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Children's Hospital Oakland new sickle cell research

July 6, 2005-Oakland, CA-- A new study led by a researcher at Children's Hospital & Research Center at Oakland is the first to find that an amino acid deficiency in sickle cell disease is the result of hemolysis, a process where red blood cells rupture and release their contents into the blood stream. Low availability of the amino acid arginine is associated with lung disease and death in adult sickle cell patients.

The study, published in the July 6th issue of the Journal of American Medical Association (JAMA) supports new therapeutic strategies aimed at increasing arginine levels either through nutritional supplements or by developing new drug therapies that disable the arginase enzyme that consumes arginine.

"Lower amino acid ratios, which indicates low arginine availability, were found in patients who had more severe pulmonary hypertension, a condition involving high blood pressure in arteries carrying blood from the heart to small vessels in the lungs," said Claudia Morris, M.D., an Emergency Department physician and researcher at Children's Hospital Oakland. "This incurable condition is the leading cause of death in adults with sickle cell disease," she added.

The published report in JAMA also highlights important new warning signs that may predict the severity of the disease and could be useful in identifying adult patients at risk for early death through a simple blood test that measures amino acid levels. The study is the result of a multi-center collaboration that includes Dr. Morris as lead investigator, Mark Gladwin, M.D. and Greg Kato M.D. from the National Heart, Lung, and Blood Institute, which is a part of the National Institutes of Health and Sidney Morris Jr., Ph.D from the University of Pittsburgh School of Medicine.

"With this new finding and continued research, we hope to increase our understanding of the development of pulmonary hypertension not only in sickle cell disease, but in thalassemia (Cooleys anemi
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Contact: Diana Yee
dyee@mail.cho.org
510-428-3120
Children's Hospital & Research Center at Oakland
5-Jul-2005


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