Our Ladys Childrens Hospital Crumlin

Dublin, Ireland

Our Ladys Childrens Hospital Crumlin

Dublin, Ireland
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Kennedy J.,Our Ladys Childrens Hospital Crumlin
Journal of Pediatric Orthopaedics | Year: 2017

BACKGROUND:: Tibial pseudoarthrosis is a source of considerable morbidity in children with neurofibromatosis. Preventing the progression of anterolateral bowing of the tibia (ALBT) to tibial pseudoarthrosis is difficult. The aim of this paper is to report the successful use of guided growth to prevent tibial pseudoarthrosis in a child with neurofibromatosis. METHODS:: With local ethical approval, we reviewed the case notes and radiographs of a child with pronounced ALBT who was treated with guided growth to correct the deformity and prevent fracture. RESULTS:: At 4-year follow-up guided growth has successfully corrected the deformity and prevented tibial pseudoarthrosis. CONCLUSIONS:: This is the first report of the use of guided growth to prevent the progression of ALBT to pseudoarthrosis of the tibia in a child with neurofibromatosis. LEVEL OF EVIDENCE:: Level IV. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Lee M.,Our Ladys Childrens Hospital Crumlin
Journal of Pediatric Orthopaedics Part B | Year: 2017

Botulinum intramuscular injections are increasingly being used in the management of hypertonic musculature. Historically, injections were administered under general anaesthesia (GA), which has service and economic implications. Our delivery changed to outpatient conscious injections to improve service efficiency. A retrospective analysis of all patient injections from January 2010 to December 2015 analysed cost–benefit and efficiency of service remodelling. 472 patients were administered injections, 298 in theatre under GA and 174 without GA. In 2010, 97 of 102 cases were performed under GA (45 theatre-hours), decreasing to 18 of 68 cases in 2015 (8.4 theatre-hours). The mean hospital stay decreased from 410 to 135 min. The reduction in theatre use and the requirement for a postanaesthetic bed led to significant savings per case. Service remodelling showed successful cost saving and reduced in-hospital stay. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.

Semple D.,Our Ladys Childrens Hospital Crumlin | Howlett M.,Our Ladys Childrens Hospital Crumlin
Archives of disease in childhood | Year: 2016

AIM: Since the discontinuation of a commercially available liquid calcium medicine, there have been various local strategies to obtain adequate calcium levels, particularly in suspected 22Q11.2 deletion syndrome children post cardiothoracic surgery. Pharmacy obtained on special order a Calcium Liquid food supplement (0.5 mmol calcium/ml) for use in those children whom effervescent tablets did not appear to improve corrected calcium. Anecdotally it was thought that calcium liquid was better tolerated and absorbed, resulting in quicker recovery time of corrected calcium than use of the effervescent tablets. This review of patients was intended to determine if the use of the liquid was associated with a significant improvement in calcium levels compared to the use of tablets.METHOD: A dispense report was undertaken to identify those patients supplied with the calcium liquid product from May 2014 to May 2015.Using the electronic prescribing system, the patients electronic health record was accessed, and the following information recorded; calcium, albumin, intake calcium in fluids, TPN, oral and Intravenous. The data was collected for seven days previous to the first administration of calcium liquid and seven days after where available. Corrected calcium was calculated using an accepted method (calcium=serum calcium+0.02* (normal albumin - patient albumin)).RESULTS: Nine children supplied with calcium liquid between May 2014 and May 2015. Four patients who did not have histories on the electronic prescribing system were excluded for ease of analysis. The electronic medication record of the five remaining patients was used to compare calcium intake from all sources including fluids and feed.As expected calcium levels (and corrected calcium level where relevant) did improve when calcium liquid was added in. However four of the patients received calcium from multiple sources. Of these three received more calcium (mmol/kg/day) in fluids and feeds than from the administered pharmacy product.One single patient did not receive calcium from any other sources (fluids/TPN/feed). However there did not appear to be a significant change in calcium levels when calcium liquid was introduced as opposed to calcium tablets.CONCLUSION: Although based on a small number of patients, from this experience it would not seem that the calcium liquid obtained by pharmacy results in higher calcium serum levels.Further analysis may be required to distinguish the effects from calcium intake in feed/fluid and calcium intake from medicines. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Agency: European Commission | Branch: H2020 | Program: RIA | Phase: PHC-18-2015 | Award Amount: 8.19M | Year: 2016

Liver cancer in the paediatric population is rare with an incidence approximately 1-1.5 per million population. The commonest tumour seen in the childhood population is hepatoblastoma (HB), usually seen in young children and infants. Much rarer (about 10% of paediatric liver cancers) is hepatocellular carcinoma (HCC), usually seen in the teenage population and sometimes associated with underlying cirrhotic liver diseases. The ChiLTERN project relates to topic PHC 18 establishing effectiveness of health care interventions in the paediatric population. The ChiLTERN project builds on a unique opportunity to undertake a comprehensive research programme linked to an ambitious global partnership which will see the single largest clinical trial (the Paediatric Hepatic International Tumour Trial - PHITT) ever undertaken in this population of patients, with several randomised questions in six subgroups of patients. ChiLTERN will allow us to move towards an era of personalised therapy in which each patient will receive the correct amount of chemotherapy and will undergo has the best surgical operation (surgical resection or liver transplant). By using both clinical and biological information, we can assign patients more accurately to risk groups based on their survival. Using genetic tests and biomarkers, we will determine those children who may be at risk of developing long term side effects (deafness, heart failure, kidney damage). In addition, biomarkers will allow us to monitor during therapy and detect toxicities early before serious damage is done so that we can adapt treatment and prevent these problems. Finally, we will be using imaging technology tools which will help our surgeons plan liver operations more safely and effectively. Ultimately ChiLTERN will allow us to cure more children with liver cancer, expose fewer children to toxic chemotherapy and ensure their surgery is both effective and safe.

Rowland M.,Dublin Academic Medical Center | Bourke B.,Our Ladys Childrens Hospital Crumlin | Bourke B.,University College Dublin
Current Opinion in Pulmonary Medicine | Year: 2011

Purpose of Review: This review looks at what is currently known about liver disease in cystic fibrosis (CF) in light of the literature over the past year, and what the ongoing challenges are from a clinical and research perspective for the optimal management of cystic fibrosis liver disease (CFLD). Recent Findings: Patients with CF who develop clinically significant liver disease have a worse overall phenotype, and whereas there is no definite evidence that they have a shorter life expectancy, longer follow-up is required to determine if liver disease is a risk factor for mortality in CF.The development of the ferret and pig animal models of CF with multiorgan involvement is an important breakthrough which will enhance our understanding of the pathogenesis of CFLD, and with which it is hoped novel therapeutic targets for the treatment of CFLD will be identified. Summary: Whereas there is still no effective treatment for liver disease in CF, recent developments of animal models of CFLD will enhance our capacity to develop new therapeutic targets and reduce the impact of liver disease on mortality in CF. © 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.

Linehan E.,Queen's University of Belfast | Dombrowski Y.,Queen's University of Belfast | Snoddy R.,Queen's University of Belfast | Fallon P.G.,St James's Hospital | And 3 more authors.
Aging Cell | Year: 2014

Aging results in deterioration of the immune system, which is associated with increased susceptibility to infection and impaired wound healing in the elderly. Phagocytosis is an essential process in both wound healing and immune defence. As such, age-related impairments in phagocytosis impact on the health of the elderly population. Phagocytic efficiency in peritoneal macrophages, bone marrow-derived macrophages and bone marrow monocytes from young and old mice was investigated. Aging significantly impaired phagocytosis by peritoneal macrophages, both in vitro and in vivo. However, bone marrow-derived macrophages and bone marrow monocytes did not exhibit age-related impairments in phagocytosis, suggesting no intrinsic defect in these cells. We sought to investigate underlying mechanisms in age-related impairments in phagocytosis by peritoneal macrophages. We hypothesized that microenvironmental factors in the peritoneum of old mice impaired macrophage phagocytosis. Indeed, macrophages from young mice injected into the peritoneum of old mice exhibited impaired phagocytosis. Proportions of peritoneal immune cells were characterized, and striking increases in numbers of T cells, B1 and B2 cells were observed in the peritoneum of old mice compared with young mice. In addition, B cell-derived IL-10 was increased in resting and LPS-activated peritoneal cell cultures from old mice. These data demonstrate that aging impairs phagocytosis by tissue-resident peritoneal macrophages, but not by bone marrow-derived macrophages/monocytes, and suggest that age-related defects in macrophage phagocytosis may be due to extrinsic factors in the tissue microenvironment. As such, defects may be reversible and macrophages could be targeted therapeutically in order to boost immune function in the elderly. © 2014 The Authors. Aging Cell published by the Anatomical Society and John Wiley & Sons Ltd.

We report a 2-year-old patient with Opitz-GBBB syndrome scheduled for a posterior sagittal anorectoplasty (PSARP). The ultrasound scan revealed the inferior end of dural sac just below sacrococcygeal membrane, although the patient had previously two successful caudal epidural blocks. Consequently, the epidural catheter was inserted under a real-time ultrasound guidance without dural puncture. Our patient had excellent pain relief without any side effects. © 2015 John Wiley & Sons Ltd.

Corcionivoschi N.,Our Ladys Childrens Hospital Crumlin
Cell host & microbe | Year: 2012

Reactive oxygen species (ROS) play key roles in mucosal defense, yet how they are induced and the consequences for pathogens are unclear. We report that ROS generated by epithelial NADPH oxidases (Nox1/Duox2) during Campylobacter jejuni infection impair bacterial capsule formation and virulence by altering bacterial signal transduction. Upon C. jejuni invasion, ROS released from the intestinal mucosa inhibit the bacterial phosphotyrosine network that is regulated by the outer-membrane tyrosine kinase Cjtk (Cj1170/OMP50). ROS-mediated Cjtk inactivation results in an overall decrease in the phosphorylation of C. jejuni outer-membrane/periplasmic proteins, including UDP-GlcNAc/Glc 4-epimerase (Gne), an enzyme required for N-glycosylation and capsule formation. Cjtk positively regulates Gne by phosphorylating an active site tyrosine, while loss of Cjtk or ROS treatment inhibits Gne activity, causing altered polysaccharide synthesis. Thus, epithelial NADPH oxidases are an early antibacterial defense system in the intestinal mucosa that modifies virulence by disrupting bacterial signaling. Copyright © 2012 Elsevier Inc. All rights reserved.

Ozaki E.,Trinity College Dublin | Campbell M.,Trinity College Dublin | Doyle S.L.,Trinity College Dublin | Doyle S.L.,Our Ladys Childrens Hospital Crumlin
Journal of Inflammation Research | Year: 2015

The inflammasome is a molecular platform formed by activation of an innate immune pattern recognition receptor seed, such as NLRP3. Once activated, NLRP3 recruits the adapter ASC (apoptosis-related speck-like protein containing a caspase recruitment domain), which in turn recruits procaspase-1. Procaspase-1 autocatalyzes its cleavage and activation, resulting in maturation of the precursor forms of interleukin (IL)-1β and IL-18 into active proinflammatory cytokines and initiation of pyroptotic cell death. The NLRP3 inflammasome has been implicated in the pathogenesis of a wide variety of diseases, including genetically inherited autoinflammatory conditions as well as chronic diseases in which NLRP3 is abnormally activated. The NLRP3 inflammasome has been linked to diseases such as Alzheimer’s disease, atherosclerosis, metabolic syndrome, and age-related macular degeneration. In this review, we describe the NLRP3 inflammasome complex and its activation in disease, and detail the current therapies that modulate either the NLRP3 inflammasome complex itself or the two cytokines it is responsible for activating, ie, IL-1β and IL-18. © 2015 Ozaki et al.

Irvine A.D.,Our Ladys Childrens Hospital Crumlin | Irvine A.D.,Trinity College Dublin
Journal of Investigative Dermatology | Year: 2014

In this issue of the Journal, Stout and colleagues report a novel and creative approach to replacement of genetically determined absence or deficiency of epidermal proteins. While these early data are certainly interesting, further validation work is required to determine the utility of this approach in genodermatoses. © 2014 The Society for Investigative Dermatology.

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