title: Respiratory Health and Related Quality of Life in Patients with Congenital Agammaglobulinemia in the Northern Region of the UK.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Institute of Cellular Medicine, Newcastle University, 4th Floor, William Leech Building, Medical School, Framlington Place, Newcastle upon Tyne, E2 4HH, UK.
authors.forename: Branwen A
authors.initials: BA
authors.lastname: Bryan
authors.affiliation: Institute of Cellular Medicine, Newcastle University, 4th Floor, William Leech Building, Medical School, Framlington Place, Newcastle upon Tyne, E2 4HH, UK.
authors.forename: Alex
authors.initials: A
authors.lastname: Battersby
authors.affiliation: Institute of Cellular Medicine, Newcastle University, 4th Floor, William Leech Building, Medical School, Framlington Place, Newcastle upon Tyne, E2 4HH, UK.
authors.forename: Benjamin Martin James
authors.initials: BM
authors.lastname: Shillitoe
authors.affiliation: Paediatric Immunology, Great North Children's Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK.
authors.forename: Dawn
authors.initials: D
authors.lastname: Barge
authors.affiliation: Paediatric Immunology, Great North Children's Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK.
authors.forename: Helen
authors.initials: H
authors.lastname: Bourne
authors.affiliation: Paediatric Immunology, Great North Children's Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK.
authors.forename: Terry
authors.initials: T
authors.lastname: Flood
authors.affiliation: Paediatric Immunology, Great North Children's Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Cant
authors.affiliation: Paediatric Immunology, Great North Children's Hospital, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK.
authors.forename: Catherine
authors.initials: C
authors.lastname: Stroud
authors.affiliation: Institute of Cellular Medicine, Newcastle University, 4th Floor, William Leech Building, Medical School, Framlington Place, Newcastle upon Tyne, E2 4HH, UK. a.r.gennery@ncl.ac.uk.|Great North Children's Hospital, Clinical Resource Building, Level 4, Block 2, Newcastle upon Tyne, NE1 4LP, UK. a.r.gennery@ncl.ac.uk.
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Gennery
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications / Q000453:epidemiology* / Q000628:therapy; D001402:B-Lymphocytes / Q000502:physiology*; D002454:Cell Differentiation; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D019468:Disease Management; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000453:epidemiology* / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D008991:Monitoring, Physiologic; D011788:Quality of Life; D012119:Respiration; D012129:Respiratory Function Tests; D012141:Respiratory Tract Infections / Q000209:etiology / Q000517:prevention & control*; D011795:Surveys and Questionnaires; D006113:United Kingdom; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous
keywords: XLA; agammaglobulinemia; bronchiectasis; immunoglobulin; quality of life