title: Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease.
abstract: Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.
authors.affiliation: Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, USA.
authors.forename: F M
authors.initials: FM
authors.lastname: Gill
authors.forename: L A
authors.initials: LA
authors.lastname: Sleeper
authors.forename: S J
authors.initials: SJ
authors.lastname: Weiner
authors.forename: A K
authors.initials: AK
authors.lastname: Brown
authors.forename: R
authors.initials: R
authors.lastname: Bellevue
authors.forename: R
authors.initials: R
authors.lastname: Grover
authors.forename: C H
authors.initials: CH
authors.lastname: Pegelow
authors.forename: E
authors.initials: E
authors.lastname: Vichinsky
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000453:epidemiology* / Q000401:mortality; D016470:Bacteremia / Q000453:epidemiology; D002561:Cerebrovascular Disorders / Q000453:epidemiology / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D004198:Disease Susceptibility; D018572:Disease-Free Survival; D017707:Erythrocyte Transfusion / Q000706:statistics & numerical data; D005260:Female; D006395:Hemarthrosis / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D015994:Incidence; D007223:Infant; D015993:Life Tables; D008171:Lung Diseases / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008581:Meningitis / Q000453:epidemiology; D010146:Pain / Q000453:epidemiology / Q000209:etiology; D012805:Sickle Cell Trait / Q000150:complications / Q000235:genetics / Q000401:mortality; D013158:Splenic Diseases / Q000209:etiology / Q000401:mortality; D014481:United States / Q000453:epidemiology; D017085:alpha-Thalassemia / Q000150:complications / Q000453:epidemiology / Q000235:genetics; D017086:beta-Thalassemia / Q000150:complications / Q000235:genetics / Q000401:mortality