Goal 2: Reduce Human Disease

How can we better understand regional tissue heterogeneity in lung disease?

Many lung diseases (IPF, COPD) are characterized by marked heterogeneity at the tissue level. Unfortunately, most of the tools we currently employ to understand lung disease are unable to elucidate the mechanisms that result in regional heterogeneity. Clinical studies and animal models, while invaluable, generally assume that all lung tissue is similarly affected based on the presence or absence of diagnostic criteria ...more »

Submitted by (@bradley.richmond)

Voting

9 net votes
26 up votes
17 down votes
Active

Goal 2: Reduce Human Disease

Does the genetic cause of pulmonary fibrosis have implications for treatment response?

Is pulmonary fibrosis due to genetic/heritable causes different than 'sporadic' 'idiopathic' pulmonary fibrosis? With findings in genetics and the emphasis on precision medicine the issue of pulmonary fibrosis being idiopathic will diminish with time. Treatment for this fatal disease should be accessible to those who have pulmonary fibrosis with no other alternatives.

Submitted by (@dappell)

Voting

1 net vote
2 up votes
1 down votes
Active

Goal 2: Reduce Human Disease

Pathobiology of Lung Fibrosis

End organ fibrosis accounts for up to 45% of deaths in developed countries. In particular, lung fibrosis is a devastating disease with poor prognosis. Despite development of two new drugs, their efficacy is still limited, highlighting the need to better understand the pathobiology that accounts for fibrotic disease progression in the presence and absence of acute exacerbation or infectious drivers.

Submitted by (@bmoore)

Voting

19 net votes
30 up votes
11 down votes
Active

Goal 2: Reduce Human Disease

Is there a Biomarker for the Pulmonary Fibrosis of HPS?

Hermansky-Pudlak Syndrome is characterized by a bleeding disorder as well as pulmonary fibrosis. Invasive procedures such as a lung biopsy are contraindicated due to bleeding and bronchoscopy is not without risk. Finding a biomarker would reduce the necessity for more invasive data collection while improving outcomes.

Submitted by (@dappell)

Voting

2 net votes
3 up votes
1 down votes
Active

Goal 2: Reduce Human Disease

mechansisms of post thrombotic syndrome

No good medical therapy exists to prevent and treat post thrombotic syndrome, the most common sequlae from a deep vein thrombosis. Recent trial data suggests that compression stockings do not prevent PTS, and thrombolysis is expensive and risky. The basic mechanisms related to fibrosis of the vein wall are not well understood.

Submitted by (@henke0)

Voting

1 net vote
5 up votes
4 down votes
Active

Goal 3: Advance Translational Research

The Future of Fibrosis Research

A critical challenge in the area of fibrosis research (including heart, lung and other organs) is the absence of a central system to act as air traffic control for progress, innovations and gaps in the science. Like the area of cancer research was 30 years ago, the fibrosis research area is fragmented and disconnected. The answer in the cancer effort to creating a centralized, focused system was with the National Cancer ...more »

Submitted by (@teresabarnes)

Voting

3 net votes
6 up votes
3 down votes
Active

Goal 2: Reduce Human Disease

Identifying Early Stages of Pulmonary Fibrosis

The majority of translational research designed to improve the lives of patients with pulmonary fibrosis has focused on studies of patients with advanced fibrotic lung disease. In contrast, little effort has been paid to understanding the natural history of pulmonary fibrosis, exploring the mechanisms/pathogenesis of the development of pulmonary fibrosis, and considering work designed to prevent the development of lung ...more »

Submitted by (@ghunninghake)

Voting

29 net votes
35 up votes
6 down votes
Active

Goal 2: Reduce Human Disease

Fibrosis Across Organs: Bringing Together Investigators of Fibrosis of the Heart, Lungs and Bone Marrow

Fibrosis can affect essentially any tissue or organ, including the heart, lungs and bone marrow. Effective anti-fibrotic therapy has long been elusive, and transplantation has been the only therapy capable of restoring patient function as fibrotic diseases progress to organ failure. Although these diseases present clinically with organ-specific manifestations, they are now thought to share many common pathogenetic mechanisms. ...more »

Submitted by (@amtager)

Voting

16 net votes
20 up votes
4 down votes
Active

Goal 2: Reduce Human Disease

Impact of lung remodeling on congestive heart failure progression

End stage congestive heart failure (CHF) causes intensive lung remodeling beyond the type-2 pulmonary hypertension. CHF induced lung remodeling includes profound lung fibrosis, lung vascular remodeling and lung inflammation. Understanding CHF-induced lung remodeling is also critical to understand the right ventricular failure. However, this area is largely unstudied. Regulating CHF-induced lung remodeling and the underlying ...more »

Submitted by (@chenx106)

Voting

13 net votes
15 up votes
2 down votes
Active

Goal 2: Reduce Human Disease

what are the molecular pheontypic differences in IPF/ILD

What are the molecular phenotypic differences in blood and tissue of IPF ILD and how do they relate to disease course and potential response to therapy. There is a need to gain understanding in humans of the differences and similarities in iPF and iLD in general to eliminate the idiopathic nature and establish human targets. The challenge is coupling such research to longer term studies/outcomes and potentially clinical ...more »

Submitted by (@inoth0)

Voting

0 net votes
3 up votes
3 down votes
Active

Goal 2: Reduce Human Disease

Correlation between abdominal/diaphragmatic fibrosis and cardiopulmonary dysfunction in Duchenne

Is there a correlation between the development of abdominal/diaphragmatic fibrosis and the development of cardio-pulmonary dysfunction? Are there mechanisms (i.e., pulmonary excursion therapy) that may prevent/postpone the development of diaphragmatic fibrosis and subsequent pulmonary dysfunction?

Submitted by (@kathi0)

Voting

-4 net votes
3 up votes
7 down votes
Active

Goal 3: Advance Translational Research

Do Alpha-1 Antitrypsin Deficiency and Cystic Fibrosis Inform COPD? Have we been looking?

Cystic fibrosis (CF) and alpha-1 antitrypsin deficiency (AATD) share phenotypic features with common COPD including airflow obstruction and airway mucociliary dysfunction. Although research in CF and AATD has advanced our understanding of those rare diseases, it has yet to explain common COPD. Do Alpha-1 Antitrypsin Deficiency and Cystic Fibrosis Inform COPD? Could therapies currently in use or under development for ...more »

Submitted by (@kerickson)

Voting

22 net votes
24 up votes
2 down votes
Active