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      Hospital Admissions in Children with Down Syndrome: Experience of a Population-Based Cohort Followed from Birth

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          Abstract

          Objective

          Children with Down syndrome, the most common genetic cause of intellectual disability, are prone to multiple and varied health-related problems. This study describes patterns of hospitalisations for children and young people with Down syndrome in Western Australia.

          Methods

          Birth records were linked to the Western Australian population-based Intellectual Disability database to identify all children born with Down syndrome in Western Australia between 1 January, 1983 and 31 December, 1999. These records were linked to the Hospital Morbidity Data System to provide information on all hospitalisations up to 31 December, 2004. Hospitalisation data, coded using ICD-9CM or ICD-10 (v0.5) were grouped into clinically relevant categories using the primary diagnosis. Rates of hospital admission for all and specific diagnoses were expressed in 1000-person-years at-risk and median age at first admission and length of stay were calculated.

          Results

          Of the 405 children, 395 had one or more hospital admissions, totalling 3786 admissions for all children and an estimated 39.5 person-years in hospital. On average, children were admitted 9.7 times, with an estimated rate of 757.2 admissions per 1000pyr (95% CI: 680, 843). A quarter of all admissions occurred in the first year of life. The average hospital length of stay was 3.8 days (95% CI: 3.7, 4.1). Upper respiratory tract conditions affected the most children (58.5%) and accounted for 12.1% of all admissions. Other disorders which affected a high percentage of children were ear/hearing conditions (50.6%), disorders of the oral cavity (38.0%) and lower respiratory tract conditions (37.5%). Overall, children with Down syndrome were hospitalised at a rate five times (95% CI = 4.3–6.2) that of the general population.

          Conclusion

          Children with Down syndrome are at increased risk of morbidity for varied causes underlining the importance of comprehensive and targeted primary care for this group.

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          Most cited references33

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          Infections and immunodeficiency in Down syndrome.

          Down syndrome (DS) is the most common genetic disease and presents with cognitive impairment, cardiac and gastrointestinal abnormalities, in addition to other miscellaneous clinical conditions. DS individuals may have a high frequency of infections, usually of the upper respiratory tract, characterized by increased severity and prolonged course of disease, which are partially attributed to defects of the immune system. The abnormalities of the immune system associated with DS include: mild to moderate T and B cell lymphopenia, with marked decrease of naive lymphocytes, impaired mitogen-induced T cell proliferation, reduced specific antibody responses to immunizations and defects of neutrophil chemotaxis. Limited evidence of genetic abnormalities secondary to trisomy of chromosome 21 and affecting the immune system is available, such as the potential consequences of gene over-expression, most significantly SOD1 and RCAN1. Secondary immunodeficiency due to metabolic or nutritional factors in DS, particularly zinc deficiency, has been postulated. Non-immunological factors, including abnormal anatomical structures (e.g. small ear canal, tracheomalacia) and gastro-oesophageal reflux, may play a role in the increased frequency of respiratory tract infections. The molecular mechanisms leading to the immune defects observed in DS individuals and the contribution of these immunological abnormalities to the increased risk of infections require further investigation. Addressing immunological and non-immunological factors involved in the pathogenesis of infectious diseases may reduce the susceptibility to infections in DS subjects. © 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology.
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            Population-based linkage of health records in Western Australia: development of a health services research linked database.

            To introduce the Western Australian Health Services Research Linked Database as infrastructure to support aetiologic, utilisation and outcomes research. To compare the study population, data resources, technical systems and organisational supports with international best practice in record linkage and health research. The WA Linked Database systematically links the available administrative health data within an Australian State of 1.7 million people. It brings together, initially, six core data elements (birth records, midwives' notifications, cancer registrations, in-patient hospital morbidity, in-patient and public out-patient mental health services data and death records). It will be updated regularly and is designed, in future extensions, to include data on primary, residential and domiciliary care and health surveys. Linkage uses probabilistic matching of patient names and other identifiers. Geocodes for spatial analysis are assigned using address linkage and mapping software. By June 1997, the project had taken 2 1/2 years to develop the system and link seven million core data records from 1980 to 1995. The system is consistent with international benchmarks, from four centres of excellence, for the study population, core datasets, matching and geocoding, and collaborative networks. There are prospects to redress deficiencies in primary medical contact and other data resources, validation studies, tracing systems and a more supportive legal framework. The WA Linked Database will be used in combination with medical record audits to provide a comprehensive evaluation of health system performance.
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              Down syndrome: a novel risk factor for respiratory syncytial virus bronchiolitis--a prospective birth-cohort study.

              Respiratory syncytial virus is the single-most important cause of lower respiratory tract infections in children. Preterm birth and congenital heart disease are known risk factors for severe respiratory syncytial virus infections. Although Down syndrome is associated with a high risk of respiratory tract infections, little is known about the incidence of respiratory syncytial virus infections in this group. The aim of our study was to determine the incidence of respiratory syncytial virus lower respiratory tract infection-associated hospitalization among children with Down syndrome. We performed a retrospective observational study and a prospective nationwide birth-cohort study of children with Down syndrome. The retrospective cohort comprised 176 children with Down syndrome. A birth cohort of 219 children with Down syndrome was prospectively followed until 2 years of age. All 276 siblings of the birth cohort were used as controls. Of the 395 patients with Down syndrome, 180 (45.6%) had a known risk factor for severe respiratory syncytial virus infections; 39 (9.9%) of these were hospitalized for respiratory syncytial virus lower respiratory tract infections. Two control children (0.7%) versus 9 term children with Down syndrome without congenital heart disease (7.6%) were hospitalized for respiratory syncytial virus lower respiratory tract infections. The median duration of hospitalization was 10 days; mechanical ventilation was required for 5 children (12.8%). This is the first study, to our knowledge, to demonstrate that Down syndrome is a novel independent risk factor for severe respiratory syncytial virus lower respiratory tract infections. These findings should prompt studies to investigate possible mechanisms that underlie severe respiratory syncytial virus lower respiratory tract infections in children with Down syndrome. The effect of respiratory syncytial virus prophylaxis in this specific population needs to be established.
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                Author and article information

                Contributors
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, USA )
                1932-6203
                2013
                13 August 2013
                : 8
                : 8
                : e70401
                Affiliations
                [1]Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Crawley, Western Australia, Australia
                IGBMC/ICS, France
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Conceived and designed the experiments: HL JB PF. Analyzed the data: PF GH. Wrote the paper: PF GH HL JB TP.

                Article
                PONE-D-13-07247
                10.1371/journal.pone.0070401
                3742744
                23967074
                58c569c7-9355-43c1-9bab-fbabc4c447a9
                Copyright @ 2013

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 8 February 2013
                : 18 June 2013
                Page count
                Pages: 10
                Funding
                Funding was provided by the Australian National Health and Medical Research Council (NHMRC, http://www.nhmrc.gov.au/) Program Grants 572742, NHMRC Research Fellowship (572568) (to HL). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Medicine
                Clinical Genetics
                Chromosomal Disorders
                Down Syndrome
                Clinical Research Design
                Cohort Studies
                Epidemiology
                Non-Clinical Medicine
                Health Care Policy
                Child and Adolescent Health Policy
                Health Statistics
                Health Informatics
                Pediatrics
                Adolescent Medicine
                Public Health
                Child Health

                Uncategorized
                Uncategorized

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