Mortality in Children and Adolescents With Sickle Cell Disease
TLDR
Comparison of this study's overall mortality with previous reports indicates improvement of survival in US patients less than 20 years of age with sickle hemoglobinopathies, and this improvement is most likely due to parental education and counseling about the illness and the early institution of antibiotics in suspected infections.Abstract:
A study of the natural history of sickle hemoglobinopathies was begun in March 1979. By August 1987, a total of 2824 patients less than 20 years of age were enrolled. There have been 14,670 person-years of follow-up. Seventy-three deaths have occurred. Most of the deaths were in patients with hemoglobin SS. The peak incidence of death was between 1 and 3 years of age, and the major cause in these young patients was infection. Cerebrovascular accidents and traumatic events exceeded infections as a cause of death in patients greater than 10 years of age. There was limited success in identifying risk factors for death. Comparison of this study's overall mortality of 2.6% (0.5 deaths per 100 person-years) with previous reports indicates improvement of survival in US patients less than 20 years of age with sickle hemoglobinopathies. This improvement is most likely due to parental education and counseling about the illness and the early institution of antibiotics in suspected infections.read more
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Journal ArticleDOI
Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death
Orah S. Platt,Donald Brambilla,Wendell F. Rosse,Paul F. Milner,Oswaldo Castro,Martin H. Steinberg,Panpit P. Klug +6 more
TL;DR: Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death.
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Sickle cell disease
TL;DR: New strategies for specific therapy, including expanded use of chronic transfusions, bone marrow transplantation, and hydroxyurea, now offer hope for prevention of many or all of the hemolytic and vaso-occlusive manifestations of sickle cell disease.
Journal ArticleDOI
Cerebrovascular Accidents in Sickle Cell Disease: Rates and Risk Factors
Kwaku Ohene-Frempong,Steven J. Weiner,Lynn A. Sleeper,Scott T. Miller,Stephen H. Embury,John W. Moohr,Doris L. Wethers,Charles H. Pegelow,Frances M. Gill +8 more
TL;DR: The highest rates of prevalence of CVA and incidence were in sickle cell anemia (SS) patients, but CVA occurred in all common genotypes, and the incidence of infarctive CVA was lowest in SS patients 20 to 29 years of age and higher in children and older patients.
Journal ArticleDOI
Pain in sickle cell disease. Rates and risk factors.
Orah S. Platt,Bruce D. Thorington,Donald Brambilla,Paul F. Milner,Wendell F. Rosse,Elliott Vichinsky,Thomas R. Kinney +6 more
TL;DR: The "pain rate" is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20, and even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival.
Journal ArticleDOI
Bone marrow transplantation for sickle cell disease.
Mark C. Walters,M. Patience,Wendy M. Leisenring,Eckman,Scott Jp,William C. Mentzer,Sally C. Davies,Kwaku Ohene-Frempong,Françoise Bernaudin,Dana C. Matthews,R Storb,Keith M. Sullivan +11 more
TL;DR: Allogeneic stem-cell transplantation can be curative in young patients with symptomatic sickle cell disease and Kaplan-Meier estimates of survival and event-free survival at four years were 91 percent and 73 percent, respectively.