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      Respiratory Health and Related Quality of Life in Patients with Congenital Agammaglobulinemia in the Northern Region of the UK

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          Abstract

          Introduction

          Patients with congenital agammaglobulinemia, characterized by a defect in B lymphocyte differentiation causing B alymphocytosis, require life-long IgG replacement. There is scant literature regarding the effectiveness of IgG treatment at preventing mucosal (particularly sinopulmonary tract) infection and whether current management adequately restores “normal” health and quality of life (QoL). We aimed to document infective episodes pre- and post-commencing IgG replacement, determine any change in lung function and structure and assess respiratory status and QoL in a cohort of patients treated in Newcastle.

          Methods

          Clinical data were extracted from medical records of 15 patients identified from the immunology database, focusing on infective episodes, serial chest CT and spirometry results. Thirteen patients completed a selection of standardized and validated questionnaires assessing physical health, respiratory health and QoL.

          Results

          Pediatric patients on IgG therapy suffered fewer infections per patient year (0.74) than adults (2.13). 6/14 patients showed deteriorating respiratory status despite adequate therapy. Health questionnaires revealed a significant burden of respiratory disease on a patient’s life.

          Conclusion

          Clinical data showed patients with congenital agammaglobulinemia receiving immunoglobulin therapy retained a higher than average infection rate, most of which affected mucosal barriers. Most patients self-reported worse respiratory symptoms, a lower respiratory-related QoL and a lower general health QoL relative to a healthy population. Most participants had progressive structural lung damage and decreased lung function. These results suggest that current management is not entirely effective at preventing deterioration of respiratory health or restoring QoL.

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          Most cited references22

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          Agammaglobulinemia.

          C Bruton (1952)
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            Interpretation of quality of life scores from the St George's Respiratory Questionnaire.

            The aim of the study was to obtain the general population norms for the St. George's Respiratory Questionnaire (SGRQ), a specific questionnaire for respiratory diseases. The IBERPOC project was a cross-sectional study of representative samples of the general population aged between 40-69 yrs. The study sample was composed of 862 individuals. All participants considered as "probable cases" of chronic obstructive pulmonary disease (COPD) (n=460) were eligible to complete the SGRQ and among the rest of the nonprobable COPD participants (n=3,571), 10 individuals from each defined age and sex group were eligible (n=402). Weights were applied to restore general population representativity of the sample. Mean forced expiratory volume in one second (FEV1) predicted was 89.4% (SD=16.5%; range: 16-131%). Chronbach's alpha coefficients were >0.7 in the symptoms, activity and impact scales, and >0.9 in the overall scale. Symptom scale score was significantly higher among males (11.6 versus 7.8; p<0.01) and activity scale score was significantly higher among females (12.2 versus 14.6; p=0.04). In a multiple linear regression model, respiratory diseases (asthma and COPD) and FEV1 % over pred showed the strongest association with the SGRQ total score. Smoking, sex, age and education were independently associated with the total SGRQ score. These results indicate that individuals from the general population presented some of the problems that are important when measuring health-related quality of life in respiratory patients, and provide St George's Respiratory Questionnaire norms, a useful method for interpreting the St George's Respiratory Questionnaire score in a given patient or study samples.
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              Assessment of the SF-36 version 2 in the United Kingdom.

              To introduce the UK SF36 Version II (SF36-II), and to (a) gain population norms for the UK SF36-II in a large community sample as well as to explore the instrument's internal consistency reliability and construct validity, and (b) to derive the Physical Component Summary (PCS) and Mental Component Summary (MCS) algorithms for the UK SF36-II. Postal survey using a questionnaire booklet, containing the SF-36-II and questions on demographics and long term illness. The sample was drawn from General Practitioner Records held by the Health Authorities for Berkshire, Buckinghamshire, Northamptonshire, and Oxfordshire. The questionnaire was sent to 13,800 randomly selected subjects between the ages of 18-64 inclusive. Scores for the eight dimensions of the UK SF36-II and the PCS and MCS summary scores. The survey achieved a response rate of 64.4% (n = 8889). Internal consistency of the different dimensions of the questionnaire were found to be high. Normative data for the SF-36 are reported, broken down by age and sex, and social class. Factor analysis of the eight domains produced a two factor solution and provided weights for the UK SF36-II. The SF36-II domains were shown to have improved reliability over the previous version of the UK SF36. Furthermore, enhancements to wording and response categories reduces the extent of floor and ceiling effects in the role performance dimensions. These advances are likely to lead to better precision as well as greater responsiveness in longitudinal studies.
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                Author and article information

                Contributors
                0191 2825234 , a.r.gennery@ncl.ac.uk
                Journal
                J Clin Immunol
                J. Clin. Immunol
                Journal of Clinical Immunology
                Springer US (New York )
                0271-9142
                1573-2592
                18 April 2016
                18 April 2016
                2016
                : 36
                : 472-479
                Affiliations
                [ ]Institute of Cellular Medicine, Newcastle University, 4th Floor, William Leech Building, Medical School, Framlington Place, Newcastle upon Tyne, E2 4HH UK
                [ ]Paediatric Immunology, Great North Children’s Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP UK
                [ ]Great North Children’s Hospital, Clinical Resource Building, Level 4, Block 2, Newcastle upon Tyne, NE1 4LP UK
                Article
                284
                10.1007/s10875-016-0284-3
                4896978
                27091141
                0269c268-4e97-4acc-80de-530e517b0f6a
                © The Author(s) 2016

                Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                : 8 January 2016
                : 5 April 2016
                Categories
                Original Article
                Custom metadata
                © Springer Science+Business Media New York 2016

                Immunology
                xla,agammaglobulinemia,immunoglobulin,bronchiectasis,quality of life
                Immunology
                xla, agammaglobulinemia, immunoglobulin, bronchiectasis, quality of life

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