@article{3e841b8c0d9a42c7bd7a9adb33688eec,
title = "Fatal pneumococcal septicemia in hemoglobin SC disease",
abstract = "We retrospectively examined the medical and autopsy records of seven previously unpublished cases of fatal pneumococcal septicemia in children with hemoglobin SC disease. The earliest death occurred in a 1-year-old child who had congenital heart disease with cyanosis; the other children were aged 31/2 to 15 years. Only one child had received pneumococcal vaccine or prophylactic penicillin therapy. All seven children had an acute febrile illness and rapid clinical deterioration despite parenterally administered antibiotic therapy and intensive medical support. Erythrocyte pit counts in two patients were 40.3% and 41.7%, respectively (normal, ≤3.6%). Autopsy data from five cases showed marked splenic congestion without infarction in five, splenomegaly in four, and bilateral adrenal hemorrhage in three. These cases illustrate that functional asplenia predisposes some children with hemoglobin SC disease to the development of fatal septicemia after the age of 3 years. We conclude that pneumococcal vaccine should be administered to all children with hemoglobin SC disease and that acute febrile illnesses should be investigated promptly for the possibility of septicemia. The routine use of prophylactic penicillin therapy in infants and children with hemoglobin SC disease remains controversial.",
author = "Lane, {Peter A.} and Rogers, {Zora R.} and Woods, {Gerald M.} and Wang, {Winfred C.} and Wilimas, {Judith A.} and Miller, {Scott T.} and Yusuf Khakoo and Buchanan, {George R.}",
note = "Funding Information: In many children with sickle cell anemia (homozygous hemoglobin S), functional asplenia develops during the first year of life, 1 and septicemia is the leading cause of death in childhood. 24 The risk of septicemia in sickle cell anemia is Supported in part by a grant from the Biomedical Research Support Grant Program (No. BRSG-05357) of the Division of Research Resources, National Institutes of Health. Dr. Rogers is the Gladyce Begelman Fellow of the Damon Runyon-Walter Winchell Cancer Research Foundation (DRW-054). Submitted for publication Dec. 21, 1993; accepted Jan. 7, 1994. Reprint requests: Peter A. Lane, MD, Campus Box C-222, University of Colorado Health Sciences Center, 4200 East Ninth Ave., Denver, CO 80262. Copyright 9 1994 by Mosby-Year Book, Inc. 0022-3476/94/$3.00 + 0 9/20/54243 greatest during the first 3 years of life 25 and is reduced markedly by prophylactic penicillin therapy. 6, 7 Considerably less is known about splenic dysfunction and the risk of overwhelming sepsis in children with hemoglobin SC disease. Functional asplenia has been documented by radionuclide liver-spleen scans in some adult patients with hemoglobin SC disease) An elevated erythrocyte pit count, a finding that indicates functional asplenia in children with sickle cell anemia\] also has been found in some children with hemoglobin SC disease, l' 9 The incidence of bacteremia is highest during the first 2 years of life, 5 and increased rates of respiratory infection and pneumococcal bacteremia have been reported in children with hemoglobin SC compared with those without hemoglobinopathy.l~ In a retrospective review of 51 children with hemoglobin SC followed for 370 person-years, 7 serious bacterial infections",
year = "1994",
month = jun,
doi = "10.1016/S0022-3476(05)83171-3",
language = "English (US)",
volume = "124",
pages = "859--862",
journal = "The Journal of pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",
number = "6",
}