Princess Margaret Hospital for Children

Perth, Australia

Princess Margaret Hospital for Children

Perth, Australia
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Tiddens H.A.W.M.,Erasmus Medical Center | Stick S.M.,Princess Margaret Hospital for Children | Davis S.,James Whitcomb Riley Hospital for Children
Paediatric Respiratory Reviews | Year: 2014

Cystic fibrosis [CF] lung disease is characterized by progressive bronchiectasis and small airways disease. To monitor CF lung disease traditionally spirometry has been the most important modality. In addition to spirometry chest radiography was used to monitor progression of structural lung abnormalities. However, the importance of chest radiography in disease management has been limited due to its poor sensitivity and specificity to detect disease progression. Over the last decade chest CT has become the gold standard for monitoring the severity and progression of bronchiectasis. Small airways disease can be monitored using spirometry, multiple breath washout techniques, and chest CT. In modern CF-care a multi-modality approach is needed to monitor CF lung disease and to personalize treatment for the needs of the patient. When state-of-the-art low dose bi-annual chest CT protocols are used radiation risk is considered to be low. In between chest CT imaging, physiologic measures are important to obtain for monitoring. Stratification of monitoring protocols based on the risk profile of the patient can help us in the future to better care for people with CF. © 2013.

With an upcoming publication in the Worldwide Leaders in Healthcare, Jessica Pougnault, RN, joins the prestigious ranks of the International Nurses Association. She is a Registered Nurse with 13 years of experience in her field and extensive expertise in perioperative nursing and management. Jessica is currently serving patients as Acting Nurse Manager – Perioperative Department at Princess Margaret Hospital for Children in Subiaco, Western Australia. Jessica obtained her Master’s Degree in Clinical Nursing – Perioperative, in 2015 from Curtin University, where she also received her Bachelor of Science Degree – Nursing in 2000. Additionally, she holds a postgraduate diploma in perioperative nursing acquired in 2004 from Fremantle Hospital. She is currently undertaking postgraduate management courses and aims to further her studies in this field.  Jessica maintains professional memberships with the Australian Health Practitioner Regulation Agency (AHPRA), the Australian Nursing & Midwifery Federation, and the Australian College of Operating Room Nurses. Jessica attributes her success as a nurse to her commitment, not only to her work but to her family as well. When she is not plotting and planning as a Perioperative Manager, Jessica enjoys shopping, reading, walking and spending time with her precious family. Learn more about Jessica Pougnault here and read her upcoming publication in the Worldwide Leaders in Healthcare.

Logan J.,Princess Margaret Hospital for Children
Studies in Health Technology and Informatics | Year: 2012

The implementation of clinical information systems and electronic medical records does not have a good track record. It is estimated that more than 50% of implementations fail. A review of electronic health information system (EHIS) models incorporating clinical information systems and electronic medical records was undertaken to determine the models developed and applied in health. Twenty one health and five non-health models were identified. The non-health models were included as a number of health models were derived form these. The findings and evaluation of the models has identified varying contents and results. The models identified were assessed to determine how these related to each other, whether models were tested and how, if benefits were identified and if cost-savings were projected or realised. This review of EHIS implementation models has identified a need for clear definition of terms used, careful categorisation and for models to be comprehensive, extensive and rigorous if successful outcomes are to occur. © 2012 The authors and IOS Press. All rights reserved.

Geelhoed G.C.,Princess Margaret Hospital for Children | Geelhoed G.C.,University of Western Australia | de Klerk N.H.,Telethon Institute for Child Health Research | de Klerk N.H.,University of Western Australia
Medical Journal of Australia | Year: 2012

Objective: To assess whether emergency department (ED) overcrowding was reduced after the introduction of the 4-hour rule in Western Australia and whether any changes in overcrowding were associated with significant changes in patient mortality rates. Design, setting and patients: Quasi-experimental intervention study using dependent pretest and post-test samples. Hospital and patient data were obtained for three tertiary hospitals and three secondary hospitals in Perth, WA, for 2007-08 to 2010-11. Main outcome measures: Mortality rates; overcrowding rates. Results: No change was shown in mortality from 2007-08 to 2010-11 for the secondary hospitals and from 2007-08 to 2009-10 for the tertiary hospitals. ED overcrowding (as measured by 8-hour access block) at the tertiary hospitals improved dramatically, falling from above 40% in July 2009 to around 10% by early 2011, and presentations increased by 10%, while the mortality rate fell significantly (by 13%; 95% CI, 7%-18%; P < 0.001) from 1.12% to 0.98% between 2009-10 and 2010-11. Monthly mortality rates decreased significantly in two of the three tertiary hospitals concurrently with decreased access block and an increased proportion of patients admitted in under 4 hours. Conclusion: Introduction of the 4-hour rule in WA led to a reversal of overcrowding in three tertiary hospital EDs that coincided with a significant fall in the overall mortality rate in tertiary hospital data combined and in two of the three individual hospitals. No reduction in adjusted mortality rates was shown in three secondary hospitals where the improvement in overcrowding was minimal.

Foster R.S.,Princess Margaret Hospital for Children | Bint L.J.,Princess Margaret Hospital for Children | Halbert A.R.,Princess Margaret Hospital for Children
Australasian Journal of Dermatology | Year: 2012

Tuberous sclerosis complex (TSC) is an autosomal dominant genodermatosis characterised by the development of hamartomatous tumours in multiple organs including the brain, skin, kidneys, heart and lungs. Facial angiofibromas are the most visible and unsightly of the cutaneous manifestations of TSC, often resulting in stigmatisation for both the affected individuals and their families. Current treatments include vascular laser, ablative lasers and other destructive techniques such as shave excision and electrodessication. For the best outcome these treatments have to be repeated throughout childhood and teenage years, necessitating multiple general anaesthetics. We report a pilot study of topical rapamycin in four children with TSC and facial angiofibromas. Two patients were trialled on 0.1% rapamycin in petrolatum and the other two patients with 0.1% rapamycin solution (Rapamune) applied topically. Both preparations were rapidly and equally effective, however the 0.1% in petrolatum was much better tolerated. Younger patients with smaller angiofibromas had the best response with near complete clearance. Both preparations were more cost effective than pulsed dye laser under general anaesthesia. Although larger studies are needed, this treatment shows a potential to be a first-line management for facial angiofibromas in TSC and appears safe to start in early childhood. © 2011 The Australasian College of Dermatologists.

Watson H.J.,Center for Clinical Interventions | Watson H.J.,Princess Margaret Hospital for Children | Watson H.J.,University of Western Australia | Bulik C.M.,University of North Carolina at Chapel Hill
Psychological Medicine | Year: 2013

Background Anorexia nervosa is a potentially deadly psychiatric illness that develops predominantly in females around puberty but is increasingly being recognized as also affecting boys and men and women across the lifespan. The aim of this environmental scan is to provide an overview of best practices in anorexia nervosa treatment across the age spectrum. Method A triangulation approach was used. First, a detailed review of randomized controlled trials (RCTs) for anorexia nervosa published between 1980 and 2011 was conducted; second, clinical practice guidelines were consulted and reviewed; third, information about RCTs currently underway was sourced. This approach facilitated a comprehensive overview, which addressed the extant evidence base, recent advances in evidence and improvements in treatment, and future directions. Results The evidence base for the treatment of anorexia nervosa is advancing, albeit unevenly. Evidence points to the benefit of family-based treatment for youth. For adults no specific approach has shown superiority and, presently, a combination of renourishment and psychotherapy such as specialist supportive clinical management, cognitive behavioral therapy, or interpersonal psychotherapy is recommended. RCTs have neither sufficiently addressed the more complex treatment approaches seen in routine practice settings, such as multidisciplinary treatment or level of care, nor specifically investigated treatment in ethnically diverse populations. Methodological challenges that hinder progress in controlled research for anorexia nervosa are explained. Conclusions The review highlights evidence-based and promising treatment modalities for anorexia nervosa and presents a triangulated analysis including controlled research, practice guidelines, and emerging treatments to inform and support clinical decision making. Copyright © Cambridge University Press 2012.

Prescott S.,Princess Margaret Hospital for Children | Nowak-Wgrzyn A.,Mount Sinai School of Medicine
Annals of Nutrition and Metabolism | Year: 2011

The need for allergy prevention strategies has never been greater. Surging rates of food allergy and eczema are now adding to the already substantial burden of asthma and respiratory allergic diseases. The parallel rise in many other immune diseases suggests that the developing immune system is highly vulnerable to modern environmental changes. These strong environmental pressures may be one reason why simple allergen avoidance strategies have not been successful. Another more recent strategy to curtail the allergy epidemic has been to identify factors associated with modern lifestyle that may be causally linked with allergic disease, in an attempt to restore more favourable conditions for immune tolerance during early development. More hygienic conditions and disruption of microbial exposure have prompted strategies to restore this balance using probiotic and prebiotic supplements. Modern dietary changes linked with allergic diseases have prompted supplementation studies to assess the preventive merits of specific immunomodulatory dietary nutrients such as polyunsaturated fatty acids. Other nutrients such as antioxidants, folate, and vitamin D are also currently under investigation. Modern environmental pollutants have also been associated with adverse effects on immune development and the risk of disease. While many of these avenues have provided some promise, they have not yet translated into specific recommendations. Current evidence-based guidelines for allergy prevention remain limited to avoidance of cigarette smoke, promotion of breastfeeding and the use of hydrolysed formula when breastfeeding is not possible. Allergen avoidance strategies have been largely removed from most guidelines. It is hoped that a number of ongoing studies will help provide clearer recommendations around the use of probiotics, prebiotics, specific dietary nutrients and the role of early introduction of allergenic foods for the promotion of tolerance. Despite the current uncertainties, prevention remains the best long-term strategy to reduce the growing burden of allergic disease. Copyright © 2011 S. Karger AG, Basel.

Dobrovoljac M.,Princess Margaret Hospital for Children | Geelhoed G.C.,Princess Margaret Hospital for Children
EMA - Emergency Medicine Australasia | Year: 2012

Objective: For children with croup controversy remains over dosage and time to onset of action of oral steroids. The Cochrane Collaboration and other reviews have suggested 0.6mg/kg dexamethasone be used (despite some evidence that 0.15mg/kg is effective) with no expectation of benefit before 4-6h. This randomized double-blinded clinical trial examines whether 0.15mg/kg dexamethasone works by 30min. Methods: Children with croup aged above 6months presenting to a tertiary paediatric ED with a Westley croup score of mild to moderate range (scores 1-6 out of 17) were randomized to receive either 0.15mg/kg dexamethasone or oral placebo solution. Vital signs and croup score were recorded at study entry and every 10min up to 1h after administration of the study drug. The main outcome measure was croup score at 30min. Results: Each group contained 35 children. Baseline characteristics were similar, except for respiratory rate, which was higher in the placebo group. There was a growing trend to a lower croup score in the dexamethasone group, evident from 10min and statistically significant from 30min. Conclusion: For children with croup an oral dose of 0.15mg/kg dexamethasone offers benefit by 30min, much earlier than the 4h suggested by the Cochrane Collaboration. This result might encourage doctors to treat more children with all severities of croup being less worried about potential side-effects and delayed benefit. © 2011 The Authors. EMA © 2011 Australasian College for Emergency Medicine and Australasian Society for Emergency Medicine.

Forbes D.,University of Western Australia | Forbes D.,Princess Margaret Hospital for Children
Journal of Paediatrics and Child Health | Year: 2013

Infantile gastroesophageal reflux is challenging because of the difficulties in differentiating normal developmental physiology from disease, the imprecision of diagnostic tests and the paucity of evidence-based therapies for clinicians to implement. Careful clinical assessment is central to avoidance of inappropriate diagnosis and utilisation of ineffective or dangerous therapies, and at the same time, identification of those infants who warrant intervention. © 2012 The Author. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Sly P.D.,University of Queensland | Gangell C.L.,University of Queensland | Chen L.,University of Queensland | Ware R.S.,University of Queensland | And 7 more authors.
New England Journal of Medicine | Year: 2013

Background: Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive. We sought to determine risk factors for the onset of bronchiectasis, using data collected by the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) intensive surveillance program. Methods: We examined data from 127 consecutive infants who received a diagnosis of cystic fibrosis after newborn screening. Chest computed tomography (CT) and bronchoalveolar lavage (BAL) were performed, while the children were in stable clinical condition, at 3 months and 1, 2, and 3 years of age. Longitudinal data were used to determine risk factors associated with the detection of bronchiectasis from 3 months to 3 years of age. Results: The point prevalence of bronchiectasis at each visit increased from 29.3% at 3 months of age to 61.5% at 3 years of age. In multivariate analyses, risk factors for bronchiectasis were presentation with meconium ileus (odds ratio, 3.17; 95% confidence interval [CI], 1.51 to 6.66; P = 0.002), respiratory symptoms at the time of CT and BAL (odds ratio, 2.27; 95% CI, 1.24 to 4.14; P = 0.008), free neutrophil elastase activity in BAL fluid (odds ratio, 3.02; 95% CI, 1.70 to 5.35; P<0.001), and gas trapping on expiratory CT (odds ratio, 2.05; 95% CI, 1.17 to 3.59; P = 0.01). Free neutrophil elastase activity in BAL fluid at 3 months of age was associated with persistent bronchiectasis (present on two or more sequential scans), with the odds seven times as high at 12 months of age and four times as high at 3 years of age. Conclusions: Neutrophil elastase activity in BAL fluid in early life was associated with early bronchiectasis in children with cystic fibrosis. (Funded by the National Health and Medical Research Council of Australia and Cystic Fibrosis Foundation Therapeutics.) Copyright © 2013 Massachusetts Medical Society. All rights reserved.

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