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      Clinical Outcomes Associated With Sickle Cell Trait : A Systematic Review

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          Abstract

          Although sickle cell trait (SCT) is largely a benign carrier state, it may increase risk for certain clinical outcomes.

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          Most cited references45

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          AHRQ series paper 5: grading the strength of a body of evidence when comparing medical interventions--agency for healthcare research and quality and the effective health-care program.

          To establish guidance on grading strength of evidence for the Evidence-based Practice Center (EPC) program of the U.S. Agency for Healthcare Research and Quality. Authors reviewed authoritative systems for grading strength of evidence, identified domains and methods that should be considered when grading bodies of evidence in systematic reviews, considered public comments on an earlier draft, and discussed the approach with representatives of the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) working group. The EPC approach is conceptually similar to the GRADE system of evidence rating; it requires assessment of four domains: risk of bias, consistency, directness, and precision. Additional domains to be used when appropriate include dose-response association, presence of confounders that would diminish an observed effect, strength of association, and publication bias. Strength of evidence receives a single grade: high, moderate, low, or insufficient. We give definitions, examples, mechanisms for scoring domains, and an approach for assigning strength of evidence. EPCs should grade strength of evidence separately for each major outcome and, for comparative effectiveness reviews, all major comparisons. We will collaborate with the GRADE group to address ongoing challenges in assessing the strength of evidence.
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            Complications associated with sickle cell trait: a brief narrative review.

            Sickle cell trait occurs in approximately 300 million people worldwide, with the highest prevalence of approximately 30% to 40% in sub-Saharan Africa. Long considered a benign carrier state with relative protection against severe malaria, sickle cell trait occasionally can be associated with significant morbidity and mortality. Sickle cell trait is exclusively associated with rare but often fatal renal medullary cancer. Current cumulative evidence is convincing for associations with hematuria, renal papillary necrosis, hyposthenuria, splenic infarction, exertional rhabdomyolysis, and exercise-related sudden death. Sickle cell trait is probably associated with complicated hyphema, venous thromboembolic events, fetal loss, neonatal deaths, and preeclampsia, and possibly associated with acute chest syndrome, asymptomatic bacteriuria, and anemia in pregnancy. There is insufficient evidence to suggest an independent association with retinopathy, cholelithiasis, priapism, leg ulcers, liver necrosis, avascular necrosis of the femoral head, and stroke. Despite these associations, the average life span of individuals with sickle cell trait is similar to that of the general population. Nonetheless, given the large number of people with sickle cell trait, it is important that physicians be aware of these associations.
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              Development of the RTI item bank on risk of bias and precision of observational studies.

              To create a practical and validated item bank for evaluating the risk of bias and precision of observational studies of interventions or exposures included in systematic evidence reviews. The item bank, developed at RTI International, was created based on 1,492 questions included in earlier instruments, organized by the quality domains identified by Deeks et al. Items were eliminated and refined through face validity, cognitive, content validity, and interrater reliability testing. The resulting item bank consisting of 29 questions for evaluating the risk of bias and precision of observational studies of interventions or exposures (1) captures all of the domains critical for evaluating this type of research, (2) is comprehensive and can be easily lifted "off the shelf" by different researchers, (3) can be adapted to different topic areas and study types (e.g., cohort, case-control, cross-sectional, and case series studies), and (4) provides sufficient instruction to apply the tool to varied topics. One bank of items, with specific instructions for focusing abstractor evaluations, can be created to judge the risk of bias and precision of the variety of observational studies that may be used in systematic and comparative effectiveness reviews. Copyright © 2012 Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Annals of Internal Medicine
                Ann Intern Med
                American College of Physicians
                0003-4819
                November 06 2018
                October 30 2018
                November 06 2018
                : 169
                : 9
                : 619
                Affiliations
                [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.)
                Article
                10.7326/M18-1161
                6487193
                30383109
                0d0549ee-f595-47e6-ba4a-fd5e57f25b32
                © 2018
                History

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