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Adair B.,Australian Catholic University | Rodda J.,Hugh Williamson Gait Laboratory | McGinley J.L.,University of Melbourne | Graham H.K.,The Royal Childrens Hospital | Morris M.E.,La Trobe University
Developmental Medicine and Child Neurology | Year: 2016

Aim: To examine the kinematic gait deviations at the trunk and pelvis of children with hereditary spastic paraplegia (HSP). Method: This exploratory observational study quantified gait kinematics for the trunk and pelvis from 11 children with HSP (7 males, 4 females) using the Gait Profile Score and Gait Variable Scores (GVS), and compared the kinematics to data from children with typical development using a Mann–Whitney U test. Results: Children with HSP (median age 11y 4mo, interquartile range 4y) demonstrated large deviations in the GVS for the trunk and pelvis in the sagittal and coronal planes when compared to the gait patterns of children with typical development (p=0.010–0.020). Specific deviations included increased range of movement for the trunk in the coronal plane and increased excursion of the trunk and pelvis in the sagittal plane. In the transverse plane, children with HSP demonstrated later peaks in posterior pelvic rotation. Interpretation: The kinematic gait deviations identified in this study raise questions about the contribution of muscle weakness in HSP. Further research is warranted to determine contributing factors for gait dysfunction in HSP, especially the relative influence of spasticity and weakness. © 2016 Mac Keith Press

Quinlan C.,The Royal Childrens Hospital | Marks S.D.,Great Ormond Street Hospital for Children NHS Trust | Tullus K.,Great Ormond Street Hospital for Children NHS Trust
Pediatric Nephrology | Year: 2016

Juvenile-onset systemic lupus erythematosus (SLE) is an aggressive multisystem autoimmune disease. Despite improvements in outcomes for adult patients, children with SLE continue to have a lower life expectancy than adults with SLE, with more aggressive disease, a higher incidence of lupus nephritis and there is an emerging awareness of their increased risk of cardiovascular disease (CVD). In this review, we discuss the evidence for an increased risk of CVD in SLE, its pathogenesis, and the clinical approach to its management. © IPNA 2015.

McGinley J.L.,Murdoch Childrens Research Institute | McGinley J.L.,University of Melbourne | Dobson F.,University of Melbourne | Ganeshalingam R.,The Royal Childrens Hospital | And 5 more authors.
Developmental Medicine and Child Neurology | Year: 2012

Aim To conduct a systematic review of single-event multilevel surgery (SEMLS) for children with cerebral palsy, with the aim of evaluating the quality of the evidence and developing recommendations for future research. Method The systematic review was conducted using standard search and extraction methods in Medline, EMBASE, CINAHL, and Cochrane electronic databases. For the purposes of this review, SEMLS was defined as two or more soft-tissue or bony surgical procedures at two or more anatomical levels during one operative procedure, requiring only one hospital admission and one period of rehabilitation. Studies were included if: (1) the primary focus was to examine the effect of SEMLS in children with cerebral palsy; (2) the results focused on multiple anatomic levels and reported findings of one or more World Health Organization International Classification of Functioning, Disability and Health (ICF) domains. Studies that focused on a single intervention or level, or on the utility of a specific outcome measure were excluded. Study quality was appraised with the Methodological Index for Non-Randomized Studies (MINORS) and the Oxford Centre for Evidence-Based Medicine scale. The review also examined the reporting of surgery, adverse events, and rehabilitation. Results Thirty-one studies fulfilled the criteria for inclusion, over the period 1985 to October 2010. The MINORS score for these studies varied from 4 to 19, with marked variation in the quality of reporting. Study quality has improved over recent years. Valid measures of gait and function have been introduced and several of the most recent studies have addressed multiple dimensions of the ICF. A statistical synthesis of the outcome data was not conducted, although a trend towards favourable outcomes in gait was evident. Caution is advised with interpretation owing to the variable study quality. Uncontrolled studies may have resulted in an overestimation of treatment efficacy. Interpretation The design and reporting of studies of SEMLS are improving with the development of multidisciplinary teamwork and frameworks such as the ICF. However, the evidence base is limited by the lack of randomized clinical trials, especially when compared with other surgical interventions such as selective dorsal rhizotomy. © The Authors. Developmental Medicine & Child Neurology © 2011 Mac Keith Press.

Huo C.W.,The Royal Childrens Hospital
Hand surgery : an international journal devoted to hand and upper limb surgery and related research : journal of the Asia-Pacific Federation of Societies for Surgery of the Hand | Year: 2012

Radial head dislocation secondary to obstetric brachial plexus palsy is a rare complication that may occur a few years after birth. Five cases were examined and a comprehensive literature search was performed. Although it is a concern for parents, the dislocation resulted in mild or minimal functional impairment for all five children. Surgical interventions such as biceps tendon transfer, radial head open reduction or excision and annular ligament reconstruction were largely ineffective in significantly improving ranges of motion. Clinicians should be aware of the potentially futile outcomes and risks associated with the surgical treatment of radial head dislocation.

Randall M.,The Royal Childrens Hospital | Imms C.,La Trobe University | Carey L.,National Stroke Research Institute
Developmental Medicine and Child Neurology | Year: 2012

Aim This paper reports the second phase of a study to extend the Melbourne Assessment for use with children with neurological impairment aged 2 to 4years. The aim was to establish if (1) children's scores on the Modified Melbourne Assessment (MMA) and the Quality of Upper Extremity Skills Test (QUEST) showed a moderate to high, positive relation, (2) children had comparable behaviours for task and time demands on both tools, and (3) scores on the MMA could discriminate between children with mild, moderate, and severe levels of upper limb impairment. Method An observational study of 30 children (19 males, 11 females) with neurological impairment aged 2 to 4years. Twenty-four children had spasticity (20 with a unilateral and four with a bilateral impairment) and two children presented with athetosis, two with ataxia, and two with hypotonia. Results A high, positive relation was found between children's scores on the MMA and the QUEST (ρ=0.90; p=0.001). The clinical use of the MMA was comparable to the QUEST. MMA scores were able to discriminate between children's levels of upper limb impairment as determined by clinicians' ratings (F 2,27=67.76, p=0.001). Interpretation These findings suggest the MMA can be clinically useful for children as young as 2.5years and has the advantage of being valid for use with older children. Scores from the tool can also provide therapists with a quantitative means of consistently reporting level of upper limb impairment. © The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.

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