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Risk for stroke, death not higher for sickle cell children with early complications

DALLAS Jan. 29, 2007 Children with sickle cell disease who experienced major complications such as pain and lung disease early in life are at no greater risk for stroke or death during later childhood, new research from UT Southwestern Medical Center shows.

In addition, sickle cell children who have pain episodes ("crises") or dactylitis, a type of painful swelling of the hands and feet, as infants or toddlers are at no greater risk of having those symptoms recur in later childhood. The study's results, however, showed that children hospitalized for chest problems early on are more likely to see those problems recur up to adulthood.

The study following more than 200 children with sickle cell disease from birth through teenage years appears in the January issue of Blood, the scientific journal of the American Society of Hematology.

The findings are an important step in trying to identify predictors that reveal how the mysterious disease will progress as children age, said Dr. Charles Quinn, assistant professor of pediatrics at UT Southwestern and the study's lead author.

"Everybody who has sickle cell disease is affected differently by the disease. Some seem to have a lot of problems with pain and lung disease and some have very few problems and may have a normal life span," said Dr. Quinn. "We don't really understand why everyone with the same disease can be so different."

The myriad medical issues make it difficult when counseling parents of babies with sickle cell disease about what they can expect, he said. "We can't give them very much in the way of specifics, exactly what this child will likely go through or what to expect from the disease in the future," said Dr. Quinn, a pediatric hematology specialist at Children's Medical Center Dallas.

People with sickle cell disease have a genetic error in their hemoglobin. The disease turns the usually soft, round red blood cell that carries oxy
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Contact: Russell Rian
russell.rian@utsouthwestern.edu
214-648-3404
UT Southwestern Medical Center
29-Jan-2007


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