Death Due to Microvascular Occlusion in Sickle-Cell Trait Following Physical Exertion

Wirthwein, Darren P.; Spotswood, Sheila D.; Barnard, Jeffrey J.; Prahlow, Joseph A.

doi:10.1520/jfs14982j

Cited by 66 publications

(69 citation statements)

References 8 publications

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“…22 Several studies have also sought to determine the pathophysiologic basis for the exertion-related sudden death described among individuals with SCT in case reports. [23][24][25][26] In a recent population study of black soldiers on active duty in the US Army, SCT was associated with an adjusted risk for exertional rhabdomyolysis that was 54% higher than that associated with absence of SCT, although all-cause mortality was not affected by SCT status.…”

Section: Discussionmentioning

confidence: 99%

“…Also, the effect of SCT on the development of other cardiovascular risk factors, as well as how this relationship is influenced by fitness, has not been previously examined despite growing evidence for an increased risk for chronic kidney disease in SCT carriers. The Coronary Artery Risk Development in Young Adults (CARDIA) study and its genetic repository provide a unique opportunity to study fitness and cardiovascular risk factors in a large cohort of young African Americans who are followed for up to 25 years. Thus, we tested the hypothesis that SCT carrier status was associated with lower fitness at baseline and worsening fitness over follow-up.…”

Section: Introductionmentioning

confidence: 99%

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Association among sickle cell trait, fitness, and cardiovascular risk factors in CARDIA

Liem

Chan

et al. 2017

Blood

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Key Points• SCT status is not significantly associated with longitudinal changes in fitness among African Americans.• SCT status is not an independent risk factor for hypertension, diabetes, or metabolic syndrome among African Americans.The contribution of sickle cell trait (SCT) to racial disparities in cardiopulmonary fitness is not known, despite concerns that SCT is associated with exertion-related sudden death. We evaluated the association of SCT status with cross-sectional and longitudinal changes in fitness and risk for hypertension, diabetes, and metabolic syndrome over the course of 25 years among 1995 African Americans (56% women, 18-30 years old) in the Coronary Artery Risk Development in Young Adults (CARDIA) study. Overall, the prevalence of SCT was 6.8% (136/1995) in CARDIA, and over the course of 25 years, 46% (738/1590), 18% (288/1631), and 40% (645/1,611) of all participants developed hypertension, diabetes, and metabolic syndrome, respectively. Compared with participants without SCT, participants with SCT had similar baseline measures of fitness in cross-section, including exercise duration (535 vs 540 seconds; P 5 .62), estimated metabolic equivalent of tasks (METs; 11.6 vs 11.7; P 5 .80), maximum heart rate (174 vs 175 beats/min; P 5 .41), and heart rate at 2 minutes recovery (44 vs 43 beats/min; P 5 .28). In our secondary analysis, there was neither an association of SCT status with longitudinal changes in fitness nor an association with development of hypertension, diabetes, or metabolic syndrome after adjustment for sex, baseline age, body mass index, fitness, and physical activity. SCT is not associated with reduced fitness in this longitudinal study of young African American adults, suggesting the increased risk for exertion-related sudden death in SCT carriers is unlikely related to fitness. SCT status also is not an independent risk factor for developing hypertension, diabetes, or metabolic syndrome. (Blood. 2017;129(6):723-728) IntroductionSickle cell trait (SCT) is caused by the inheritance of a single copy of the variant b globin gene, which results in production of abnormal sickle hemoglobin. The coinheritance of 2 copies of the abnormal gene results in sickle cell anemia (SCA), a clinically significant blood disorder seen primarily among individuals of African or Afro-Caribbean descent. Historically, SCT, which is observed in 8% of the African American population in the United States, has been considered a clinically benign condition. Recent studies, however, suggest SCT may confer a higher risk for chronic kidney disease, thrombosis, stroke, and pregnancy-related complications in its carriers.1-6 Importantly, there is growing concern SCT may be associated with sudden death during strenuous physical activity and exercise, leading to the National College Athletics Association to mandate carrier status testing among all student athletes in division I institutions in 2010, and later in all division II and III institutions. [7][8][9] Growing evidence suggests SCT carriers may exh...

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Association among sickle cell trait, fitness, and cardiovascular risk factors in CARDIA

Liem

Chan

et al. 2017

Blood

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“…In contrast to the homozygous form of sickle cell disease (SS haemoglobinopathy), in the heterozygous form or sickle cell trait, only one of the beta globin genes is affected (AS haemoglobinopathy) [9]. As people with sickle cell trait have no sickle cells under normal conditions and therefore show no increased hospitalisation or mortality rates compared to people without the trait, it is not widely recognised as a cause of life-threatening illness or death [15]. However, sudden deaths due to sickle cell crises of individuals with sickle cell trait have been described repeatedly [3,4,6,7,8,10,11,12,13,15].…”

Section: Introductionmentioning

confidence: 99%

“…However, sudden deaths due to sickle cell crises of individuals with sickle cell trait have been described repeatedly [3,4,6,7,8,10,11,12,13,15]. Physical activity with exertional rhabdomyolysis as well as pathological processes such as heat stress, hypoxic stress, viral illness and poor physical condition that may cause hypoxia, acidosis, dehydration, hyperosmolality, hypothermia or elevated erythrocyte 2,3-diphosphoglycerate (DPG) levels, all of which may transform the silent sickle cell trait into a syndrome resembling sickle cell disease and therefore may contribute to development of a sickle cell crisis [10,13,15].…”

Section: Introductionmentioning

confidence: 99%

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Sudden death due to a haemoglobin variant

Bock

Seidl

Hausmann

et al. 2004

International Journal of Legal Medicine

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A previous healthy 35-year-old man was found dead in his truck. Shortly before death he merely complained of influenza-like symptoms. The histological examination revealed evidence of a massive accumulation of sickle cells in smaller blood vessels. After molecular genetic analysis, the preliminary diagnosis of "sickle cell disease" was finally changed to the diagnosis of a sickle cell trait. It is presumed that an epileptic attack which also has to be considered as a concurring cause of death, precipitated sickling of the erythrocytes and led to a fatal sickle cell crisis.

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Sickle Cell Trait Is a Risk Factor for Early Stroke

Golomb

2005

Arch Neurol

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Death Due to Microvascular Occlusion in Sickle-Cell Trait Following Physical Exertion

Cited by 66 publications

References 8 publications

Association among sickle cell trait, fitness, and cardiovascular risk factors in CARDIA

Association among sickle cell trait, fitness, and cardiovascular risk factors in CARDIA

Sudden death due to a haemoglobin variant

Sickle Cell Trait Is a Risk Factor for Early Stroke

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