Rakhi P Naik1, Kim Smith-Whitley2, Kathryn L Hassell3, Nkeiruka I Umeh4, Mariane de Montalembert5, Puneet Sahota2, Carlton Haywood6, Jean Jenkins4, Michele A Lloyd-Puryear7, Clinton H Joiner8, Vence L Bonham4, Gregory J Kato9. 1. Johns Hopkins University, Baltimore, Maryland (R.P.N.). 2. Children's Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania (K.S., P.S.). 3. University of Colorado Denver School of Medicine, Denver, Colorado (K.L.H.). 4. National Human Genome Research Institute of the National Institutes of Health, Bethesda, Maryland (N.I.U., J.J., V.L.B.). 5. Hôpital Universitaire Necker-Enfants Malades, Paris, France (M.D.). 6. Johns Hopkins Berman Institute of Bioethics, Baltimore, Maryland (C.H.). 7. American College of Medical Genetics and Genomics, Bethesda, Maryland (M.A.L.). 8. University of Pennsylvania, Philadelphia, Pennsylvania; Johns Hopkins Berman Institute of Bioethics, Baltimore, Maryland; Emory University School of Medicine, Atlanta, Georgia (C.H.J.). 9. Vascular Medicine Institute at the University of Pittsburgh, Pittsburgh, Pennsylvania (G.J.K.).
Abstract
Background: Although sickle cell trait (SCT) is largely a benign carrier state, it may increase risk for certain clinical outcomes. Purpose: To evaluate associations between SCT and clinical outcomes in children and adults. Data Sources: English-language searches of PubMed, CINAHL, the Cochrane Library, Current Contents Connect, Scopus, and Embase (1 January 1970 to 30 June 2018) and bibliographies of review articles. Study Selection: Observational controlled studies (published in English) in children or adults that examined an association between SCT and any of 24 clinical outcomes specified a priori in the following 6 categories: exertion-related injury; renal, vascular, pediatric, and surgery- or trauma-related outcomes; and overall mortality. Data Extraction: A single reviewer extracted study data, which was checked by another; 2 reviewers independently assessed study quality; and strength of evidence was assessed by consensus. Data Synthesis: Of 7083 screened studies, 41 met inclusion criteria. High-strength evidence supported a positive association between SCT and risk for pulmonary embolism, proteinuria, and chronic kidney disease. Moderate-strength evidence supported a positive association between SCT and exertional rhabdomyolysis and a null association between SCT and deep venous thrombosis, heart failure or cardiomyopathy, stroke, and pediatric height or weight. Absolute risks for thromboembolism and rhabdomyolysis were small. For the remaining 15 clinical outcomes, data were insufficient or strength of evidence was low. Limitation: Publication bias was possible, and high-quality evidence was scant. Conclusion: Sickle cell trait is a risk factor for a few adverse health outcomes, such as pulmonary embolism, kidney disease, and exertional rhabdomyolysis, but does not seem to be associated with such complications as heart failure and stroke. Insufficient data or low-strength evidence exists for most speculated complications of SCT. Primary Funding Source: National Human Genome Research Institute.
Background: Although sickle cell trait (SCT) is largely a benign carrier state, it may increase risk for certain clinical outcomes. Purpose: To evaluate associations between SCT and clinical outcomes in children and adults. Data Sources: English-language searches of PubMed, CINAHL, the Cochrane Library, Current Contents Connect, Scopus, and Embase (1 January 1970 to 30 June 2018) and bibliographies of review articles. Study Selection: Observational controlled studies (published in English) in children or adults that examined an association between SCT and any of 24 clinical outcomes specified a priori in the following 6 categories: exertion-related injury; renal, vascular, pediatric, and surgery- or trauma-related outcomes; and overall mortality. Data Extraction: A single reviewer extracted study data, which was checked by another; 2 reviewers independently assessed study quality; and strength of evidence was assessed by consensus. Data Synthesis: Of 7083 screened studies, 41 met inclusion criteria. High-strength evidence supported a positive association between SCT and risk for pulmonary embolism, proteinuria, and chronic kidney disease. Moderate-strength evidence supported a positive association between SCT and exertional rhabdomyolysis and a null association between SCT and deep venous thrombosis, heart failure or cardiomyopathy, stroke, and pediatric height or weight. Absolute risks for thromboembolism and rhabdomyolysis were small. For the remaining 15 clinical outcomes, data were insufficient or strength of evidence was low. Limitation: Publication bias was possible, and high-quality evidence was scant. Conclusion: Sickle cell trait is a risk factor for a few adverse health outcomes, such as pulmonary embolism, kidney disease, and exertional rhabdomyolysis, but does not seem to be associated with such complications as heart failure and stroke. Insufficient data or low-strength evidence exists for most speculated complications of SCT. Primary Funding Source: National Human Genome Research Institute.
Authors: Patricia A Deuster; Carmen L Contreras-Sesvold; Francis G O'Connor; William W Campbell; Kimbra Kenney; John F Capacchione; Mark E Landau; Sheila M Muldoon; Elisabeth J Rushing; Yuval Heled Journal: Eur J Appl Physiol Date: 2013-03-31 Impact factor: 3.078
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Authors: Camille Faes; Anton Ilich; Amandine Sotiaux; Erica M Sparkenbaugh; Michael W Henderson; Laura Buczek; Joan D Beckman; Patrick Ellsworth; Denis F Noubouossie; Lantarima Bhoopat; Mark Piegore; Céline Renoux; Wolfgang Bergmeier; Yara Park; Kenneth I Ataga; Brian Cooley; Alisa S Wolberg; Nigel S Key; Rafal Pawlinski Journal: Blood Date: 2019-04-05 Impact factor: 22.113