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Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia
Author(s) -
Patiroglu Türkan,
Akyıldız Başak,
Patiroglu Tahir E.,
Gulmez Inci Y.
Publication year - 2008
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20818
Subject(s) - medicine , pulmonary alveolar proteinosis , immunology , pathology , lung
Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of surfactant derived material in the lung of patients. PAP is rare in children. The patient presented with respiratory failure. In the history she was diagnosed with agammaglobulinemia at 8 months of age and has been treated by IVIG once in a month. She had two pulmonary alveolary proteinosis attacks before. Chest X‐ray showed bilateral diffuse infiltrates. Initial diagnosis were pneumonia, ARDS, and lung edema. Whole‐lung lavage revealed lipoproteinaceous material similar to surfactant. This findings and high level of LDH was as evaluated pulmonary alveolary proteinosis. She discharged from the hospital without any respiratory complication on the ninth day. This is the first case report recurrent PAP associated with agammaglobulinemia. Pediatr Pulmonol. 2008; 43:710–713. © 2008 Wiley‐Liss, Inc.