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(Chest. 2001;119:645-647.)
© 2001 American College of Chest Physicians

Focal Congenital Alveolar Proteinosis Associated With Abnormal Surfactant Protein B Messenger RNA*

Eva Mildenberger, MD; Daphne E. deMello, MD; Zhenwu Lin, PhD; Hans Kössel, MD; Thomas Hoehn, MD and Hans T. Versmold, MD

* From the Department of Pediatrics (Drs. Mildenberger, Kössel, and Versmold), Universitätsklinikum Benjamin Franklin, Freie Universität, Berlin, Germany; Department of Pathology (Dr. deMello), Cardinal Gennon Children’s Hospital, St. Louis, MO; Department of Cellular and Molecular Physiology (Dr. Lin), Pennsylvania State University, College of Medicine, Hershey, PA; and Department of Neonatology (Dr. Hoehn), Charité, Campus Virchow-Klinikum, Humboldt-University, Berlin, Germany.

Correspondence to: Eva Mildenberger, MD, Kinderklinik, Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, Hindenburgdamm 30, 12200 Berlin, Germany

Two siblings presented with typical clinical features of congenital pulmonary alveolar proteinosis (PAP). Necropsy of one sibling revealed scattered foci of the diagnostic histologic changes in the lung tissue. In contrast to infantile and adult PAP, focal distribution is uncommon in congenital PAP. Defective expression of the granulocyte-macrophage colony-stimulating factor receptor was ruled out. The surfactant protein B (SP-B) content in the lung tissue of the autopsied patient was low, and a deletion in the SP-B messenger RNA was detected. We speculate that the PAP in our patients was related to the reduced quantity and/or to the altered quality of SP-B.

Key Words: granulocyte-macrophage colony-stimulating factor receptor deficiency • pulmonary alveolar proteinosis • surfactant protein B deficiency




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