December 01 2013 – Human stem cells converted to functional lung cells

NEW YORK, NY — For the first time, scientists have succeeded in transforming human stem cells into functional lung and airway cells. The advance, reported by Columbia University Medical Center (CUMC) researchers, has significant potential for modeling lung disease, screening drugs, studying human lung development, and, ultimately, generating lung tissue for transplantation. The study was published today in the journal Nature Biotechnology. “Researchers have had relative success in turning human stem cells into heart cells, pancreatic beta cells, intestinal cells, liver cells, and nerve cells, raising all sorts of possibilities for regenerative medicine,” said study leader Hans-Willem Snoeck, MD, PhD, professor of medicine (in microbiology & immunology) and affiliated with the Columbia Center for Translational Immunology and the Columbia Stem Cell Initiative. “Now, we are finally able to make lung and airway cells. This is important because lung transplants have a particularly poor prognosis. Although any clinical application is still many years away, we can begin thinking about making autologous lung transplants—that is, transplants that use a patient’s own skin cells to generate functional lung tissue.”

Read the entire article at:

http://www.eurekalert.org/pub_releases/2013-12/cumc-hsc112713.php

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

 

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical | Tagged , , , , , , , , , , , , , , , , , , | Leave a comment

November 30 2013 – Keyhole lung transplant performed on 60-year-old in Chennai

Surgeons at Global Health City have performed a minimally-invasive lung transplant on a 60-year-old patient, who has been suffering from idiopathic pulmonary fibrosis for the last 11 years.

The lung transplant was done through a seven-inch-long incision, which is nearly one-third the length used in the conventional procedure, doctors said.

Raja Babu Shah, an Indian-origin businessman residing in Nepal, had severe breathlessness and had required oxygen supply fulltime for the last four years. He was confined to his bed and a wheelchair.

In idiopathic pulmonary fibrosis, the cause for which is not known, the lung becomes stiff and does not expand well. There are poor oxygen levels in the blood, said Vijil Rahulan, head of the department, respiratory medicine and senior consultant pulmonologist, Global Health City.

Read the entire article at:

http://www.thehindu.com/news/cities/chennai/keyhole-lung-transplant-performed-on-60yearold-in-chennai/article5405805.ece

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical | Tagged , , , , , , , , , , , , , , , | Leave a comment

November 27 2013 – FibroGen Announces Observations of Possible Disease Stabilization and Improvement in Interim Analysis of Phase 2 Study of FG-3019

SAN FRANCISCO–(BUSINESS WIRE)–FibroGen, Inc. (FibroGen), announced today that interim results from an ongoing open-label Phase 2 clinical study to evaluate FG-3019 as a treatment for idiopathic pulmonary fibrosis (IPF) continue to suggest that the investigational drug has the potential to stabilize and improve fibrosis in patients with the disease. The results substantiate earlier results reported in an oral presentation at the European Respiratory Society Annual Congress in September 2012.

“A substantial subset of IPF patients who received FG-3019 in the Phase 2 open label study experienced stabilization or improvement of lung fibrosis, based on computer-assisted high-resolution radiographic scores of lung fibrosis”

“A substantial subset of IPF patients who received FG-3019 in the Phase 2 open label study experienced stabilization or improvement of lung fibrosis, based on computer-assisted high-resolution radiographic scores of lung fibrosis,” noted Frank H. Valone, M.D., Chief Medical Officer of FibroGen. “This observation of IPF fibrosis improvement is without clinical precedent and directly challenges the widely held belief that improvement in fibrosis is not a therapeutic possibility. Data from the Phase 2 FG-3019 study also suggest a correlation between improved fibrosis and improved pulmonary function. This evidence supports our belief in the potential clinical impact of FG-3019 across a number of IPF clinical outcomes.”

Read the entire article at:

http://www.businesswire.com/news/home/20131126006311/en/FibroGen-Announces-Observations-Disease-Stabilization-Improvement-Interim#.VCnKcM90xhE

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical | Tagged , , , , , , , , , , , , , , , , , | Leave a comment

November 15 2013 – Enzo Biochem Announces Study in Leading Scientific Journal Linking Idiopathic Pulmonary Fibrosis, a Deadly Human Disease with No Effective Treatment Options, to Presence of a Monkey Virus

Enzo Biochem Inc. (NYSE:ENZ) today announced publication of a study in a leading scientific journal Modern Pathology a Nature Publishing Group publication that shows a strong association of idiopathic pulmonary fibrosis (IPF) a pulmonary disease with 100% mortality within five years with the presence of the herpesvirus saimiri virus a virus native to squirrel monkeys. The breakthrough discovery of the IPF’s origin is expected to result in a clinical diagnostic that could lead to screening and diagnosis and perhaps result in potential treatment for this fatal disease.

The publication was authored by scientists from Enzo Biochem Ohio State University the Medical College of Wisconsin and Cornell University. The discovery is the subject of a patent application that is exclusively assigned to Enzo Biochem. “The significance of this discovery beyond providing a reliable marker for screening and diagnosis is the potential for development of new therapeutic strategies and better understanding of the progression of the disease in humans” said Elazar Rabbani study co-author and Chief Executive Officer of Enzo. “This may also translate into a patient’s ability to live with the disease or even be cured if the fibrosis is not too advanced.”

Read the entire article at:

http://www.pharmiweb.com/PressReleases/pressrel.asp?ROW_ID=80738#.VCnJ-c90xhF

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical | Tagged , , , , , , , , , , , , , , , , | Leave a comment

November 10 2013 – Research by Saint Louis University scientists offers way to disrupt fibrosis

ST. LOUIS – A team of scientists that includes Saint Louis University researchers has identified a new way to intervene in the molecular and cellular cascade that causes fibrosis – a condition where the body’s natural process of forming scars for wound healing goes into overdrive and causes diseases. The findings, published Nov. 10 in the advance online issue of Nature Medicine, demonstrate a potential novel therapeutic approach to treat fibrotic diseases such as idiopathic pulmonary fibrosis and liver fibrosis.

The research targets a pathway that turns off the trigger for the major molecular mediator of fibrosis, a protein called Transforming Growth Factor (TGF) beta. This protein is normally present in the body in an inactive state and must be turned on to cause fibrosis. Once activated, TGF beta protein stimulates cells called myofibroblasts to produce excess collagen, which is a principle component of scars.

The researchers showed that removing a gene in the myofibroblasts that makes a specific subset of proteins called alpha v integrins blocks the ability of these cells to trigger activation of TGF beta. Furthermore, they were able to replicate the effect of the gene deletion by treatment with a small molecule compound, thus opening the door to a potential new therapy for patients.

Read the entire article at:

http://www.eurekalert.org/pub_releases/2013-11/slu-rbs110713.php

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical | Tagged , , , , , , , , , , , , , , | Leave a comment

November 4 2013 – Development Plan for PTL-202, a Treatment for Progressive Organ Scarring, a $1.1 Billion Opportunity

Worldwide, there are over 5,000,000 people living with Idiopathic Pulmonary Fibrosis (IPF)(IPF Coalition).IPF therapy sales across the US, France, Germany, Italy, Spain, and the UK to rise to over $1.1 billion by 2017, at a Compound Annual Growth Rate (CAGR) of 86.6% (RnR Market Research, 2013). IPF kills more patients per year than either prostate or breast cancer.

Pacific Therapeutics Ltd. (the “Company”) lead drug candidate for fibrosis (progressive scarring of the organ), PTL-202 is a combination of Pentoxifylline (a FDA approved drug used for treating leg cramps) and N-Acetyl-Cysteine (NAC) an amino acid and an extremely potent and important antioxidant.

As previously announced the company has completed a phase 1 trial of the combination in humans. The positive results from the phase 1 clinical trial and positive pre-clinical results, will lead to further development of the product for treating fibrosis such as Idiopathic Pulmonary Fibrosis and Liver Cirrhosis.

Read the entire article at:

http://www.baystreet.ca/viewarticle.aspx?id=409245

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical | Tagged , , , , , , , , , , , , , , , , , | Leave a comment

October 28 2013 – Ambrisentan (Letairis) stopped early in IPF trial (Annals Int Med)

Ambrisentan (Letairis) stopped early in IPF trial

A large phase III randomized trial testing Ambrisentan (Letairis) for early idiopathic pulmonary fibrosis was stopped early after an interim analysis of the data showed possible harm. The results were reported in Annals of Internal Medicine.

Patients being treated with Letairis had more evidence of progression of idiopathic pulmonary fibrosis and were hospitalized more often, including diagnoses of acute exacerbations of IPF and pneumonia. There were 492 patients in the trial, at 136 clinical sites around the world.

Read the entire article at:

http://pulmccm.org/2013/randomized-controlled-trials/ambrisentan-letairis-stopped-early-ipf-trial-annals-int-med/?art

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Longfibrose, Lung Cancer, Lungen Fibrose, Medical, Pulmonary Fibrosis | Tagged , , , , , , , , , , , , , , , , , | Leave a comment

October 21 2013 – Chronic hypersensitivity pneumonitis in patients diagnosed with idiopathic pulmonary fibrosis: a prospective case-cohort study

Chronic hypersensitivity pneumonitis in patients diagnosed with idiopathic pulmonary fibrosis: a prospective case-cohort study

Summary

Background

The clinical features of idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis can be indistinguishable; the need to eliminate occult environmental factors known to cause pulmonary fibrosis in patients suspected to have IPF during diagnostic evaluation is evident. We aimed to investigate occult, putative causes in the environments of patients diagnosed with IPF using tests beyond those conventionally used.

Methods

In this case-cohort study, 60 consecutive patients diagnosed with IPF on the basis of the 2000 American Thoracic Society (ATS) and the European Respiratory Society (ERS) criteria were prospectively followed up every 4 months for 6 years between Jan 1, 2004, and Dec 31, 2009. At each visit a uniformly applied questionnaire was administered to these 60 patients to identify occult antigen exposure known to cause hypersensitivity pneumonitis. Patients underwent specific IgG determination, bronchoalveolar lavage, bronchial challenge testing with suspected antigens, and re-review of histopathological features in existing and subsequently obtained surgical lung biopsy samples and from lung explants. Specimens obtained from suspected sources from the patient’s environment were subjected to cultures in microbiology laboratory. These clinical data and discussions among pulmonologists and radiologists familiar with IPF were used to confirm the diagnosis in accordance with 2011 ATS, ERS, Japanese Respiratory Society, and Latin American Thoracic Association guidelines; 46 of the 60 patients had IPF according to the 2011 guidelines, and our analyses in this study were focused on these 46 patients.

Read the entire article at:

http://www.thelancet.com/journals/lanres/article/PIIS2213-2600(13)70191-7/fulltext

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Lung Cancer, Lungen Fibrose, Medical, Pulmonary Fibrosis, Uncategorized | Tagged , , , , , , , , , , , , , , | Leave a comment

October 18 2013 – UW researchers to test novel therapy for idiopathic pulmonary fibrosis

Dear Colleagues:

I am very pleased to report that UW researchers have received a grant from the National Institutes of Health (NIH) to test a promising, novel surgical therapy for treating idiopathic pulmonary fibrosis (IPF). IPF is a chronic, progressive lung disease of unknown cause and increasing prevalence in the United States. An estimated 100,000 Americans die from IPF each year, typically within three to five years of diagnosis.

The disease is characterized by development of excess fibrous tissue in the lungs. Aside from lung transplantation, which only one percent of affected patients receive, there is no FDA-approved therapy. The grant brings new hope for slowing progression of a fatal disease through safe, minimally invasive surgery.

Read the entire article at:

http://engage.washington.edu/site/PageNavigator/UWMedicine/OnlineNews/OnlineNews_10_18_2013.html

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Lung Cancer, Lungen Fibrose, Medical, Pulmonary Fibrosis, Uncategorized | Tagged , , , , , , , , , , , , , , , , | Leave a comment

October 13 2013 – Deranged fatty acid composition causes pulmonary fibrosis in Elovl6-deficient mice

Despite the established role of alveolar type II epithelial cells for the maintenance of pulmonary function, little is known about the deregulation of lipid composition in the pathogenesis of pulmonary fibrosis. The elongation of long-chain fatty acids family member 6 (Elovl6) is a rate-limiting enzyme catalysing the elongation of saturated and monounsaturated fatty acids. Here we show that Elovl6 expression is significantly downregulated after an intratracheal instillation of bleomycin (BLM) and in human lung with idiopathic pulmonary fibrosis.

Read the entire article at:

http://www.nature.com/ncomms/2013/131011/ncomms3563/full/ncomms3563.html

For more information about Idiopathic Pulmonary Fibrosis please visit our websitewww.ipftoday.com and don’t forget to like us on www.facebook.com/IPFToday

Posted in Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, IPF, Long Fibrose, Lung Cancer, Lungen Fibrose, Medical, Pulmonary Fibrosis, Uncategorized | Tagged , , , , , , , , , , , , , , , , | Leave a comment