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Section of Neonatology, Perinatal and Pulmonary Biology

Section of Neonatology, Perinatal and Pulmonary Biology

 

Significant Accomplishments

Diagnosis and Treatment of Pulmonary Alveolar Proteinosis 

National Institutes of Health funding was approved for Bruce Trapnell, MD, MS, and coworkers who have developed lung markers for the diagnosis of hereditary and acquired disorders of surfactant metabolism.  They have found that pulmonary alveolar proteinosis (PAP) is a heterogeneous disorder caused, in part, by autoimmunity against GM-CSF and by mutations in GM-CSF receptors, enabling correct diagnosis and treatment of these life-threatening disorders.  New treatment modalities, including gene therapy, are being tested in the clinics.

Pathogenesis of Interstitial Lung Disease and Emphysema

New NIH funding was approved for studies, led by Timothy Weaver, PhD, to identify the mechanisms causing lung remodeling in idiopathic pulmonary fibrosis associated with mutations in the surfactant protein-C gene, a cause of life-threatening pulmonary disease in children and adults.  Anne Karina Perl, PhD, leads the work identifying the role of growth factors to enhance lung growth in children and adults with emphysema.

Genetic Networks Linking Mucus Metaplasia and Inflammation

NIH funding was approved for the study of a new genetic network regulating airway epithelial cell differentiation and mucus production related to cystic fibrosis, asthma and COPD in work led by Thomas Korfhagen, MD, PhD, and Jeffrey Whitsett, MD.  A master regulator of mucus production and its impact on lung structure and inflammation are being studied as a target for therapy for chronic lung diseases in children and adults.
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