Pediatr Allergy Respir Dis.  2007 Dec;17(4):372-383.

Pulmonary Complications in Primary Immunodeficiency Diseases

Affiliations
  • 1Department of Pediatrics, Severance Childrens Hospital, College of Medicine, Yonsei University, Seoul, Korea. dskim6634@yumc.yonsei.ac.kr

Abstract

PURPOSE:Primary immunodeficiency diseases are disorders in which part of the body's immune system is missing or does not function properly due to intrinsic defects in the immune system. These patients have an increased susceptibility to pulmonary complications as well as primary infections. This study was to investigate pulmonary complications in patients with primary immunodeficiency diseases and characterize their patterns according to specific immune defects.
METHODS
We retrospectively reviewed the medical records of 37 different cases of primary immunodeficiency diseases that were admitted to Severance hospital in Seoul between 1990 and 2006.
RESULTS
Many patients had a previous history of recurrent respiratory infections and some suffered from pulmonary complications. Eight patients with hypogammaglobulinemia showed peribronchial wall thickening or bronchiectasis, and pneumatoceles or emphysematous changes were characteristic complications in two patients with hyper IgE syndrome. On microbiological examination, various bacteria, including Staphylococcus aureus, Pseudomonas aeroginosa, Streptococcus pneumoniae, were isolated from the hypogammaglobulinemia patients' sputum or ear discharge. In some patients with cell-mediated immune defects, such as chronic granulomatous disease or severe combined immune deficiency, yeast and ameba were isolated from the sputum and bronchial washing fluid respectively.
CONCLUSION
Infections account for most of these complications, but the host reaction to infection seems to cause characteristic findings that could be helpful for diagnosis. The physician should be alert for the early diagnosis of children with primary immunodeficiency in order to prevent pulmonary complications.

Keyword

Immunologic deficiency syndrome; Pulmonary disease

MeSH Terms

Agammaglobulinemia
Amoeba
Bacteria
Bronchiectasis
Child
Diagnosis
Ear
Early Diagnosis
Granulomatous Disease, Chronic
Humans
Immune System
Immunologic Deficiency Syndromes
Job Syndrome
Lung Diseases
Medical Records
Pseudomonas
Respiratory Tract Infections
Retrospective Studies
Seoul
Sputum
Staphylococcus aureus
Streptococcus pneumoniae
Yeasts
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