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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with sickle cell disease are predisposed to infection caused by Streptococcus pneumoniae. However, there has been only one published case of bacteremic pneumococcal meningitis in an adult with sickle cell anemia. We report here the cases of six adults with sickle cell disease, pneumococcal sepsis, and meningitis. Five patients were male and one was female. Their ages ranged from 18 to 34 years (mean, 25.7 years). Five patients had the SS and one had the SC hemoglobin phenotype. Only one patient had received pneumococcal vaccine (14 valent). This vaccine did not protect against the pneumococcal serotype causing his infection. All patients had high fever (mean, 39.8 degrees C [103.7 degrees F]) on admission; five had generalized weakness and four had neck stiffness. Leukocyte counts were greater than 30,000/mm3 in all patients. Streptococcus pneumoniae was isolated from the blood and the cerebrospinal fluid in all patients. The cerebrospinal fluid showed pleocytosis in six patients, an elevated protein level in five, and hypoglycorrhachia in two. Complications included renal failure in four patients, disseminated intravascular coagulation in one, and seizures in another. Two patients died. Pneumococcal sepsis and meningitis are uncommon in adults with sickle cell disease, but they carry a high morbidity and mortality. Wider use of the new 23-valent polysaccharide vaccine in these patients is recommended.
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PMID:Pneumococcal sepsis and meningitis in adults with sickle cell disease. 240 40

Although polyvalent pneumococcal vaccine and prophylactic penicillin are used to prevent overwhelming Streptococcus pneumoniae septicemia in infants and young children with sickle cell anemia, infection rates remain high. We have reviewed our seven-year experience with a regimen of twice daily oral penicillin V potassium prophylaxis in 88 affected children. The median age at the start of prophylaxis was 10 months, and the median duration of prophylaxis was 29 months (range, three months to seven years). The total period of observation of patients who were prescribed penicillin was 248 person-years. Most patients also received one or two doses of polyvalent pneumococcal vaccine. Despite penicillin prophylaxis and pneumococcal vaccine, eight episodes of S pneumoniae septicemia have occurred and three have been fatal. Four episodes were in children older than 3 years. Suboptimal compliance with the prescribed oral penicillin regimen was usually apparent. With one possible exception, the infections occurred when penicillin had not been taken during the previous 24 hours. The S pneumoniae septicemia rate in this patient population, 3.2 per 100 person-years, is somewhat less than that described in previous reports of children not receiving penicillin but is still unacceptably high. Vigorous advocacy of a penicillin prophylaxis regimen does not eliminate the risk of pneumococcal septicema in this patient population.
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PMID:Pneumococcal septicemia despite pneumococcal vaccine and prescription of penicillin prophylaxis in children with sickle cell anemia. 396 35

Pneumococcal sepsis has contributed to increased morbidity and mortality in patients with sickle cell disease. This case highlights the urgency of early diagnosis and the necessity of aggressive intervention in the management of sickle cell patients, particularly during the years of early childhood. It cannot be overemphasized that sickle cell patients, especially those less than 5 years of age, should be monitored carefully and institution of parenteral antibiotics should be undertaken early, even in those patients that present with mild symptoms.
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PMID:Pneumococcal sepsis in children with sickle cell disease. 671 4

Pneumococcal sepsis and/or meningitis are major causes of morbidity and mortality in young children with sickle cell disease. Abnormal complement activity, poor splenic function and a lack of type-specific pneumococcal antibody are responsible for the severity and frequency of these infections. A program consisting of early institution of antibiotic therapy for febrile episodes, antimicrobial prophylaxis, and administration of pneumococcal vaccine may be effective in reducing the incidence of pneumococcal disease. Specific guidelines for infection prevention are presented. Other infections that are more frequent or more severe in children with sickle cell disease (e.g., Salmonella, Haemophilus and mycoplasma infections) are also discussed.
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PMID:Infections in children with sickle cell anemia. Special reference to pneumococcal and salmonella infections. 716 89