities from more physically vigorous activities.

     

Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the hemophilia joint health score

Objective

Repeated hemarthrosis in hemophilia causes arthropathy with pain and dysfunction. The Hemophilia Joint Health Score (HJHS) was developed to be more sensitive for detecting arthropathy than the World Federation of Hemophilia (WFH) physical examination scale, especially for children and those using factor prophylaxis. The HJHS has been shown to be highly reliable. We compared its validity and sensitivity to the WFH scale.

Methods

We studied 226 boys with mild, moderate, and severe hemophilia at 5 centers. The HJHS was scored by trained physiotherapists. Study physicians at each site blindly determined individual and total joint scores using a series of visual analog scales.

Results

The mean age was 10.8 years. Sixty‐eight percent were severe (93% of whom were treated with prophylaxis), 15% were moderate (24% treated with prophylaxis), and 17% were mild (3% treated with prophylaxis). The HJHS correlated moderately with the physician total joint score (r_s = 0.42, P < 0.0001) and with overall arthropathy impact (r_s = 0.42, P < 0.0001). The HJHS was 97% more efficient than the WFH at differentiating severe from mild and moderate hemophilia. The HJHS was 74% more efficient than the WFH at differentiating subjects treated with prophylaxis from those treated on demand. We identified items on the HJHS that may be redundant or rarely endorsed and could be removed from future versions.

Conclusion

Both the HJHS and WFH showed evidence of strong construct validity. The HJHS is somewhat more sensitive for mild arthropathy; its use should be considered for studies of children receiving prophylaxis.     

C5a anaphylatoxin is a major regulator of activating versus inhibitory FcgammaRs in immune complex-induced lung disease

IgG Fc receptors (FcgammaRs, especially FcgammaRIII) and complement (in particular, C5a anaphylatoxin) are critical effectors of the acute inflammatory response to immune complexes (ICs). However, it is unknown whether and how these two key components can interact with each other in vivo. We use here a mouse model of the acute pulmonary IC hypersensitivity reaction to analyze their potential interaction. FcgammaRIII and C5aR are coexpressed on alveolar macrophages (AMs), and both FcgammaRIII and C5aR mutant mice display impaired immune responses. We find that recombinant human C5a (rhC5a) can control inverse expression of various FcgammaRs, and costimulation of ICs with rhC5a results in strong enhancement of FcgammaRIII-triggered cellular activation in vitro and in vivo. Moreover, we show here that early IC-induced bioactive C5a, and its interaction with C5aR, causes induction of activating FcgammaRIII and suppression of inhibitory FcgammaRII on AMs that appears crucial for efficient cytokine production and neutrophil recruitment in lung pathology. Therefore, C5a, which is a potent chemoattractant, has a broader critical function in regulating the inhibitory/activating FcgammaRII/III receptor pair to connect complement and FcgammaR effector pathways in immune inflammation.