Sickle-Cell Trait: Novel Clinical Significance

Key, Nigel S.; Derebail, Vimal K.

doi:10.1182/asheducation-2010.1.418

Cited by 102 publications

(106 citation statements)

References 39 publications

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“…3 However, many Hispanic individuals have not been screened as part of this program (eg, those born outside the United States). Two facts may reduce the frequency of SCT testing in this group.…”

Section: Acknowledgments and Compliance With Ethical Standardsmentioning

confidence: 99%

“…2 Carriers of a single altered copy of HBB have sickle cell trait (SCT), which has generally been viewed as a benign condition with symptoms only presenting under extreme circumstances, such as high altitudes combined with exercise. 3 SCT is common with an estimated 300 million carriers worldwide. 3 In the United States, it is estimated that SCT occurs in approximately 73 out of every 1,000 births in Blacks and about 7 out of every 1,000 births in Hispanics.…”

Section: Introductionmentioning

confidence: 99%

“…3 SCT is common with an estimated 300 million carriers worldwide. 3 In the United States, it is estimated that SCT occurs in approximately 73 out of every 1,000 births in Blacks and about 7 out of every 1,000 births in Hispanics. 4 While it is well-established that chronic kidney disease (CKD) and decreased glomerular filtration rate are common in patients with SCA, 2 results from a re- Sickle cell anemia (SCA) is a common hematological disorder among individuals of African descent in the United States; the disorder results in the production of abnormal hemoglobin.…”

Section: Introductionmentioning

confidence: 99%

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Sickle Cell Trait and Renal Function in Hispanics in the United States: the Northern Manhattan Study

et al. 2017

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Sickle cell anemia (SCA) is a common hematological disorder among individuals of African descent in the United States; the disorder results in the production of abnormal hemoglobin. It is caused by homozygosity for a genetic mutation in HBB; rs334. While the presence of a single mutation (sickle cell trait, SCT) has long been considered a benign trait, recent research suggests that SCT is associated with renal dysfunction, including a decrease in estimated glomerular filtration rate (eGFR) and increased risk of chronic kidney disease (CKD) in African Americans. It is currently unknown whether similar associations are observed in Hispanics. Therefore, our study aimed to determine if SCT is associated with mean eGFR and CKD in a sample of 340 Dominican Hispanics from the Northern Manhattan Study. Using regression analyses, we tested rs334 for association with eGFR and CKD, adjusting for age and sex. eGFR was estimated using the Chronic Kidney Disease Epidemiology Collaboration equation and CKD was defined as eGFR < 60 mL/min/1.73 m2. Within our sample, there were 16 individuals with SCT (SCT carriers). We found that SCT carriers had a mean eGFR that was 12.12 mL/min/1.73m2 lower than non-carriers (P=.002). Additionally, SCT carriers had 2.72 times higher odds of CKD compared with non-carriers (P=.09). Taken together, these novel results show that Hispanics with SCT, as found among African Americans with SCT, may also be at increased risk for kidney disease.Ethn Dis. 2017; 27(1):11-14; doi:10.18865/ed.27.1.11.

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Section: Acknowledgments and Compliance With Ethical Standardsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Sickle Cell Trait and Renal Function in Hispanics in the United States: the Northern Manhattan Study

et al. 2017

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“…Sickle-cell trait (SCT) is the term used to describe the presence, in an estimated 300 million individuals worldwide, of a heterozygous glutamic acid-to-valine substitution in the β-globin gene on chromosome 11 [1]. Individuals with the SCT have a heterozygous genotype (HbAS) compared to the homozygous genotype (HbSS) carried by individuals with sickle cell disease (SCD).…”

Section: Introductionmentioning

confidence: 99%

Severely Damaged Hip Joints from Avascular Necrosis in a patient with Sickle Cell Trait: Case Report

Efie¹,

Joseph²,

Yeika³

et al. 2017

J Blood Disord Transfus

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Background: Avascular necrosis of the femoral head is an increasingly recognized entity especially in the context of sickle cell disease. This entity is however less frequently seen in sickle cell trait; consequently, fewer cases of hip damage secondary to avascular necrosis in persons with sickle cell trait have been reported. This case shows how avascular necrosis can occur, affecting both hip joints in patients with sickle cell trait.

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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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Sickle-Cell Trait: Novel Clinical Significance

Cited by 102 publications

References 39 publications

Sickle Cell Trait and Renal Function in Hispanics in the United States: the Northern Manhattan Study

Sickle Cell Trait and Renal Function in Hispanics in the United States: the Northern Manhattan Study

Severely Damaged Hip Joints from Avascular Necrosis in a patient with Sickle Cell Trait: Case Report

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

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