| Project/Area Number |
16390239
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Respiratory organ internal medicine
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| Research Institution | Niigata University |
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Principal Investigator |
NAKATA Koh Niigata University, Medical and dental Hospital, professor, 医歯学総合病院, 教授 (80207802)
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| Co-Investigator(Kenkyū-buntansha) |
KEICHO Naoto International Medical Center of Japan, Department of Respiraymy Diseases, Director, 研究所, 研究部長 (80332386)
NAKAGAKI Kazuhide Nippon Veterinary and Life Science University, Department of Veterin Medicine, Associate Professor, 獣医学部, 助教授 (90143635)
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| Project Period (FY) |
2004 – 2005
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| Project Status |
Completed (Fiscal Year 2005)
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| Budget Amount *help |
¥14,600,000 (Direct Cost: ¥14,600,000)
Fiscal Year 2005: ¥3,500,000 (Direct Cost: ¥3,500,000)
Fiscal Year 2004: ¥11,100,000 (Direct Cost: ¥11,100,000)
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| Keywords | idiopathic Pulmonary Alveolar Proteinosis / GM-CSF / autoimmune disease GM-CSF / Monoclonal Antibody / Lymphocyte cen surface antigen / 肺胞蛋白症 / 顆粒球マクロファージコロニー刺激因子 / 自己抗体 / アフィニティー / 中和能 |
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| Research Abstract |
Pulmonary alveolar proteinosis (PAP) is a rare clinical syndrome with currently three recognised forms ; congenital, secondary and acquired. More than 90% of cases are of the latter type, which recently has been characterized as an acquired autoimmune disorder. In PAP excess accumulation of surfactant leads to characteristic bilateral airspace opacities on chest radiograph and extensive alveolar densities with thickened interlobular septa on computed tomography scan. PAP typically appears in previously healthy individuals, who present with several months of dyspnoea and impaired pulmonary gas transfer of variable severity. "Milky" appearing, lipid-rich fluid recovered at bronchoalveolar lavage is characteristic, while lung biopsy, when performed, shows minimally inflamed alveoli uniformly filled with amorphous eosinophilic material that on electron microscopy contains characteristic lamellar bodies. Current concepts of pathogenesis in acquired PAP are that the excess surfactant results from impaired clearance by alveolar macrophages, as a result of inhibition by specific polyclonal autoantibodies of trophic physiological signals from the endogenous cytokine granulocyte-macrophage colony-stimulating factor (GM-CSF). Current therapy consists of whole-lung lavage under general anesthesia, repeated as needed by clinical status, while some adult patients also respond to the repeated administration of recombinant human GM-CSF.
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