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Volume 356:547-549 February 8, 2007 Number 6
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Pulmonary Alveolar Proteinosis — Is Host Defense Awry?
Claire M. Doerschuk, M.D.

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 by Uchida, K.

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 by Uchida, K.
Pulmonary alveolar proteinosis is a rare disorder caused by abundant accumulation of surfactant-derived components in the lungs. The incidence is estimated to be 0.36 case per million population, and the prevalence, 3.70 cases per million.1 About 500 cases have been recorded in the literature.

The condition usually presents as progressive dyspnea and a minimally productive cough. There are elevated serum levels of products derived from pulmonary epithelial cells, including cytokeratin 19, the mucin KL-6, and surfactant proteins A, B, and D. Pulmonary-function testing reveals a restrictive pattern and a disproportionate reduction in diffusing capacity. Patients often have hypoxemia, and the . . . [Full Text of this Article]


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Dr. Doerschuk is vice chair of research and chief of the Division of Integrative Biology in the Department of Pediatrics, Rainbow Babies and Children's Hospital, and a professor of pediatrics, pathology, and biomedical engineering at Case Western Reserve University School of Medicine — both in Cleveland.


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