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Sickle cell trait (SCT) increases the risk for pulmonary embolism, kidney disease, and exertional rhabdomyolysis. However, SCT is not associated with heart failure or stroke, and evidence for other complications is limited.

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Area of Science:

  • Hematology
  • Genetics
  • Public Health

Background:

  • Sickle cell trait (SCT) is generally considered a benign carrier state.
  • Emerging evidence suggests SCT may be associated with specific clinical outcomes.
  • Understanding these associations is crucial for risk assessment and management.

Purpose of the Study:

  • To systematically evaluate the association between sickle cell trait (SCT) and various clinical outcomes in both children and adults.
  • To synthesize evidence regarding SCT's role in exertion-related injuries, renal, vascular, pediatric, and surgical/trauma outcomes, and mortality.

Main Methods:

  • Comprehensive literature search of multiple databases (PubMed, CINAHL, Cochrane, etc.) from 1970 to 2018.
  • Inclusion of observational controlled studies in English examining SCT and 24 predefined clinical outcomes.
  • Data extraction, quality assessment, and strength of evidence evaluation performed by independent reviewers.

Main Results:

  • High-strength evidence links SCT to increased risk of pulmonary embolism, proteinuria, and chronic kidney disease.
  • Moderate-strength evidence supports an association between SCT and exertional rhabdomyolysis.
  • No significant association found between SCT and deep venous thrombosis, heart failure, stroke, or pediatric growth issues; absolute risks are small.

Conclusions:

  • Sickle cell trait is a confirmed risk factor for specific adverse outcomes including pulmonary embolism, kidney disease, and exertional rhabdomyolysis.
  • Evidence does not support an association between SCT and complications like heart failure or stroke.
  • Further research is needed for many suspected SCT-related complications due to insufficient or low-strength evidence.