Sinopulmonary Complications in Subjects With Primary Immunodeficiency.

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 Dece...

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Publicado en:Respiratory Care Vol. 61; no. 8; pp. 1067 - 1073
Autores principales: Owayed, Abdullah, Al-Herz, Waleed
Formato: research tables/charts Journal Article
Publicado: Mary Ann Liebert, Inc. Aug2016
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Aug2016
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      pub: Mary Ann Liebert, Inc.
      place: New Rochelle, New York
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        10.4187/respcare.04479
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        atl: Sinopulmonary Complications in Subjects With Primary Immunodeficiency.
      aug:
        au:
          Owayed, Abdullah
          Al-Herz, Waleed
        affil: Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait
      sug:
        subj:
          Immunologic Diseases Complications
          Lung Diseases
          Human
          Registries, Disease
          Kuwait
          Record Review
          Immunologic Diseases Familial and Genetic
          Descriptive Research
          Descriptive Statistics
          Immunologic Diseases Epidemiology
          Prospective Studies
          Convenience Sample
          Age of Onset
          Immunologic Diseases Classification
          Otitis Media
          Pneumonia
          Asthma
          Bronchiectasis
          Sinusitis
          Lung Diseases, Interstitial
          Symptoms
      ab: 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.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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