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Do my pasco capstone signal generator

Do my pasco capstone signal generator do my best researchable topics hp selective storage presentation ´╗┐my name is dr. Lewis Clinton's I am an associate professor of medicine at Boston University School of Medicine director of the Center for Excellence in sickle cell disease there and I also am a member of the pulmonary hypertension center at Boston University and Boston Medical Center today I'd like to talk to you about sickle cell disease and how it relates to pulmonary hypertension so sickle cell disease is is a genetic disease so it's inherited and in order to inherit the disease you need to obtain you need to have one gene from your mother and one gene from your father it is something that's called autosomal recessive and it's within the way that it's inherited and the gene that's involved is a gene called hemoglobin s patients who have sickle cell disease have either two of the hemoglobin s genes or they have one hemoglobin s gene and a gene for another abnormal hemoglobin such as hemoglobin C or hemoglobin beta thalassemia kids who are affected with this will be actually diagnosed in the United States at birth because of screening a newborn diagnostic testing they may start to develop problems during childhood and most frequently patients get admitted to the hospital during childhood for what's called a vey to occlusive or a painful crisis but then there are other complications that can happen as well in different organs such as strokes people certain people can get problems with their kidneys over time there were diseases that can happen to the bones and joints something that's called the avascular necrosis and then there are different complications that can happen within the heart and lungs pulmonary hypertension is one of these type of complications that can happen to patients with sickle cell disease we find that it happens more commonly as people get older and older in sickle cell disease is over the age of 30 or 35 so it may not be what people in the general population think of as being older and so we know that if about a hundred people with sickle-cell disease were in a room somewhere between a 6 to 11 of them would have full hypertension three years ago now the American Thoracic Society published guidelines for the diagnosis and treatment of pulmonary hypertension related to sickle cell disease which I was very heavily involved with it was the first author on and what we recommend for adults so anybody over the age of eighteen with sickle cell disease is that they obtain an echocardiogram as an ultrasound test of the heart at least once every couple of years to evaluate for the possible presence of pulmonary hypertension and that's one of the ways to try to screen people for the presence in the disease the other thing to be aware of is that there are symptoms that may suggest pulmonary hypertension such as increased shortness of breath pain or pressure in your chest or worsening dizziness or passing out with exertion people with sickle-cell disease may have some of these symptoms and it may not relate to pulmonary hypertension at all we know that just being anemic related to the sickle-cell disease can cause some of these symptoms as well and it's one of the reasons that we advocate for doing if cardiograms and everyone to try to identify who may have pulmonary hypertension so first of all you need to realize that sickle-cell disease is an international problem it is not just limited to the United States and so while a hundred thousand individuals are living with sickle cell disease in the United States of America there are about 250 thousand births that occur each year worldwide the majority of patients with sickle cell disease are being born in sub-saharan Africa and India and many of those patients don't live to adulthood because of complications of their disease but what I will say is that the scope of potential patients that may be affected with pulmonary hypertension is actually quite large and that's one of the reasons to try to increase awareness of this disease because the frequency of adults were affected with the disease is similar to scleroderma another rare disease but with a fairly common incidence of pulmonary tension within it one of the things that came out of the guidelines and the work that's followed is that there are still many unanswered questions in the area of pulmonary hypertension related to sickle cell disease and one of the reasons that is is that this is a rare complication of a rare disease and that is a challenge for us in terms of how to answer the questions facing this disease so what we know in the disease frequency is that if you obtain echocardiograms on adults with sickle-cell disease particularly if you have hemoglobin SS disease about a third of adults will have a nemurum electrocardiogram suggestive of pulmonary hypertension but only about 6 to 11 percent of the adults actually had truly had pulmonary hypertension and it is necessary and essential for anybody who is suspected of having pulmonary hypertension to undergo a right heart or pulmonary artery catheter ization to actually diagnose the disease that was one of the things that came out of the guidelines from a couple of years ago the other thing that remains an unanswered question is if once you have pulmonary hypertension where to go from there what we know about pulmonary hypertension and sickle cell disease is not everybody who has pH actually has pulmonary arterial hypertension at least half of the people with pulmonary hypertension have elevated pulmonary pressures related to problems with the left side of their heart it's not clear at this point if those patients should be treated the same as patients who have pulmonary arterial hypertension the other thing that we don't completely understand is whether or not patients who have pulmonary hypertension and it's a sickle-cell disease will respond the same way to traditional pulmonary hypertension treatments and there's a lot of interest in this community and really trying to investigate that we're more intensely what has been clear from the randomized control trials that have been attempted in this population is that pulmonary hypertension in many ways is different when it back to patients with sickle cell disease than in the general population and the sickle cell disease is always playing a role in the disease process so you can't just focus on the pH you have to keep also treat the sickle cell disease in these patients there's an important need to look for other complications such as pulmonary emboli and thrombosis or blood clots within the lungs which likely occur more commonly in patients with sickle cell disease and likely make pulmonary hypertension worse there's also a need to look for hypoxia or low oxygen levels in this population as well that may occur with either sleep or with exercise because that would be another important target of treatment so I think one of the big questions that remains even though we wrote a guidelines document three years ago is how we should be treating patients with pulmonary hypertension from my clinical experience patients who have hemodynamics or hemodynamic numbers that are consistent with pulmonary arterial hypertension those patients do actually respond well to medications that treat other types of patients with cobra hypertension and many of us in the field will treat our patients with these medications if they meet those qualifications but I think it really is important for patients with sickle cell disease and pulmonary hypertension to be treated in the center where there is expertise in both aspects of their disease the pulmonary hypertension piece as well as the sickle cell disease piece because there really is it is necessary for sort of a multidisciplinary treatment plan to this I'm doctor loose clings and I'm aware that I'm rare [Music] write for me capstone thesis examples order School of Jazz.

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