title: Infection in major sickle hemoglobinopathies: should management strategies change?
abstract: Four children with major sickle hemoglobinopathies developed severe pneumococcal infection. Three had sickle cell hemoglobin C (Hb SC) disease and one had sickle cell anemia (Hb SS). In three instances, there was a fatal outcome. The authors' experience with these cases leads them to question whether any patient with a major sickle hemoglobinopathy should be excluded from receiving prophylactic penicillin or if outpatient management with long-acting cephalosporin treatment in the sickle cell patient with suspected sepsis is appropriate therapy.
authors.affiliation: Sinai Hospital of Baltimore.
authors.forename: D
authors.initials: D
authors.lastname: Freeman
authors.forename: R E
authors.initials: RE
authors.lastname: Luddy
authors.forename: A D
authors.initials: AD
authors.lastname: Schwartz
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D002648:Child; D005260:Female; D006450:Hemoglobin SC Disease / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D011008:Pneumococcal Infections / Q000150:complications* / Q000188:drug therapy / Q000517:prevention & control