Gene expression patterns predict rapid decline in idiopathic pulmonary fibrosis patientsMay 31, 2007Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease typically characterized by the slow but progressive onset of shortness of breath or cough. Most patients live about five years after diagnosis. However, according to a new study being published today in the online journal PLoS ONE, a subset of patients with a specific genetic profile has a much more rapid progression to complete pulmonary failure and death without a lung transplant. Based on observations in the clinic that some IPF patients display a more rapidly progressing disease course, researchers at the Simmons Center for Interstitial Lung Disease at the University of Pittsburgh School of Medicine, collaborating with pulmonary scientists in Mexico and California, used DNA microarray analysis to measure the gene expression patterns of 26 rapid progressors and 88 slow progressors. They identified 437 differentially expressed genes between the groups. Specifically, lungs of rapid progressors, who were predominantly males who smoked, overexpressed genes involved in the development of tissues and organs (morphogenesis), oxidative stress, cell migration and proliferation and genes from fibroblasts and smooth muscle cells. According to Naftali Kaminski, M.D., director of the Simmons Center and director of the Lung Translational Genomics Center, division of pulmonary, allergy and critical care medicine, University of Pittsburgh School of Medicine, these findings offer strong evidence that rapid progressors represent a distinct clinical phenotype compared with the usual slower progressing patients. "We are only now starting to really understand IPF and to characterize it," Dr. Kaminski said. "Therefore, it is critical for patients with the disease to be seen in centers that are actively involved in IPF research, so we can help them better decide a course of action." These findings also highlight the variability in the progression and outcome of IPF and may explain, in part, the difficulty in obtaining significant and reproducible results in studies of therapeutic interventions in patients with IPF, noted first author Moisés Selman, M.D., director of research at the National Institute of Respiratory Research in Mexico City. "This study suggests that physicians should pay more attention to the time of onset of symptoms in their patients," Dr. Selman said. "Although preliminary, these results may allow investigators to identify biomarkers of disease progression and, more importantly, better evaluate the effectiveness of potential therapies," added Talmadge E. King Jr., M.D., chief of medicine at San Francisco General Hospital and an internationally renowned expert in research and management of pulmonary fibrosis. University of Pittsburgh Schools of the Health Sciences |
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| Related Pulmonary Fibrosis Current Events and Pulmonary Fibrosis News Articles Common weed could provide clues on aging and cancer A common weed and human cancer cells could provide some very uncommon details about DNA structure and its relationship with telomeres and how they affect cellular aging and cancer, according to a team led by scientists from Texas A&M University and the University of Cincinnati (UC). New data: Hospital imaging centers poised to pull back, hitting patients hardest in rural areas Survivors and patients with cancers and heart disease, along with patient advocate organizations and physicians, today urged policymakers to enhance early diagnosis of deadly diseases by preserving access to advanced imaging, such as MRI and CT scans, in final health care reform legislation. Pitt team first to profile genes in acutely ill idiopathic pulmonary fibrosis patients The first findings from a one-of-a-kind, patient-driven effort to provide lung tissue for research might help doctors predict when patients with idiopathic pulmonary fibrosis (IPF) are becoming dangerously ill and also could point the way to interventions that could sustain them until life-saving transplants can be performed. Protein may be strongest indicator of rare lung disease, study shows Researchers at the University of Cincinnati (UC) have discovered a protein in the lungs that can help in determining progression of the rare lung disease Idiopathic Pulmonary Fibrosis (IPF). Large clinical trial finds pirfenidone may help lung function in IPF patients A large, well-controlled, multi-national clinical trial program has demonstrated the effectiveness and safety of what may become the first FDA-approved medicine for idiopathic pulmonary fibrosis, or IPF. UT Southwestern researchers identify gene linked to inherited form of fatal lung disease Researchers at UT Southwestern Medical Center have determined that a mutation in a gene known for its role in defending the lungs against invading pathogens is responsible for some inherited cases of a lethal lung disease affecting older adults. The same mutation may also be associated with lung cancer, the researchers said. Double threat: Deadly lung disease also linked to heart attacks Patients with idiopathic pulmonary fibrosis (IPF) are three times as likely to experience severe coronary events-including heart attacks-than people without the disease. Note to people with scarred and stiffened lungs: Monitor your sleep before severe fatigue sets in Family, friends and neighbors remember Lisa Sandler Spaeth as an active mother of two in Potomac, Md., with a lot on the go, juggling her son's baseball games and her daughter's horseback-riding lessons with numerous committee obligations, organizing women's activities at her local synagogue. Biomarkers identified for idiopathic pulmonary fibrosis The first evidence of a distinctive protein signature that could help to transform the diagnosis and improve the monitoring of the devastating lung disease idiopathic pulmonary fibrosis (IPF) is being reported by University of Pittsburgh School of Medicine researchers in this month's edition of PLoS Medicine, an open-access journal of the Public Library of Science. UCSF marks a milestone with 500th transplant in heart and lung program UCSF marked a milestone this week with the 500th procedure in its Thoracic Transplant Program, which specializes in transplantation of the heart and lung. More Pulmonary Fibrosis Current Events and Pulmonary Fibrosis News Articles |
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