Article
Elderly-onset hereditary pulmonary alveolar proteinosis and its cytokine profile.
BMC pulmonary medicine - 17 Feb 2017
Ito Masayuki, Nakagome Kazuyuki, Ohta Hiromitsu, Akasaka Keiichi, Uchida Yoshitaka, Hashimoto Atsushi, Shiono Ayako, Takada Toshinori, Nagata Makoto, Tohyama Jun, Hagiwara Koichi, Kanazawa Minoru, Nakata Koh, Tazawa Ryushi
Abstract excerpt
BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation, and is caused by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling. Abnormalities in CSF2 receptor alpha (CSF2RA) were reported to cause pediatric hereditary PAP. We report here the first case of CSF2RA-mutated, elderly-onset hereditary (h) PAP. CASE PRESENTATION: The...
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