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O'Regan G.M.,Our Ladys Childrens Hospital | Irvine A.D.,Our Ladys Childrens Hospital | Irvine A.D.,Trinity College Dublin
Clinical and Experimental Allergy | Year: 2010

Atopic dermatitis (AD) is an inflammatory disease characterized by pruritic skin lesions, immunodysregulation, disrupted epidermal barrier function and IgE-mediated sensitization to food and environmental allergens. Identification of the aetiology of AD has become increasingly a priority, as it is clear that the disease burden exceeds AD alone, with many children suffering severe, multi-system and occasionally life-threatening allergic disease. Previous approaches to understanding AD have centred on mechanisms in the adaptive immune system, often with an emphasis on the Th1-Th2 paradigm. Recently, the conceptual focus has increasingly shifted to include a primary defect in the epithelial barrier as a threshold event in moderate-to-severe AD. Familial aggregation of the disease is well established through many family studies of AD, asthma and allergic rhinitis, suggesting a significant heritable component. The identification of loss-of-function mutations in the filaggrin (FLG) gene, whose product is a key structural protein in the outermost layer of the epidermis in up to 50% of patients with AD, provides a significant insight into explaining disease initiation and points to a complex secondary interplay of environmental and immunological sequelae once barrier disruption is established. The elucidation of the environmental, genetic and immunobiological modifiers of this structural molecule may also direct our understanding of the pathomechanisms and endotypes central to the atopic diathesis. The recent identification of a murine model for FLG-AD, with the detection of a homozygous frame-shift mutation in the Flg gene in flaky-tail (ft/ft) mice, stands to rapidly accelerate our understanding of mechanisms and therapeutic intervention points in AD. Refining the molecular understanding of AD and its subtypes will allow for specific diagnostic, treatment and ultimately, preventative algorithms, and has opened an exciting new world of investigative challenges and collaborations. © 2010 Blackwell Publishing Ltd.

Kavanagh R.G.,Our Ladys Childrens Hospital
The Journal of bone and joint surgery. American volume | Year: 2013

Pediatric orthopaedic surgery owes its development to many pioneering individuals, and the studies that these individuals have undertaken form the basis for the clinical decisions made on the modern pediatric orthopaedic service. The aim of our study was to use citation analysis to identify the top 100 papers in pediatric orthopaedic surgery. Using the Thomson Reuters Web of Knowledge, we searched for citations of all papers relevant to pediatric orthopaedics. The number of citations, authorship, year of publication, journal of publication, and country and institution of origin were recorded for each paper. The most cited paper was found to be the classic paper from 1963 by Salter and Harris that introduced the now-eponymous classification system for physeal injuries in the skeletally immature patient. The second most cited was Salter's paper describing the widely used osteotomy for the treatment of developmental dysplasia of the hip, and the third most cited was Catterall's description of the natural history of Legg-Calvé-Perthes disease. Most papers originated in the U.S., and most were published in this journal. A number of authors including Salter, Ponseti, Graf, and Loder had more than one paper in the top-100 list. This paper's identification of the classic papers of pediatric orthopaedic surgery gives us a unique insight into the development of pediatric orthopaedic surgery in the twentieth and early twenty-first centuries and identifies those individuals who have contributed the most to the body of knowledge used to guide evidence-based clinical decision-making in pediatric orthopaedics today.

McNicholas F.,Our Ladys Childrens Hospital
European Child and Adolescent Psychiatry | Year: 2012

Two factors predict treatment outcome, how effective the treatment is and whether the patient takes or follows the treatment plan. As clinicians or scientists, we strive to develop newer and more effective treatments, both pharmacological and non-pharmacological to improve treatment outcome in our patient population. Adherence is the single most modifiable factor associated with treatment outcome, yet how often is the issue of adherence addressed in clinical consultations? The best treatment is rendered useless if not adhered to. Initial adherence rates are low and get worse with time, but methodological difficulties in studies make it difficult to determine both the clinical implication of suboptimal adherence and successful strategies. Further research should apply more rigour to the area of definition and measurement, be sufficiently powered and long term, and measure possible confounders, to allow for an understanding on the link and impact between adherence and clinical outcome. This article reviews some of the main issues with regard to adherence and cost implications of suboptimal adherence and suggests future directions. © 2012 Springer-Verlag.

Cox D.W.,Our Ladys Childrens Hospital
Irish medical journal | Year: 2011

The prevalence of Methicillin Resistant Staphylococcus Aureus (MRSA) in patients with Cystic Fibrosis (CF) has risen dramatically over the past 10 years. The clinical significance of MRSA in CF patients remains undetermined. We conducted a review of patients with CF infected with MRSA over a 10 year period at Our Lady's Children's Hospital, Crumlin between 1999 and 2009. We collected data from 24 patients infected with MRSA and 24 control patients without MRSA There was a significant difference between the two groups in the rate of decline in percentage FEV1 two years after MRSA infection (Difference: -17.4, 95% CI: -30.48, -4.31, p = 0.01). A similar trend was seen for FVC% and FEF25-75% predicted. This study suggests that persistent MRSA infection in the airways of children with CF is associated with diminished lung function two years post acquisition, when compared to a matched control cohort without MRSA.

Lyons B.,Our Ladys Childrens Hospital
Irish Medical Journal | Year: 2013

On November 29, 2011 Dr Conrad Murray was sentenced to four years in prison after being convicted of the involuntary manslaughter of Michael Jackson. Expert witness statements indicated that Murray's actions were an "extreme departure from the standard of care, particularly with regard to (1) inappropriately treating insomnia with a surgical anaesthetic (propofol); (2) failing to acquire sufficiently informed consent; (3) administering propofol without the necessary monitoring equipment; (4) delaying contacting the emergency services; and (5) making ineffective resuscitation efforts. Further medical evidence argued that Murray s care of Jackson contained" 17 egregious violations, defined as acts that posed a foreseeable danger to the patient's life. These deficiencies, it was stated, constituted gross negligence.

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