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For children with sickle cell disease, lung disease is part of the package
Date:5/18/2008

data was collected from a large number of children with sickle cell disease across childhood.

They also found that of children with SCD, hemoglobin-beta genotype SS was associated with an increased risk of loss of lung function across childhood. The hemoglobin SS genotype is more common than hemoglobin SC genotype (one in 600 versus one in 800 life births) and is associated with more complications than hemoglobin SC disease. This pattern of decline in lung function may simply reflect a greater risk of lung injury in this group, but alternatively may also suggest differences in response to lung injury or lung repair with different genotypes of sickle cell disease.

Our findings confirm that lung disease in SCD begins in childhood, explained Dr. MacLean. Using statistical modeling, we are able to predict the rate of decline of lung function. These results can be used as a baseline against which results from intervention studies can be compared.

While changes in treatment over the last 20 years have led to a significant reduction in overall mortality and morbidity for children with SCD, prospective longitudinal studies focusing on the identification and treatment of sickle cell associated lung disease are needed to continue to improve the long term health of children and adults with sickle disease.

The results of this project emphasize the need for further investigation into the pathophysiology and treatment of lung disease in children with sickle cell disease, concluded Dr. MacLean.


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Contact: Keely Savoie
ksavoie@thoracic.org
212-315-8620
American Thoracic Society
Source:Eurekalert

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