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High blood pressure in the lungs a major risk for death in adults with sickle cell disease

A new study reveals that nearly one third of adults with sickle cell disease develop high blood pressure in their lungs and that the condition, known as pulmonary hypertension, causes a much higher death rate in patients with the complication than those without it. The findings, according to researchers from the Warren Grant Magnuson Clinical Center at the National Institutes of Health (NIH), demonstrate an urgent need to diagnose this complication in adults with sickle cell disease as it is a major risk factor for death. The study was conducted as a multi-center collaboration between NIH and the Howard University Center for Sickle Cell Disease. A complete report will publish in the February 26 edition of the New England Journal of Medicine.

Sickle cell disease is a chronic, often fatal anemia that is classically characterized by severe attacks of pain from blood vessels being blocked by red blood cells that become rigid and form a sickle shape when deoxygenated. In the United States this genetic disease occurs predominantly in people of African descent, and is accompanied by episodic severe pain in the joints, leg ulcers, jaundice and multi-organ failure. A serious complication of sickle cell disease is pulmonary hypertension.

Pulmonary hypertension is high blood pressure (not related to the pressure measured by cuff on the arm) in the arteries that supply the lungs. The blood vessels that supply the lungs narrow and their walls thicken, so they can't carry as much blood. Like a kinked garden hose, pressure builds up and backs up. The heart works harder, trying to force the blood through. If the pressure is high enough, eventually the heart can't keep up, and is unable to pump enough blood through the lungs to pick up adequate amounts of oxygen. Patients become tired, dizzy and short of breath. When an underlying cause can't be found, the condition is called primary pulmonary hypertension. When a pre-existing disease such as sickle cell trig
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Contact: Dianne Needham
301-496-2563
NIH/National Institutes of Health
25-Feb-2004


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