Preventing Infections in Sickle Cell Disease: The Unfinished Business

Obaro, Stephen K.; Tam, Pui Ying Iroh

doi:10.1002/pbc.25911

Cited by 22 publications

(21 citation statements)

References 50 publications

(76 reference statements)

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“…The predominance of NTS species and other enteric Gram-negatives suggests a need to re-evaluate optimal antimicrobial prophylaxis and empirical treatment in this population. [28] Despite its success in resource-rich settings, penicillin prophylaxis may not be optimal for PCV and Hib-vaccinated patients with SCA in The Gambia. However, it is unclear whether possible alternatives such as co-trimoxazole or azithromycin would be safe or cost-effective.…”

Section: Discussionmentioning

confidence: 99%

Invasive bacterial infections in Gambians with sickle cell anemia in an era of widespread pneumococcal and hemophilus influenzae type b vaccination

et al. 2016

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There is relatively little data on the etiology of bacterial infections in patients with sickle cell anemia (SCA) in West Africa, and no data from countries that have implemented conjugate vaccines against both Streptococcus pneumoniae and Haemophilus influenzae type b (Hib).We conducted a retrospective analysis of SCA patients admitted to the Medical Research Council Unit, The Gambia, during a 5-year period when there was high coverage of Hib and Pneumococcal conjugate vaccination. We evaluated 161 admissions of 126 patients between April 2010 and April 2015.Pathogenic bacteria were identified in blood cultures from 11 of the 131 admissions that had cultures taken (8.4%, 95% CI 4.5–14.1%). The most frequent isolate was Salmonella Typhimurium (6/11; 54.5%), followed by Staphylococcus aureus (2/11; 18.2%) and other enteric Gram-negative pathogens (2/11; 18.2%) and there was 1 case of H influenzae non-type b bacteremia (1/11; 9.1%). There were no episodes of bacteremia caused by S pneumoniae or Hib.The low prevalence of S pneumoniae and Hib and the predominance of nontyphoidal Salmonella as a cause of bacteremia suggest the need to reconsider optimal antimicrobial prophylaxis and the empirical treatment regimens for patients with SCA.

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Section: Discussionmentioning

confidence: 99%

Invasive bacterial infections in Gambians with sickle cell anemia in an era of widespread pneumococcal and hemophilus influenzae type b vaccination

et al. 2016

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“…Long-term penicillin prophylaxis has raised concerns about the development of penicillin-resistant pneumococcal colonization and disease 204 , especially in low-income countries, although the benefit to risk ratio of prophylaxis is still high. The pneumococcal conjugate vaccine PCV13 and pneumococcal polysaccharide vaccine PPSV23 205 can prevent infection by most -but not all -serotypes.…”

Section: [H2] Prevention Of Complicationsmentioning

confidence: 99%

Sickle cell disease

Kato

Piel

Reid

et al. 2018

Nat Rev Dis Primers

938

929

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Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. Common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs. Hydroxycarbamide, blood transfusions and haematopoietic stem cell transplantation can reduce the severity of the disease. Early diagnosis is crucial to improve survival, and universal newborn screening programmes have been implemented in some countries but are challenging in low-income, high-burden settings.

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“…[23][24][25] In childhood, recurrent splenic infarction from sickled RBCs increases the risk for life-threatening infections. 10,[26][27][28][29] In addition, young children with SCD are at risk of life-threatening cerebral vasculopathy. 29 Afflicted patients who survive to adulthood can suffer both acute and chronic painful crises as well as cumulative organ damage and early mortality.…”

Section: Introductionmentioning

confidence: 99%