Paediatrics (RCH) - Research Publications

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    Stability of combined Le Fort I maxillary advancement and mandibular reduction.
    Arpornmaeklong, P ; Shand, JM ; Heggie, AA (Walter de Gruyter GmbH, 2003-11)
    BACKGROUND: There have been reports that correction of severe Class III abnormality by single jaw surgery may invite relapse in the long-term. The purpose of this study was to retrospectively evaluate the stability of combined Le Fort I maxillary advancement and bilateral sagittal split osteotomies for mandibular reduction. METHODS: Thirty patients with a skeletal Class III malocclusion underwent bimaxillary surgery using rigid fixation and interpositional bone grafting of the maxilla. The average age was 24.4 years, and the mean follow-up period was 20 months (Range: 12-63 months). Post-operative changes were measured on lateral cephalometric radiographs using an anatomical best-fit technique. RESULTS: The maxilla was advanced, on average, 6.1 mm (SD: 1.8 mm) and repositioned superiorly at PNS 1.9 mm (SD: 2.1 mm). The mandible was repositioned posteriorly 5.6 mm ISD: 4.2 mm) at menton, which also auto-rotated superiorly. At follow-up, the maxilla relapsed horizontally 0.6 mm (SD: 1.1 mm, p < 0.01) with no significant vertical change. The maxillary central incisors were proclined and the interincisal angle was reduced. Menton relapsed anteriorly 1.4 mm (SD: 2.7 mm, p < 0.01), and gonion rotated superiorly 1.5 mm (SD: 2.3 mm, p < 0.001). In 67 per cent of cases menton moved anteriorly less than 2.5 mm. The overjet and overbite did not change significantly. CONCLUSIONS: The data show that 12-months post-operatively, maxillary advancement combined with mandibular setback was relatively stable in the horizontal and vertical planes.
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    Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex I
    Lazarou, M ; McKenzie, M ; Ohtake, A ; Thorburn, DR ; Ryan, MT (AMER SOC MICROBIOLOGY, 2007-06)
    Complex I of the respiratory chain is composed of at least 45 subunits that assemble together at the mitochondrial inner membrane. Defects in human complex I result in energy generation disorders and are also implicated in Parkinson's disease and altered apoptotic signaling. The assembly of this complex is poorly understood and is complicated by its large size and its regulation by two genomes, with seven subunits encoded by mitochondrial DNA (mtDNA) and the remainder encoded by nuclear genes. Here we analyzed the assembly of a number of mtDNA- and nuclear-gene-encoded subunits into complex I. We found that mtDNA-encoded subunits first assemble into intermediate complexes and require significant chase times for their integration into the holoenzyme. In contrast, a set of newly imported nuclear-gene-encoded subunits integrate with preexisting complex I subunits to form intermediates and/or the fully assembly holoenzyme. One of the intermediate complexes represents a subassembly associated with the chaperone B17.2L. By using isolated patient mitochondria, we show that this subassembly is a productive intermediate in complex I assembly since import of the missing subunit restores complex I assembly. Our studies point to a mechanism of complex I biogenesis involving two complementary processes, (i) synthesis of mtDNA-encoded subunits to seed de novo assembly and (ii) exchange of preexisting subunits with newly imported ones to maintain complex I homeostasis. Subunit exchange may also act as an efficient mechanism to prevent the accumulation of oxidatively damaged subunits that would otherwise be detrimental to mitochondrial oxidative phosphorylation and have the potential to cause disease.
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    The Australasian Society for Infectious Diseases guidelines for the diagnosis, management and prevention of infections in recently arrived refugees: an abridged outline
    Murray, RJ ; Davis, JS ; Burgner, DP (WILEY, 2009-04-20)
    About 13,000 refugees are currently accepted for migration into Australia each year, many of whom have spent protracted periods living in extremely disadvantaged circumstances. As a result, medical practitioners are increasingly managing recently arrived refugees with acute and chronic infectious diseases. The Australasian Society for Infectious Diseases has formulated guidelines for the diagnosis, management and prevention of infection in newly arrived refugees. This article is an abridged version of the guidelines, which are available in full at . All refugees should be offered a comprehensive health assessment, ideally within 1 month of arrival in Australia, that includes screening for and treatment of tuberculosis, malaria, blood-borne viral infections, schistosomiasis, helminth infection, sexually transmitted infections, and other infections (eg, Helicobacter pylori) as indicated by clinical assessment; and assessment of immunisation status, and catch-up immunisations where appropriate. The assessment can be undertaken by a general practitioner or within a multidisciplinary refugee health clinic, with use of an appropriate interpreter when required. The initial assessment should take place over at least two visits: the first for initial assessment and investigation and the second for review of results and treatment or referral.
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    Avoiding conflict: What do adolescents with disordered eating say about their mothers in music therapy?
    McFerran, K ; Baker, F ; Kildea, C ; Patton, G ; Sawyer, S (SAGE Publications, 2008-06-01)
    Music therapy is an integral part of the inpatient treatment programme for young women with disordered eating at the Royal Children's Hospital in Melbourne. As part of ongoing clinical audit activities, an investigation was undertaken to analyse retrospectively the lyrics of young women who had participated in the music therapy programme. The¬¬¬¬¬¬ purpose was to monitor and improve local clinical practice and clarify the specific contribution of music therapy to the diagnosis of anorexia nervosa. Results highlighted the role of mothers in the experiences of the young participants, with references to this relationship exceeding those to any other relationships. These findings are discussed in conjunction with an abandoned study where parental consent was not forthcoming for participation in a group music therapy research project. This article promotes a continuing awareness of the importance of the mother-daughter relationship in the treatment of eating disorders.
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    New Mid-Cretaceous (latest Albian) dinosaurs fromWinton, Queensland, Australia.
    Hocknull, SA ; White, MA ; Tischler, TR ; Cook, AG ; Calleja, ND ; Sloan, T ; Elliott, DA ; Sereno, P (Public Library of Science (PLoS), 2009-07-03)
    BACKGROUND: Australia's dinosaurian fossil record is exceptionally poor compared to that of other similar-sized continents. Most taxa are known from fragmentary isolated remains with uncertain taxonomic and phylogenetic placement. A better understanding of the Australian dinosaurian record is crucial to understanding the global palaeobiogeography of dinosaurian groups, including groups previously considered to have had Gondwanan origins, such as the titanosaurs and carcharodontosaurids. METHODOLOGY/PRINCIPAL FINDINGS: We describe three new dinosaurs from the late Early Cretaceous (latest Albian) Winton Formation of eastern Australia, including; Wintonotitan wattsi gen. et sp. nov., a basal titanosauriform; Diamantinasaurus matildae gen. et sp. nov., a derived lithostrotian titanosaur; and Australovenator wintonensis gen. et sp. nov., an allosauroid. We compare an isolated astragalus from the Early Cretaceous of southern Australia; formerly identified as Allosaurus sp., and conclude that it most-likely represents Australovenator sp. CONCLUSION/SIGNIFICANCE: The occurrence of Australovenator from the Aptian to latest Albian confirms the presence in Australia of allosauroids basal to the Carcharodontosauridae. These new taxa, along with the fragmentary remains of other taxa, indicate a diverse Early Cretaceous sauropod and theropod fauna in Australia, including plesiomorphic forms (e.g. Wintonotitan and Australovenator) and more derived forms (e.g. Diamantinasaurus).
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    Decline in Physical Fitness From Childhood to Adulthood Associated With Increased Obesity and Insulin Resistance in Adults
    Dwyer, T ; Magnussen, CG ; Schmidt, MD ; Ukoumunne, OC ; Ponsonby, A-L ; Raitakari, OT ; Zimmet, PZ ; Blair, SN ; Thomson, R ; Cleland, VJ ; Venn, A (AMER DIABETES ASSOC, 2009-04)
    OBJECTIVE: To examine how fitness in both childhood and adulthood is associated with adult obesity and insulin resistance. RESEARCH DESIGN AND METHODS: A prospective cohort study set in Australia in 2004-2006 followed up a cohort of 647 adults who had participated in the Australian Schools Health and Fitness Survey in 1985 and who had undergone anthropometry and cardiorespiratory fitness assessment during the survey. Outcome measures were insulin resistance and obesity, defined as a homeostasis model assessment index above the 75th sex-specific percentile and BMI >or=30 kg/m(2), respectively. RESULTS: Lower levels of child cardiorespiratory fitness were associated with increased odds of adult obesity (adjusted odds ratio [OR] per unit decrease 3.0 [95% CI 1.6-5.6]) and insulin resistance (1.7 [1.1-2.6]). A decline in fitness level between childhood and adulthood was associated with increased obesity (4.5 [2.6-7.7]) and insulin resistance (2.1 [1.5-2.9]) per unit decline. CONCLUSIONS: A decline in fitness from childhood to adulthood, and by inference a decline in physical activity, is associated with obesity and insulin resistance in adulthood. Programs aimed at maintaining high childhood physical activity levels into adulthood may have potential for reducing the burden of obesity and type 2 diabetes in adults.
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    Duration of Nocturnal Hypoglycemia Before Seizures
    Buckingham, B ; Wilson, DM ; Lecher, T ; Hanas, R ; Kaiserman, K ; Cameron, F (AMER DIABETES ASSOC, 2008-11)
    OBJECTIVE: Despite a high incidence of nocturnal hypoglycemia documented by the use of continuous glucose monitoring (CGM), there are no reports in the literature of nocturnal hypoglycemic seizures while a patient is wearing a CGM device. RESEARCH DESIGN AND METHODS: In this article, we describe four such cases and assess the duration of nocturnal hypoglycemia before the seizure. RESULTS: In the cases where patients had a nocturnal hypoglycemic seizure while wearing a CGM device, sensor hypoglycemia (<60 mg/dl) was documented on the CGM record for 2.25-4 h before seizure activity. CONCLUSIONS: Even with a subcutaneous glucose lag of 18 min when compared with blood glucose measurements, glucose sensors have time to provide clinically meaningful alarms. Current nocturnal hypoglycemic alarms need to be improved, however, since patients can sleep through the current alarm systems.
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    Paradoxical myopic shift following cycloplegia in retinopathy of prematurity patients: a case series.
    London, NJ ; Carden, SM ; Good, WV (Springer Science and Business Media LLC, 2009-08-25)
    INTRODUCTION: Spectacle non-compliance is a significant problem in pediatric patients, and may have a variety of consequences. Non-compliance with myopic refractive correction could be secondary to a variety of issues, including age, discomfort, gender, urban vs. rural residence, presenting visual acuity, and degree of refractive error. We observed a phenomenon in our pediatric patients with retinopathy of prematurity that may add another possible explanation: incorrect prescription due to measures of increased, rather than decreased, myopia after cycloplegia. CASE PRESENTATION: An unmasked, prospective study of 8 consecutive patients seen in a single practice. Retinoscopic refraction measurements were obtained before and after pharmacologic cycloplegia. In all 13 eyes, there was either no change (2 eyes) or a myopic shift (11 eyes) in the measured refractive error. The average change in refraction was -1.58 and -1.54 for the right and left eyes, respectively (range 0 to -3.00 OD and 0 to -3.00 OS). CONCLUSIONS: The contribution of ocular components to refractive status differs between ROP and non-ROP eyes. Unanticipated myopic shift following cycloplegia in ROP patients may result in inappropriate glasses prescription with poor correction of visual acuity. This may contribute to spectacle noncompliance in this group.
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    Unravelling the molecular control of calvarial suture fusion in children with craniosynostosis.
    Coussens, AK ; Wilkinson, CR ; Hughes, IP ; Morris, CP ; van Daal, A ; Anderson, PJ ; Powell, BC (Springer Science and Business Media LLC, 2007-12-12)
    BACKGROUND: Craniosynostosis, the premature fusion of calvarial sutures, is a common craniofacial abnormality. Causative mutations in more than 10 genes have been identified, involving fibroblast growth factor, transforming growth factor beta, and Eph/ephrin signalling pathways. Mutations affect each human calvarial suture (coronal, sagittal, metopic, and lambdoid) differently, suggesting different gene expression patterns exist in each human suture. To better understand the molecular control of human suture morphogenesis we used microarray analysis to identify genes differentially expressed during suture fusion in children with craniosynostosis. Expression differences were also analysed between each unfused suture type, between sutures from syndromic and non-syndromic craniosynostosis patients, and between unfused sutures from individuals with and without craniosynostosis. RESULTS: We identified genes with increased expression in unfused sutures compared to fusing/fused sutures that may be pivotal to the maintenance of suture patency or in controlling early osteoblast differentiation (i.e. RBP4, GPC3, C1QTNF3, IL11RA, PTN, POSTN). In addition, we have identified genes with increased expression in fusing/fused suture tissue that we suggest could have a role in premature suture fusion (i.e. WIF1, ANXA3, CYFIP2). Proteins of two of these genes, glypican 3 and retinol binding protein 4, were investigated by immunohistochemistry and localised to the suture mesenchyme and osteogenic fronts of developing human calvaria, respectively, suggesting novel roles for these proteins in the maintenance of suture patency or in controlling early osteoblast differentiation. We show that there is limited difference in whole genome expression between sutures isolated from patients with syndromic and non-syndromic craniosynostosis and confirmed this by quantitative RT-PCR. Furthermore, distinct expression profiles for each unfused suture type were noted, with the metopic suture being most disparate. Finally, although calvarial bones are generally thought to grow without a cartilage precursor, we show histologically and by identification of cartilage-specific gene expression that cartilage may be involved in the morphogenesis of lambdoid and posterior sagittal sutures. CONCLUSION: This study has provided further insight into the complex signalling network which controls human calvarial suture morphogenesis and craniosynostosis. Identified genes are candidates for targeted therapeutic development and to screen for craniosynostosis-causing mutations.
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    A cross-sectional survey of complementary and alternative medicine use by children and adolescents attending the University Hospital of Wales.
    Crawford, NW ; Cincotta, DR ; Lim, A ; Powell, CVE (Springer Science and Business Media LLC, 2006-05-02)
    BACKGROUND: A high prevalence of CAM use has been documented worldwide in children and adolescents with chronic illnesses. Only a small number of studies, however, have been conducted in the United Kingdom. The primary aim of this study was to examine the use of CAM by children and adolescents with a wide spectrum of acute and chronic medical problems in a tertiary children's hospital in Wales. METHODS: Structured personal interviews of 100 inpatients and 400 outpatients were conducted over a 2-month period in 2004. The yearly and monthly prevalence of CAM use were assessed and divided into medicinal and non-medicinal therapies. This use was correlated with socio-demographic factors. RESULTS: There were 580 patients approached to attain 500 completed questionnaires. The use of at least one type of CAM in the past year was 41% (95% CI 37-46%) and past month 26% (95% CI 23-30%). The yearly prevalence of medicinal CAM was 38% and non-medicinal 12%. The users were more likely to have parents that were tertiary educated (mother: OR = 2.3, 95%CI 1.6-3.3) and a higher family income (Pearson chi-square for trend = 14.3, p < 0.001). The most common medicinal types of CAM were non-prescribed vitamins and minerals (23%) and herbal therapies (10%). Aromatherapy (5%) and reflexology (3%) were the most prevalent non-medicinal CAMs. None of the inpatient medical records documented CAM use in the past month. Fifty-two percent of medicinal and 38% of non-medicinal CAM users felt their doctor did not need to know about CAM use. Sixty-six percent of CAM users did not disclose the fact to their doctor. Three percent of all participants were using herbs and prescription medicines concurrently. CONCLUSION: There is a high prevalence of CAM use in our study population. Paediatricians need to ensure that they ask parents and older children about their CAM usage and advise caution with regard to potential interactions.CAM is a rapidly expanding industry that requires further evidence-based research to provide more information on the effectiveness and safety of many CAM therapies. Statutory or self-regulation of the different segments of the industry is important. Integration of CAM with allopathic western medicine through education and better communication is slowly progressing.