Pulmonary Branch

For children and adult with advanced lung diseases, transplantation is often the only treatment. Unfortunately, half of these patients will die within 5 – 6 years after transplant because of transplant rejection. Transplant rejection is detected by analyzing a biopsy of the transplanted organ. Obtaining biopsy samples require anesthesia and is quite an invasive procedure. Additionally, biopsy has low sensitivity and usually picks up rejection at late stages when treatments options are limited and often with limited benefits. Could early detection and treatment of rejection save the transplanted organ from rejection? The focus of APO is to investigate whether early detection and treatment of rejection improves transplant survival.

Asthma affects approximately 8% of the U.S. population with significant associated morbidity and mortality.  Despite recent advances, there is an ongoing need to develop new treatments for severe asthmatics who do not respond to current therapies (Please see this video to learn more how asthma affects patients who are participating in clinical asthma research at NIH: https://www.youtube.com/watch?v=jVUZLcZwyxk).  To address this clinical problem, the Laboratory of Asthma and Lung Inflammation has identified novel roles for apolipoproteins and lipoprotein receptors in modifying airway inflammation, airway remodeling, and airway hyperreactivity in asthma.  Furthermore, by defining new mechanisms that modify asthma pathobiology, our laboratory has identified novel treatment approaches that are currently being advanced to clinical trials so that we can begin to assess their safety and effectiveness for asthma.  

The Laboratory of Chronic Airway Infection, led by Dr. Kevin Fennelly, focuses on bronchiectasis, a condition that damages the body's ability to clear mucus from the airways and increases risk of severe lung infections by nontuberculous mycobacteria (NTM), which commonly are found in wet environments such as streams, rivers, and marshes. People with inflammatory lung disease such as cystic fibrosis are particularly vulnerable to this condition. Dr. Fennelly hopes to contribute to reducing the impact of such infections by studying common genetic characteristics of the people who suffer from them, defining how the infections lead to disease, and ultimately developing more effective, efficient treatments. Dr. Fennelly and his collaborators also seek to address potential genetic risk loci that might explain overlapping characteristics of health conditions such as cystic fibrosis, primary ciliary dyskinesia, connective tissue disorders including Marfan or Ehlers-Danlos syndromes, and immune-related disorders.

The Laboratory of Translational Research, led by Dr. Joel Moss, focuses on clinical and translational areas of investigation. Primary clinical and translational studies address the pathogenesis and treatment of cystic lung diseases, such as lymphangioleiomyomatosis (LAM). Studies of LAM focus on the growth, genetics and metastasis, or spreading, of LAM cells, which is responsible for destruction of the lung parenchyma. Basic studies examine the post-translational modification of proteins by ADP-ribosylation, both that catalyzed by bacterial toxins involved in disease as well as the reversible ADP-ribosylation cycle endogenous to human cells, which participates in membrane repair and response to oxidative stress.

Many disease processes and therapies may impact the respiratory system.  Pulmonary function testing employs a wide variety of tests to assess the function and response of the respiratory system.  This enables characterization of baseline status of the system as well as longitudinal measurement of changes related to disease progression, improvement,  and response to or adverse effects from therapies.  Testing modalities employed in the laboratory include spirometry, plethysmography, nitrogen washout, diffusion capacity, cardiopulmonary exercise testing, walk testing, respiratory muscle strength, high altitude simulation, shunt studies, impulse oscillometry, bronchoprovocation, and exhaled nitric oxide analysis.  Customized tests may be developed with investigators as required. 

Contact: Andrew Lipton, M.S., M.D., MPH&TM, Medical Director, andrew.lipton@nih.gov

NIH/NHLBI Track University of Maryland Pulmonary and Critical Care Fellowship Program