Complications Associated with Sickle Cell Trait: A Brief Narrative Review

Tsaras, Geoffrey; Owusu-Ansah, Amma; Boateng, Freda; Amoateng-Adjepong, Yaw

doi:10.1016/j.amjmed.2008.12.020

Cited by 314 publications

(311 citation statements)

References 37 publications

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“…First, heterozygotes AS do not appear to have symptoms of anemia or other effects (costs) of carrying the S allele in nonmalarial environments (Sheng et al, 2010), although some negative effects for AS individuals during strenuous exercise have been suggested (Connes et al, 2008;Tsaras et al, 2009). As a result, the National Collegiate Athletic Association in the USA has instituted mandatory testing for sickle-cell carrier status for student athletes in its Division 1 sports (Bonham et al, 2010).…”

Section: Sickle Cell S or B S Or Hbsmentioning

confidence: 99%

Population genetics of malaria resistance in humans

2011

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Section: Sickle Cell S or B S Or Hbsmentioning

confidence: 99%

Population genetics of malaria resistance in humans

2011

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“…2 The argument for postponing testing becomes problematic when there are possible health consequences of a carrier status, 2 for example, in the case of sickle cell trait (SCT), which confers rare possible clinical associations including haematuria, hyposthenuria, and exertional rhabdomyolysis due to hypoxia. 5 However, clinical complications are contentious 6,7 and there is little evidence that the implications require early SCT identification. If professionals do not regard SCT as medically significant, 6 a situation could arise whereby parents are informed that their newborn might experience health difficulties associated with SCT, 8 while concurrently being informed that older children do not require SCT testing on the basis it has no clear medical benefit.…”

Section: Introductionmentioning

confidence: 99%

“…12 If children are not deemed competent, decisions can be made on their behalf. Although collaborative decision making is encouraged, discerning children's best interests may be difficult for parents and professionals if they have competing views from the outset [12][13][14] and the clinical 5 and psychosocial implications 15 of childhood carrier identification are unclear. When professionals and parents have differing opinions about testing, there is no agreement about who has the power to make decisions.…”

Section: Introductionmentioning

confidence: 99%

A qualitative study to explore how professionals in the United Kingdom make decisions to test children for a sickle cell carrier status

et al. 2015

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European guidelines recommend that, unless there are clear benefits of autosomal recessive carrier testing in childhood, it should be deferred to protect children's autonomous decision making. Although it is believed that children receive testing in the United Kingdom, it is unclear how or why professionals make decisions to provide tests. Semi-structured interviews were conducted with 25 professionals in the United Kingdom who advise about, and undertake, childhood sickle cell trait testing. Data were analysed using thematic analysis. Few professionals were aware of, or used, guidelines to inform testing decisions and instead, considered the reproductive and clinical relevance of testing, and autonomous rights of parents. Many professionals believed testing was important and readily offered it to parents. Professionals who discouraged testing were met with parental resistance and often provided testing when conflict was difficult to manage. Children were rarely considered to be capable of making decisions and few were engaged in discussions. When consulted, older children demonstrated interest, but younger children usually declined testing. Wide variation in testing advice emerged because of opposing beliefs about children's best interests and potential benefits or harms of testing. An explanation of how children's best interests should be determined in light of conflicting evidence regarding the psychosocial and clinical implications of carrier status is needed. Improved awareness of guidelines might encourage professionals to support the role of children in testing decisions. Strategies are also required to help professionals determine children's cognitive capacity and to protect children's future autonomy during discussions with persistent parents.

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“…[3][4][5][6][7] With these aberrations, one might expect HbAS to be common in patients with renal disease. Although not conclusive, some epidemiologic data support this contention in CKD patients, particularly patients requiring maintenance dialysis.…”

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confidence: 99%

Sickle Trait in African-American Hemodialysis Patients and Higher Erythropoiesis-Stimulating Agent Dose

Derebail¹,

Lacson

Kshirsagar³

et al. 2014

Journal of the American Society of Nephrology

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African Americans require higher doses of erythropoiesis-stimulating agents (ESAs) during dialysis to manage anemia, but the influence of sickle cell trait and other hemoglobinopathy traits on anemia in dialysis patients has not been adequately evaluated. We performed a cross-sectional study of a large cohort of adult African-American hemodialysis patients in the United States to determine the prevalence of hemoglobinopathy traits and quantify their influence on ESA dosing. Laboratory and clinical data were obtained over 6 months in 2011. Among 5319 African-American patients, 542 (10.2%) patients had sickle cell trait, and 129 (2.4%) patients had hemoglobin C trait; no other hemoglobinopathy traits were present. Sickle cell trait was more common in this cohort than the general African-American population (10.2% versus 6.5%-8.7%, respectively, P,0.05). Among 5002 patients (10.3% sickle cell trait and 2.4% hemoglobin C trait) receiving ESAs, demographic and clinical variables were similar across groups, with achieved hemoglobin levels being nearly identical. Patients with hemoglobinopathy traits received higher median doses of ESA than patients with normal hemoglobin (4737.4 versus 4364.1 units/treatment, respectively, P=0.02). In multivariable analyses, hemoglobinopathy traits associated with 13.2% more ESA per treatment (P=0.001). Within subgroups, sickle cell trait patients received 13.2% (P=0.003) higher dose and hemoglobin C trait patients exhibited a similar difference (12.9%, P=0.12). Sensitivity analyses using weight-based dosing definitions and separate logistic regression models showed comparable associations. Our findings suggest that the presence of sickle cell trait and hemoglobin C trait may explain, at least in part, prior observations of greater ESA doses administered to African-American dialysis patients relative to Caucasian patients.

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Complications Associated with Sickle Cell Trait: A Brief Narrative Review

Cited by 314 publications

References 37 publications

Population genetics of malaria resistance in humans

Population genetics of malaria resistance in humans

A qualitative study to explore how professionals in the United Kingdom make decisions to test children for a sickle cell carrier status

Sickle Trait in African-American Hemodialysis Patients and Higher Erythropoiesis-Stimulating Agent Dose

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