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Pulmonaryalveolarproteinosis (PAP )wasfirstdescribedbyRosenetal1 in 195 8 PAPischaracterizedbytheaccumulationof proteinaceousmaterialinthealveolileadingtovaryingdegreesofimpairmentin gasexchange Recentstudiesdemonstratethatdeficiencyofgranulocyte macrophagecolonystimulatingfactor (GM CSF )isastronglysuspectedmechanismforadultacquiredPAP ,andsubcutaneousadministrationofrecombinanthumanGM CSFiseffectiveinapproximately 5 0 %ofpatientswithPAP ;2 4 butwholelunglavage (WLL)remainsthemo… 相似文献
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