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Clinical events in the first decade in a cohort of infants with sickle cell
disease. Cooperative Study of Sickle Cell Disease [see comments]
FM Gill, LA Sleeper, SJ Weiner, AK Brown, R Bellevue, R Grover, CH Pegelow and E Vichinsky
Department of Pediatrics, University of Pennsylvania School of Medicine,
Philadelphia, USA.
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.
Volume 86,
Issue 2,
pp. 776-783,
07/15/1995
Copyright © 1995 by The American Society of Hematology
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