title: Sinopulmonary Complications in Subjects With Primary Immunodeficiency.
abstract: BACKGROUND
  The aim of this work was to describe the frequency and spectrum of sinopulmonary complications among subjects with primary immunodeficiency disorders.
  
  
  METHODS
  The subjects included all patients with primary immunodeficiency who were registered prospectively between January 2004 and December 2013 in the Kuwait National Primary Immunodeficiency Disorders Registry.
  
  
  RESULTS
  A total of 202 subjects were registered during the study period. Subjects with combined immunodeficiencies were the most prevalent (65 subjects, 32.1%), followed by well-defined syndromes with immunodeficiency (45 subjects, 22.2%) and predominantly antibody deficiencies (35 subjects, 17.3%). A total of 295 sinopulmonary manifestations were observed in 127 subjects (63%); 157 manifestations (53.2%) were observed among the presenting symptoms, and 138 manifestations (46.8%) occurred after establishment of the primary immunodeficiency disorder diagnosis. Sinopulmonary manifestations were more common in subjects with predominantly antibody deficiencies (2.3 manifestations/subject), followed by subjects with combined immunodeficiencies (1.75 manifestations/subject). Pneumonia was the most common manifestation (108 episodes affecting 80 subjects), followed by otitis media (81 episodes affecting 59 subjects), bronchiectasis in 28 subjects (13.8%), and asthma in 22 subjects (11%). Microbial organisms were isolated during 46 episodes of pneumonia (42.5%) (cytomegalovirus and Pneumocystis jirovecii were the most common). There were 57 deaths (28%) during the study period. Twenty-four deaths (42%) were due to pulmonary complications as follows: pneumonia (16 subjects, 8%), pulmonary hemorrhage (6 subjects, 3%), and aspiration pneumonia (2 subjects, 1%).
  
  
  CONCLUSIONS
  Sinopulmonary complications are common in subjects with primary immunodeficiency. They can be serious and continue to occur even after proper treatment is initiated. The pulmonologist should play an important role in the management of subjects with primary immunodeficiency disorder.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait. Pulmonary Unit, Department of Pediatrics, Mubarak Hospital, Kuwait City, Kuwait.
authors.forename: Abdullah
authors.initials: A
authors.lastname: Owayed
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait. Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, Kuwait. wemh@hotmail.com.
authors.forename: Waleed
authors.initials: W
authors.lastname: Al-Herz
mesh_terms: D001249:Asthma / Q000453:epidemiology / Q000276:immunology; D001987:Bronchiectasis / Q000453:epidemiology / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D007231:Infant, Newborn; D007730:Kuwait / Q000453:epidemiology; D008137:Longitudinal Studies; D008171:Lung Diseases / Q000453:epidemiology* / Q000276:immunology; D008297:Male; D010033:Otitis Media / Q000453:epidemiology / Q000276:immunology; D011014:Pneumonia / Q000453:epidemiology / Q000276:immunology; D015995:Prevalence; D011446:Prospective Studies; D012042:Registries; D012140:Respiratory Tract Diseases / Q000453:epidemiology* / Q000276:immunology
keywords: asthma; bronchiectasis; complications; pneumonia; primary immunodeficiency