Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization

Makani, Julie; Komba, Albert; Cox, Sharon E.; Oruo, Julie; Mwamtemi, Khadija; Kitundu, Jesse; Magesa, Pius; Rwezaula, Stella; Meda, Elineema; Mgaya, Josephine; Pallangyo, Kisali; Okiro, Emelda A.; Muturi, David; Newton, Charles R.; Fegan, Greg; Marsh, Kevin; Williams, Thomas N.

doi:10.1182/blood-2009-07-233528

Cited by 144 publications

(156 citation statements)

References 26 publications

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“…This finding is similar to the low malaria prevalence found recently in Dar es Salaam among sickle cell anaemia patients (Makani et al, 2010). Low prevalence of malaria among patients with HbSS has also been reported in other studies in Africa (Freidman et al, 1978;Aluoch, 1997;Cholera et al, 2008;Komba et al, 2009;Sadarangani et al, 2009).…”

Section: Discussionsupporting

confidence: 78%

High prevalence of Plasmodium falciparum pfcrt K76T mutation in children with sickle cell disease at a tertiary hospital in north-western Tanzania

Mongella

Enweji

Mnongone

et al. 2014

Tanzania J Hlth Res

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Abstract:The high prevalence of sickle cell disease (SCD) and trait in Sub-Saharan Africa coincides with the distribution of Plasmodium falciparum malaria. Due to prolonged heavy use of chloroquine (CQ) as an antimalarial, drug resistance has developed. Many countries including Tanzania abandoned the use of CQ for uncomplicated malaria, except its use as prophylaxis in patients with sickle cell disease. This study investigated the prevalence of malaria in SCD patients and mutations associated with CQ resistance. Children diagnosed with sickle cell disease attending both outpatient clinic and those admitted at Bugando Medical Centre in north-western Tanzania were screened for malaria using thick blood smear. A dried blood spot on Whatman filter paper was also taken for polymerase chain reaction (PCR) and restriction fragment length polymorphism. Among 123 known patients with sickle cell disease, the prevalence of malaria by blood smear microscopy was 3.2% and by PCR was 13.8%. The prevalence of K76T mutation among the patients was 81.3%. The majority of the patients (72.4%) were using chloroquine prophylaxis. In conclusion, the prevalence of malaria parasitaemia among children with sickle cell disease attending BMC is low (3.2%) by microscopy but several children maintain sub patent infection detectable by PCR. The prevalence of chloroquine resistant P. falciparum in these children was higher than that previously seen in normal population in Tanzania. We recommend special attention to be paid to patients with sickle cell disease while studying the dynamics of drug resistant parasites. _________________________________________________________________________________

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Section: Discussionsupporting

confidence: 78%

High prevalence of Plasmodium falciparum pfcrt K76T mutation in children with sickle cell disease at a tertiary hospital in north-western Tanzania

Mongella

Enweji

Mnongone

et al. 2014

Tanzania J Hlth Res

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“…Conversely, if the risk of malaria-specific death was not elevated, alternative approaches to prophylaxis and treatment might be considered. Recently, we reported that malaria was a rare cause of morbidity in patients with confirmed SCA in both Dar-es-Salaam, Tanzania 13 and in the Kilifi District on the coast of Kenya. 11 Nevertheless, the diagnosis of SCA is often delayed until the disease becomes clinically manifest, and these studies could not have recorded malaria deaths in undiagnosed children.…”

Section: Introductionmentioning

confidence: 99%

“…8 However, studies suggesting that malaria is an important cause of death in children with SCA are balanced by others that show the opposite: children with SCA might even be less susceptible to malaria than those without the disease. 6,[9][10][11][12][13] Determining the true risk of death from malaria in subjects with SCA is important for several reasons. From a policy perspective, documenting an association between SCA and malaria death would provide strong justification for early-life SCA screening and the targeted prescription of effective malaria prophylaxis.…”

mentioning

confidence: 99%

High mortality from Plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya

McAuley

Webb

Makani

et al. 2010

Blood

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122

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Although malaria is widely considered a major cause of death in young children born with sickle cell anemia (SCA) in sub-Saharan Africa, this is poorly quantified. We attempted to investigate this question through 4 large case-control analyses involving 7164 children living on the coast of Kenya. SCA was associated with an increased risk of admission to hospital both with nonmalaria diseases in general (odds ratio [OR] ‫؍‬ 4.17; 95% confidence interval [CI], 1.95-8.92; P < .001) and with invasive bacterial diseases in particular (OR ‫؍‬ 8.73; 95% CI, 4.51-16.89; P < .001). We found no evidence for a strongly increased risk of either uncomplicated malaria (OR ‫؍‬ 0.43; 95% CI, 0.09-2.10; P ‫؍‬ .30) or malaria complicated by a range of well-described clinical features of severity (OR ‫؍‬ 0.80; 95% CI, 0.25-2.51; P ‫؍‬ .70) overall; nevertheless, mortality was considerably higher among SCA than non-SCA children hospitalized with malaria. Our findings highlight both the central role that malaria plays in the high early mortality seen in African children with SCA and the urgent need for better quantitative data. Meanwhile, our study confirms the importance of providing all children living with SCA in malaria-endemic areas with effective prophylaxis. (Blood. 2010;116(10):1663-1668) IntroductionThe common causes of morbidity and mortality in children living with sickle cell anemia (SCA) in developed countries have been well documented through projects, such as the Cooperative Study of Sickle Cell Disease in the United States and the Jamaican Cohort study. Nevertheless, surprisingly little research has been conducted in Africa, 1-3 where more than 230 000 children with SCA, approximately 80% of the global burden, are born every year. 4 Although malaria is widely considered a major cause of death in African children with SCA, 1,5-7 this assumption is supported by surprisingly few data. Most reports have involved small, hospital-based studies and have lacked control data that enable comparisons of risk to be made with non-SCA subjects. 1 Perhaps the strongest evidence comes from the Garki project, conducted in northern Nigeria during the 1970s, which reported a nonsignificant trend toward higher survival of children with SCA in an area exposed to intensive malaria control. 8 However, studies suggesting that malaria is an important cause of death in children with SCA are balanced by others that show the opposite: children with SCA might even be less susceptible to malaria than those without the disease. 6,[9][10][11][12][13] Determining the true risk of death from malaria in subjects with SCA is important for several reasons. From a policy perspective, documenting an association between SCA and malaria death would provide strong justification for early-life SCA screening and the targeted prescription of effective malaria prophylaxis. Conversely, if the risk of malaria-specific death was not elevated, alternative approaches to prophylaxis and treatment might be considered. Recently, we reported that malaria was a rare cause...

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“…However, nongenetic factors may explain much of the clinical variability. Most dramatically, the survival of children with sickle cell disease in highincome countries approaches that of unaffected children, 54 whereas in most of sub-Saharan Africa, up to 90% of children with sickle cell disease die, 55 even though these are genetically very similar populations. Nongenetic factors include climate and air quality, as well as socioeconomic factors, which are assessed, for example, on the basis of access to medical care, safe blood transfusions, and treatment of infections.…”

Section: Nongene Tic Modifier S Of Dise a Se Se V Er It Ymentioning

confidence: 99%

“…Studies in Kenya and Tanzania showed that the incidence of malaria was not increased among patients with sickle cell disease but that the risk of death was higher once malaria developed. 55,85 In high-income countries, infection also contributes significantly to morbidity and mortality among patients with sickle cell disease, particularly as a cause of death in children (Streptococcus pneumoniae) and as a cause of osteomyelitis (salmonella, Staphylococcus aureus, gram-negative bacilli, and Mycobacterium tuberculosis) 86 and the acute chest syndrome (chlamydia, mycoplasma, and viruses) in all patients, regardless of age. 23 Although the spectrum of infections may vary across environments, the effect is greatly modified by the availability of facilities for prophylaxis and treatment, including access to antibiotics and safe blood transfusion.…”

Section: Infectious Diseasesmentioning

confidence: 99%

Sickle Cell Disease

2017

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Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization

Cited by 144 publications

References 26 publications

High prevalence of Plasmodium falciparum pfcrt K76T mutation in children with sickle cell disease at a tertiary hospital in north-western Tanzania

High prevalence of Plasmodium falciparum pfcrt K76T mutation in children with sickle cell disease at a tertiary hospital in north-western Tanzania

High mortality from Plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya

Sickle Cell Disease

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