Children with sickle cell anemia have an increased susceptibility to bacterial infections, especially to those caused by Streptococcus pneumoniae. We therefore conducted a multicenter, randomized, double-blind, placebo-controlled clinical trial to test whether the regular, daily administration of oral penicillin would reduce the incidence of documented septicemia due to S.pneumoniae in children with sickle cell anemia who were under the age of three years at the time of entry. The children were randomly assigned to receive either 125 mg of penicillin V potassium (105 children) or placebo (110 children) twice daily. The trial was terminated 8 months early, after an average of 15 months of follow-up, when an 84 percent reduction in the incidence of infection was observed in the group treated with penicillin, as compared with the group given placebo (13 of 110 patients vs. 2 of 105; P = 0.0025), with no deaths from pneumococcal septicemia occurring in the penicillin group but three deaths from the infection occurring in the placebo group. On the basis of these results, we conclude that children should be screened in the neonatal period for sickle cell hemoglobinopathy and that those with sickle cell anemia should receive prophylactic therapy with oral penicillin by four months of age to decrease the morbidity and mortality associated with pneumococcal septicemia.
This article has been cited by other articles:
Obaro, S
(2009). Pneumococcal infections and sickle cell disease in Africa: does absence of evidence imply evidence of absence?. Arch. Dis. Child.
94: 713-716
[Abstract][Full Text]
Bailey, D. B. Jr, Armstrong, F. D., Kemper, A. R., Skinner, D., Warren, S. F.
(2009). Supporting Family Adaptation to Presymptomatic and "Untreatable" Conditions in an Era of Expanded Newborn Screening. J Pediatr Psychol
34: 648-661
[Abstract][Full Text]
Ross, L. F.
(2009). Ethical and Policy Issues in Newborn Screening: Historical, Current, and Future Developments. NeoReviews
10: e71-e81
[Abstract][Full Text]
Howard-Jones, M, Randall, L, Bailey-Squire, B, Clayton, J, Jackson, N
(2009). An audit of immunisation status of sickle cell patients in Coventry, UK. J. Clin. Pathol.
62: 42-45
[Abstract][Full Text]
Rahimy, M C, Gangbo, A, Ahouignan, G, Alihonou, E
(2009). Newborn screening for sickle cell disease in the Republic of Benin. J. Clin. Pathol.
62: 46-48
[Abstract][Full Text]
Dick, M C
(2008). Standards for the management of sickle cell disease in children. EDUCATION AND PRACTICE
93: 169-176
[Abstract][Full Text]
Boemer, F., Ketelslegers, O., Minon, J.-M., Bours, V., Schoos, R.
(2008). Newborn Screening for Sickle Cell Disease Using Tandem Mass Spectrometry. Clin. Chem.
54: 2036-2041
[Abstract][Full Text]
Adamkiewicz, T. V., Silk, B. J., Howgate, J., Baughman, W., Strayhorn, G., Sullivan, K., Farley, M. M.
(2008). Effectiveness of the 7-Valent Pneumococcal Conjugate Vaccine in Children With Sickle Cell Disease in the First Decade of Life. Pediatrics
121: 562-569
[Abstract][Full Text]
Boyd, J. H., Macklin, E. A., Strunk, R. C., DeBaun, M. R.
(2007). Asthma is associated with Increased mortality in individuals with sickle cell anemia. haematol
92: 1115-1118
[Abstract][Full Text]
Villella, A. D.
(2007). The Effect of the Pneumococcal Conjugate Vaccine on Children with Sickle Cell Disease. AAP Grand Rounds
18: 1-2
[Full Text]
Roberts, I., de Montalembert, M.
(2007). Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in Europe. haematol
92: 865-871
[Full Text]
Telfer, P., Coen, P., Chakravorty, S., Wilkey, O., Evans, J., Newell, H., Smalling, B., Amos, R., Stephens, A., Rogers, D., Kirkham, F.
(2007). Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. haematol
92: 905-912
[Abstract][Full Text]
Kizito, M E, Mworozi, E, Ndugwa, C, Serjeant, G R
(2007). Bacteraemia in homozygous sickle cell disease in Africa: is pneumococcal prophylaxis justified?. Arch. Dis. Child.
92: 21-23
[Abstract][Full Text]
Okumura, M. J., Campbell, A. D., Nasr, S. Z., Davis, M. M.
(2006). Inpatient Health Care Use Among Adult Survivors of Chronic Childhood Illnesses in the United States.. Arch Pediatr Adolesc Med
160: 1054-1060
[Abstract][Full Text]
Kaye, C. I., and the Committee on Genetics,
(2006). Newborn Screening Fact Sheets. Pediatrics
118: e934-e963
[Abstract][Full Text]
Smith, L. A., Oyeku, S. O., Homer, C., Zuckerman, B.
(2006). Sickle Cell Disease: A Question of Equity and Quality. Pediatrics
117: 1763-1770
[Abstract][Full Text]
Grosse, S. D., Boyle, C. A., Kenneson, A., Khoury, M. J., Wilfond, B. S.
(2006). From Public Health Emergency to Public Health Service: The Implications of Evolving Criteria for Newborn Screening Panels. Pediatrics
117: 923-929
[Full Text]
Botkin, J. R.
(2005). Research for Newborn Screening: Developing a National Framework. Pediatrics
116: 862-871
[Abstract][Full Text]
Serjeant, G. R
(2005). Mortality from sickle cell disease in Africa. BMJ
330: 432-433
[Full Text]
Weatherall, D., Hofman, K., Rodgers, G., Ruffin, J., Hrynkow, S.
(2005). A case for developing North-South partnerships for research in sickle cell disease. Blood
105: 921-923
[Abstract][Full Text]
Wurst, K. E., Sleath, B. L.
(2004). Physician knowledge and adherence to prescribing antibiotic prophylaxis for sickle cell disease. Int J Qual Health Care
16: 245-251
[Abstract][Full Text]
Quinn, C. T., Rogers, Z. R., Buchanan, G. R.
(2004). Survival of children with sickle cell disease. Blood
103: 4023-4027
[Abstract][Full Text]
Sox, C. M., Cooper, W. O., Koepsell, T. D., DiGiuseppe, D. L., Christakis, D. A.
(2003). Provision of Pneumococcal Prophylaxis for Publicly Insured Children With Sickle Cell Disease. JAMA
290: 1057-1061
[Abstract][Full Text]
Little, J., Khoury, M. J., Bradley, L., Clyne, M., Gwinn, M., Lin, B., Lindegren, M.-L., Yoon, P.
(2003). The Human Genome Project Is Complete. How Do We Develop a Handle for the Pump?. Am J Epidemiol
157: 667-673
[Full Text]
Khoury, M. J., McCabe, L. L., McCabe, E. R.B.
(2003). Population Screening in the Age of Genomic Medicine. NEJM
348: 50-58
[Full Text]
Hepburn, A L, Davies, K A, Gilliland, W R, Tsokos, G C
(2002). Infection and SLE. Ann Rheum Dis
61: 668-669
[Full Text]
Section on Hematology/Oncology and Committee on Ge,
(2002). Health Supervision for Children with Sickle Cell Disease. Pediatrics
109: 526-535
[Abstract][Full Text]
MINTER, K. R., GLADWIN, M. T.
(2001). Pulmonary Complications of Sickle Cell Anemia . A Need for Increased Recognition, Treatment, and Research. Am. J. Respir. Crit. Care Med.
164: 2016-2019
[Full Text]
(2001). Acute complications of sickle cell disease in children. DTB
39: 33-37
[Abstract][Full Text]
Mentzer, W. C., Kan, Y. W.
(2001). Prospects for Research in Hematologic Disorders: Sickle Cell Disease and Thalassemia. JAMA
285: 640-642
[Abstract][Full Text]
O'Brien, K. L., Swift, A. J., Winkelstein, J. A., Santosham, M., Stover, B., Luddy, R., Gootenberg, J. E., Nold, J. T., Eskenazi, A., Snader, S. J., Lederman, H. M., for the Pneumococcal Conjugate Vaccine Study Group,
(2000). Safety and Immunogenicity of Heptavalent Pneumococcal Vaccine Conjugated to CRM197 Among Infants With Sickle Cell Disease. Pediatrics
106: 965-972
[Abstract][Full Text]
Wayne, A. S., Schoenike, S. E., Pegelow, C. H.
(2000). Financial analysis of chronic transfusion for stroke prevention in sickle cell disease. Blood
96: 2369-2372
[Abstract][Full Text]
(2000). Update: Newborn Screening for Sickle Cell Disease--California, Illinois, and New York, 1998. JAMA
284: 1373-1374
[Full Text]
Nowak-Wegrzyn, A., Winkelstein, J. A., Swift, A. J., Lederman, H. M., The Pneumococcal Conjugate Vaccine Study Group,
(2000). Serum Opsonic Activity in Infants with Sickle-Cell Disease Immunized with Pneumococcal Polysaccharide Protein Conjugate Vaccine. CVI
7: 788-793
[Abstract][Full Text]
Overturf, G. D., the Committee on Infectious Diseases, , Committee on Infectious Diseases, 1999-2000, , Ex-Officio, , Liaisons, , Consultant, , Staff,
(2000). Technical Report: Prevention of Pneumococcal Infections, Including the Use of Pneumococcal Conjugate and Polysaccharide Vaccines and Antibiotic Prophylaxis. Pediatrics
106: 367-376
[Abstract][Full Text]
Bowman, J. E.
(2000). Anthropology: From Bones to the Human Genome. The ANNALS of the American Academy of Political and Social Science
568: 140-153
[Abstract]
Steinberg, M. H.
(1999). Management of Sickle Cell Disease. NEJM
340: 1021-1030
[Full Text]
Cronin, E K, Normand, C, Henthorn, J S, Hickman, M, Davies, S C
(1998). Costing model for neonatal screening and diagnosis of haemoglobinopathies. Arch. Dis. Child. Fetal Neonatal Ed.
79: 161F-167
[Abstract][Full Text]
Cohen, A. R.
(1998). Sickle Cell Disease -- New Treatments, New Questions. NEJM
339: 42-44
[Full Text]
Teach, S. J., Lillis, K. A., Grossi, M.
(1998). Compliance With Penicillin Prophylaxis in Patients With Sickle Cell Disease. Arch Pediatr Adolesc Med
152: 274-278
[Abstract][Full Text]
Holtzman, N. A., Murphy, P. D., Watson, M. S., Barr, P. A.
(1997). Predictive Genetic Testing: From Basic Research to Clinical Practice. Science
278: 602-605
[Abstract][Full Text]
Davies, S. C, Oni, L.
(1997). Fortnightly review: Management of patients with sickle cell disease. BMJ
315: 656-660
[Full Text]
Daw, N. C., Wilimas, J. A., Wang, W. C., Presbury, G. J., Joyner, R. E., Harris, S. C., Davis, Y., Chen, G., Chesney, P. J.
(1997). Nasopharyngeal Carriage of Penicillin-resistant Streptococcus pneumoniae in Children With Sickle Cell Disease. Pediatrics
99: e7-e7
[Abstract][Full Text]
Section on Hematology/Oncology,
(1997). Guidelines for the Pediatric Cancer Center and Role of Such Centers in Diagnosis and Treatment. Pediatrics
99: 139-141
[Abstract][Full Text]
,
(1996). Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. BMJ
312: 430-434
[Full Text]
Lee, A., Thomas, P., Cupidore, L., Serjeant, B., Serjeant, G.
(1995). Improved survival in homozygous sickle cell disease: lessons from a cohort study. BMJ
311: 1600-1602
[Abstract][Full Text]
Pejaver, R.K., Ahmad, F., Bedawi, H.
(1995). Sickle cell anaemia in Saudi-Arabian children. The Journal of the Royal Society for the Promotion of Health
115: 156-158
[Abstract]
Sprinkle, R. H., Konrad, T. R.
(1995). Is Universal Neonatal Hemoglobinopathy Screening Cost-effective?-Reply. Arch Pediatr Adolesc Med
149: 466-467
[Abstract]
Sprinkle, R. H., Hynes, D. M., Konrad, T. R.
(1994). Is Universal Neonatal Hemoglobinopathy Screening Cost-Effective?. Arch Pediatr Adolesc Med
148: 461-469
[Abstract]
HILL, S. A.
(1994). MOTHERHOOD AND THE OBFUSCATION OF MEDICAL KNOWLEDGE:: The Case of Sickle Cell Disease. Gender Society
8: 29-47
[Abstract]
Caskey, C. T.
(1993). Molecular Medicine: A Spin-off From the Helix. JAMA
269: 1986-1992
[Abstract]
Brown, R. T., Armstrong, F. D., Eckman, J. R.
(1993). Neurocognitive Aspects of Pediatric Sickle Cell Disease. J Learn Disabil
26: 33-45
[Abstract]
Janoff, E. N., Breiman, R. F., Daley, C. L., Hopewell, P. C.
(1992). Pneumococcal Disease during HIV Infection: Epidemiologic, Clinical, and Immunologic Perspectives. ANN INTERN MED
117: 314-324
[Abstract]
Listernick, R., Frisone, L., Silverman, B. L.
(1992). Delayed Diagnosis of Infants With Abnormal Neonatal Screens. JAMA
267: 1095-1099
[Abstract]
McCabe, E R
(1991). Utility of PCR for DNA analysis from dried blood spots on filter paper blotters.. Genome Res
1: 99-106
Githens, J. H., Lane, P. A., McCurdy, R. S., Houston, M. L., McKinna, J. D., Cole, D. M.
(1990). Newborn Screening for Hemoglobinopathies in Colorado: The First 10 Years. Arch Pediatr Adolesc Med
144: 466-470
[Abstract]
McKinney, W. P., Young, M. J., Hartz, A., Bi-Fong Lee, M.
(1989). The Inexact Use of Fisher's Exact Test in Six Major Medical Journals. JAMA
261: 3430-3433
[Abstract]
Rogers, Z. R., Powars, D. R., Kinney, T. R., Williams, W. D., Schroeder, W. A.
(1989). Nonblack Patients With Sickle Cell Disease Have African {beta}s Gene Cluster Haplotypes. JAMA
261: 2991-2994
[Abstract]
Powars, D., Overturf, G.
(1987). Penicillin in Sickle Cell Anemia: A Panacea for the Lost Spleen?. Arch Pediatr Adolesc Med
141: 250-252
[Abstract]
