pulmonary hypertension diagnosis

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Patient credits Greeley pulmonary hypertension program with saving his life - Greeley Tribune Posted: 16 Mar 2019 12:00 AM PDT About two years ago, John Kamprath was planning for something very different from the spring break vacation he planned this year. The now-58-year-old Estes Park resident had been diagnosed with high blood pressure of the lungs, something known as "pulmonary hypertension." The online literature Kamprath had read said pulmonary hypertension patients faced an average of two years before the complications become fatal. With two girls to take care of, Kamprath sat down with his wife at the time to confront what seemed like a very real possibility: in two years, it would just be her and the girls. "Are you guys going to be okay when I pass away?" Kamprath remembers thinking. "Where are we finances-wise? Let's make sure we don't drain funds. My life insurance policy's good, so the family's going to be just fine." Fortunately for Kamprath, things started to turn around after a referral to Dr. Lin-Wang Dong, director of the CardioVascular Institute's Pulmonary Hypertension program, 1800 15th St., No. 310, in Greeley. Where other doctors in Denver and Arizona kept referring Kamprath from the cardiologist — the heart doctor — to the pulmonologist — the lungs and respiratory tract doctor — and back again, Dong took a step back and looked at Kamprath as a total patient. Dong started to help Kamprath recover in ways other doctors couldn't. Pulmonary hypertension can be difficult to diagnose and treat because of the complex interplay between the heart and the lungs. The pulmonary arteries take de-oxygenated blood in the heart to the lungs, where the blood picks up oxygen. The pulmonary veins then return oxygenated blood to the heart, where it sends the blood out to the rest of the body. Issues at various points in this system can cause pulmonary hypertension, which is a problem for cardiologists, who specialize only in the heart, and pulmonologists, who specialize only in the lungs and respiratory tract. Focusing only on the tests of the systems they've specialized in, the two specialists can end up passing patients with pulmonary hypertension back and forth, like Kamprath experienced. High blood pressure in the lungs can deprive the body of the right amount of oxygen, causing shortness of breath and a range of other problems. For Kamprath, it didn't seem like much more than a cold about five years ago, but as his condition worsened, he started seeing specialists. Though doctors thought pulmonary hypertension was a possibility, they contradicted themselves and weren't able to bring Kamprath lasting relief or treatment. Eventually he decided he at least needed a cardiologist near his work at the University of Colorado Boulder, where he's the deferred maintenance program manager for facilities. That cardiologist recommended he start seeing Dong. Just as Kamprath started seeing Dong, about a year and a half ago, he ended up in the hospital with pneumonia. He caught an illness because pulmonary hypertension weakened his immune system. Kamprath said he was having a terrible time breathing and gained more than a dozen pounds of fluid from around his lungs. In about 48 hours, Kamprath said he had nearly 16 pounds of fluid drained. With visits around morning, midday and night, Dong kept a watchful eye on Kamprath. "I think if I hadn't been here, I doubt I would have made it," he said. "I credit him with saving my life." Dong, thanks to an extensive background including 16 years in a research lab as a cardiovascular pathophysiology major, started the pulmonary hypertension program in 2008. The Pulmonary Hypertension Association designated the program a Regional Clinical Program earlier this year. Only six other programs have earned the accreditation in the U.S. — the nearest one to Greeley is located in Dallas. Dong said they see patients from all over Colorado, Kansas, Nebraska and Wyoming. Margo Karsten, CEO for Banner Health in northern Colorado, said the accreditation helps a high-risk, vulnerable group of patients find the care they need by meeting the standards of a third party. "Because of the way that Dr. Dong has coordinated their care, their quality of life is drastically impacted in a positive way," Karsten said. For the past year and a half, Kamprath has primarily seen Dong, and he still can't say enough about Dong's expertise. When he first met Dong, Kamprath was on four liters of oxygen and expecting his life to end soon. These days, he's comfortable with a one-liter oxygen tank and is talking about visiting colleges with his soon-to-graduate daughter. "He opened a door that, two years ago, was closed. … His ability to take where I was at and transform it into where I am today — I'm speechless," Kamprath said. "I'm more active than I've ever been. I go to my kids' basketball games."

Bcl-xL Is a Potential New Therapeutic Target for PAH, Researchers Say - Pulmonary Hypertension News Posted: 27 Mar 2019 12:00 AM PDT Mutations in the BMPR2 gene alter the ratio of pro- and anti-death signals in cells lining the blood vessels, promoting cells destined to die to escape, which in turn culminates in a build-up of cells in blood vessels and their subsequent blockage, leading to pulmonary arterial hypertension (PAH). The factor mediating this resistance to cell death, called Bcl-xL, is thus a potential therapeutic target for PAH, researchers suggest. The study reporting the findings, titled "BMPRII deficiency impairs apoptosis via the BMPRII-ALK1-BclX-mediated pathway in pulmonary arterial hypertension (PAH)," was published in the journal Human Molecular Genetics. "PAH remains a challenging condition to manage, and the findings reported in the present work offer additional insights as to both the process of development of PAH and ways of monitoring the progression of the disease," Richard Trembath, MD, Kings College London, U.K., and a study author, said in a press release. Researchers at the University of Bradford, U.K. previously showed that loss-of-function mutations in the BMPR2 (bone morphogenetic protein type II receptor) gene are present in more than 80% of cases of familial and heritable PAH. Mutations are also found in around 40% of isolated PAH cases. The faulty BMPR2 gene, researchers found, contributes to two steps of PAH development – uncontrolled proliferation of cells lining the arteries supplying blood to the lungs, and the resistance of damaged cells to undergo apoptosis (cell death), culminating in the blockage of blood vessels with these cells. In 2012, researchers showed how BMPR2 drives the excessive proliferation of pulmonary arterial smooth muscle cells (PASMCs). Now, researchers investigated the mechanism behind how a faulty BMPR2 enhances the cells' resistance to apoptosis in PAH. "We wanted to find out why the cells are not dying, but instead were building up inside the wall of the pulmonary arteries. To do this, we needed to identify and investigate the proteins that are influenced by this gene," said Talat Nasim, MD, of the Bradford's School of Pharmacy and Medical Sciences, and the study's lead author. The Bcl-x gene is a key regulator of apoptosis, and can generate a pro-apoptotic (Bcl-xS) and anti-apoptotic (Bcl-xL) protein, meaning one that promotes or prevents cell death. Researchers first saw that patients with PAH carried high levels of the anti-apoptotic Bcl-xL transcript (the molecule that gives rise to proteins), while the pro-apoptotic Bcl-xS was reduced. This occurred in patients with and without mutations in the BMPR-II gene. Then the team used a non-genetic PAH experimental model, the hypoxia-induced rat model of PAH, and confirmed that the animals had reduced levels of BMPR2 gene and higher levels of the anti-apoptotic Bcl-xL. Also, PAH-PASMCs harboring a disease-linked BMPR2 mutation had higher levels of anti-apoptotic Bcl-xL, suggesting BMPR2 dysfunction might potentiate resistance to apoptosis. Overall, the results suggested that measuring the ratio of Bcl-xL and Bcl-xS can help identify early stages of PAH. Moreover, Bcl-xL is a potential novel therapeutic target against PAH. "This protein can be used as a biomarker for accurately identifying PAH in patients," Nasim said. "This could help us diagnose PAH at an earlier stage, possibly leading to better treatment options for patients. We can also make other services available, such as genetic counseling, to help patients understand the disease and to identify those at risk of developing it." Nick Morrell, MD, from the University of Cambridge, and a study author, said: "This exciting work adds significantly to our understanding of how inherited forms of PAH are caused, and potentially offers a new way to diagnose the disease early. Early diagnosis and early treatment mean better outcomes for our patients." Nasim's team has filed a patent for the biomarker, and is investigating its potential as a therapeutic target.

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