In acquired pulmonary alveolar proteinosis, lipids and proteins accumulate within the
alveoli because alveolar macrophages cannot catabolize surfactants. Surprisingly, alveolar …

▪ Abstract Recent studies in transgenic mice have revealed important insights into the roles
of GM-CSF in regulation of surfactant homeostasis and lung host defense. Interruption of the …

Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of
alveolar surfactant and dysfunction of alveolar macrophages. PAP results in progressive …

Direct transfer of the normal cystic fibrosis (CF) transmembrane conductance regulator (CFTR)
gene to airway epitheiium was evaluated using a replicationdeficient recombinant …

Background Lymphangioleiomyomatosis (LAM) is a progressive, cystic lung disease in
women; it is associated with inappropriate activation of mammalian target of rapamycin (mTOR) …

… toshev, and BC Trapnell. 1994. Evaluation of the efficacy and safety of in vitro, adenovirus-mediated
transfer of the human cystic fibrosis transmembrane conductance regulator cDNA. …

… PU.1 protein levels in AMs in vivo and that the 2 Correspondence: bruce.trapnell@chmcc.org …
BC (2001). Alveolar macrophage deficiency in M-CSF-deficient, group B streptococcal …

… Trapnell, BC etal. Down-regulation of cystic fibrosis gene mRNAtranscript levels and
induction of the cystic fibrosis chloride secretory phenotype in epithelial cells by phorbol ester. J. …

Background Stricturing and penetrating complications account for substantial morbidity and
health-care costs in paediatric and adult onset Crohn's disease. Validated models to predict …

Rationale: Considerable confusion exists regarding nomenclature, classification, and
management of pediatric diffuse lung diseases due to the relative rarity and differences in the …