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KMID : 0390020070170040372
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Pediatric Allergy and Respiratory Disease
2007 Volume.17 No. 4 p.372 ~ p.383
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Pulmonary Complications in Primary Immunodeficiency Diseases
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Kim Ki-Hwan
Kim Khi-Joo
Lee Taek-Jin
Chun Jin-Kyoung
Kim Dong-Soo
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Abstract
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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.
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KEYWORD
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Immunologic deficiency syndrome, Pulmonary disease
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