Associated Morbidities in Children with Sickle-Cell Anaemia Presenting with Severe Anaemia in a Malarious Area

Ambe, J P; Fatunde, J O; Sodeinde, Olugbemiro

doi:10.1177/004947550103100109

Cited by 31 publications

(29 citation statements)

References 7 publications

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“…2 Although malaria is widely viewed as a major problem in African children with SCA, 1,22,24 the supportive evidence is inconclusive. There is no doubt that such subjects can develop severe and fatal malaria [24][25][26][27][28][29] ; however, their risk relative to those without the disease remains unknown. Most existing studies have been small and uncontrolled and have focused on children whose SCA has already been confirmed, and the many biases of this approach make the results of such studies difficult to interpret.…”

Section: Discussionmentioning

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

122

109

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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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Section: Discussionmentioning

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

122

109

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“…Malaria is a serious infection for children with SCD in areas of the world where it is endemic (Ambe et al 2001 ). While children with sickle cell trait have a lower risk of complications and death from malaria (Aidoo et al 2002 ;Williams et al 2005 ), individuals with SCD do not appear to enjoy this same protection.…”

Section: Infectionmentioning

confidence: 99%

Clinical Manifestations of Sickle Cell Anemia: Infants and Children

Nickel

Hsu

2016

Sickle Cell Anemia

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Children with sickle cell disease (SCD) have varied clinical problems. The hallmark manifestation of SCD, the pain crisis, typically fi rst occurs in early childhood presenting as dactylitis. Pain increases in frequency and severity as children age, especially during adolescence. Due to functional asplenia, children with SCD are at signifi cantly increased risk for certain infections, most notably Streptococcus pneumoniae. Other infections like parvovirus B19 are also special threats and can trigger an aplastic crisis. Unique, potentially life-threatening acute complications like splenic sequestration and acute chest syndrome occur in these children. They are at risk for neurologic disease, the most serious being stroke. In addition, the chronic hemolysis of SCD causes gallstones, which can lead to biliary tract disease. Children with SCD also frequently face chronic issues that include nocturnal enuresis and decreased growth. Despite these many potential problems, with advances in care including antibiotic treatment, stroke screening, blood transfusions, and hydroxyurea therapy, children with SCD rarely die in childhood and can become productive adults.

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“…Severe anaemia (defined as haemoglobin less than 5 g/dL) is a common complication of childhood malaria; it is also seen in association with protein and micronutrient deficiencies notably iron and folic acid, helminthic infections and sickle cell disease. [1][2][3][4] These patients with severe anaemia are usually admitted as emergencies and blood transfusion is invariably given as a life-saving measure in spite of the welldocumented risks. 5 This retrospective study was carried out to determine (a) the pattern of presentation of these patients and (b) the outcome of management.…”

Section: Introductionmentioning

confidence: 99%

Blood transfusion in an emergency paediatric unit in Owerri, Nigeria: pattern and outcome

Nwolisa

Owoeye

et al. 2006

Trop Doct

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Associated Morbidities in Children with Sickle-Cell Anaemia Presenting with Severe Anaemia in a Malarious Area

Cited by 31 publications

References 7 publications

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

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

Clinical Manifestations of Sickle Cell Anemia: Infants and Children

Blood transfusion in an emergency paediatric unit in Owerri, Nigeria: pattern and outcome

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