How benign is sickle cell trait?

Gibson, John S.; Rees, David C.

doi:10.1016/j.ebiom.2016.08.023

Cited by 27 publications

(25 citation statements)

References 9 publications

(11 reference statements)

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“…As such, we believe that the malaria protective effect of HbAS is strongly analogous to that which might be seen with malaria-specific interventions, including an effective malaria vaccine. While it has been shown that under specific circumstances, HbAS can result in adverse consequences 15,16 , it has never been associated with benefit against any other diseases in a non-malaria-endemic environment 16,17 . Specifically, while it has been shown that the risk of invasive bacterial infections is significantly reduced in HbAS children 10,18 , this has only been observed in areas endemic for malaria 10 .…”

Section: Discussionmentioning

confidence: 99%

The indirect health effects of malaria estimated from health advantages of the sickle cell trait

et al. 2019

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Most estimates of the burden of malaria are based on its direct impacts; however, its true burden is likely to be greater because of its wider effects on overall health. Here we estimate the indirect impact of malaria on children’s health in a case-control study, using the sickle cell trait (HbAS), a condition associated with a high degree of specific malaria resistance, as a proxy indicator for an effective intervention. We estimate the odds ratios for HbAS among cases (all children admitted to Kilifi County Hospital during 2000–2004) versus community controls. As expected, HbAS protects strongly against malaria admissions (aOR 0.26; 95%CI 0.22–0.31), but it also protects against other syndromes, including neonatal conditions (aOR 0.79; 0.67–0.93), bacteraemia (aOR 0.69; 0.54–0.88) and severe malnutrition (aOR 0.67; 0.55–0.83). The wider health impacts of malaria should be considered when estimating the potential added benefits of effective malaria interventions.

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Section: Discussionmentioning

confidence: 99%

The indirect health effects of malaria estimated from health advantages of the sickle cell trait

et al. 2019

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“…carrier status for the HbS variant) has been associated in some studies with a risk of sickling symptoms in particular circumstances, for example during extreme physical exertion and/or increased altitude [ 10 , 11 ]. While uncertainty remains as to the nature, severity, and extent of the risk associated with carrier status for sickle cell disease [ 12 ], particularly for the general population of carriers [ 13 – 17 ], it bears relevance to a broader debate concerning the disclosure to families of carrier results incidentally identified by NBS [ 18 , 19 ]. Specifically, while most NBS programs have opted for disclosure of all carrier status results to families, some have argued that this may impose undue burden, requiring families to be informed of incidentally generated and clinically ambiguous findings when they may neither need nor prefer to know [ 20 ].…”

Section: Introductionmentioning

confidence: 99%

Health services use by children identified as heterozygous hemoglobinopathy mutation carriers via newborn screening

et al. 2021

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Background Newborn screening (NBS) for sickle cell disease incidentally identifies heterozygous carriers of hemoglobinopathy mutations. In Ontario, Canada, these carrier results are not routinely disclosed, presenting an opportunity to investigate the potential health implications of carrier status. We aimed to compare rates of health services use among children identified as carriers of hemoglobinopathy mutations and those who received negative NBS results. Methods Eligible children underwent NBS in Ontario from October 2006 to March 2010 and were identified as carriers or as screen-negative controls, matched to carriers 5:1 based on neighbourhood and timing of birth. We used health care administrative data to determine frequencies of inpatient hospitalizations, emergency department (ED) visits, and physician encounters through March 2012, using multivariable negative binomial regression to compare rates of service use in the two cohorts. We analyzed data from 4987 carriers and 24,935 controls. Results Adjusted incidence rate ratios (95% CI) for service use in carriers versus controls among children < 1 year of age were: 1.11 (1.06–1.17) for ED visits; 0.97 (0.89–1.06) for inpatient hospitalization; and 1.02 (1.00–1.04) for physician encounters. Among children ≥1 year of age, adjusted rate ratios were: 1.03 (0.98–1.07) for ED visits; 1.14 (1.03–1.25) for inpatient hospitalization and 0.92 (0.90–0.94) for physician encounters. Conclusions While we identified statistically significant differences in health services use among carriers of hemoglobinopathy mutations relative to controls, effect sizes were small and directions of association inconsistent across age groups and health service types. Our findings are consistent with the assumption that carrier status is likely benign in early childhood.

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“…Before, it was considered as a benign condition with protection to malaria and no painful episodes. However, several complications have been described in the literature as case reports, including venous thromboembolism, renal involvement, and splenic infarcts [5]. There are less than 100 reported cases of splenic infarcts in SCT associated with high altitude exposition [6,7].…”

Section: Introductionmentioning

confidence: 99%

Splenic Infarct Secondary to High Altitude Exposure in Sickle Cell Trait Patients: A Case Series

González

Shapiro

Tafur

et al. 2020

Cureus

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The sickle cell trait is considered a benign entity that generally does not show clinical manifestations. However, some complications have been described under certain conditions, such as a decrease in oxygen level, dehydration, and strenuous physical efforts. Among them, splenic infarct is a rare complication that presents as left upper abdominal pain in a situation of stress such as high altitude exposure. We present two cases of splenic infarcts in patients with undiagnosed sickle cell trait who showed to our institution with severe abdominal pain after coming from high altitude cities.

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How benign is sickle cell trait?

Cited by 27 publications

References 9 publications

The indirect health effects of malaria estimated from health advantages of the sickle cell trait

The indirect health effects of malaria estimated from health advantages of the sickle cell trait

Health services use by children identified as heterozygous hemoglobinopathy mutation carriers via newborn screening

Splenic Infarct Secondary to High Altitude Exposure in Sickle Cell Trait Patients: A Case Series

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