ABSTRACT
The impact of hemoglobinopathies on healthcare in the United States, particularly sickle cell disease (SCD), has been significant. Enactment of the Sickle Cell Anemia Control Act in 1972 significantly increased the federal interest in the SCDs and other hemoglobinopathies. Only since May 1, 2006, have all states required and provided universal newborn screening for SCD despite a national recommendation to this effect in 1987. In this article, we review the history of screening for SCD and other hemoglobinopathies, along with federal and state activities that have contributed to improved health outcomes for patients with SCD, as well as current newborn screening practices. We also chronicle the federal activities that have helped to shape and to refine laboratory screening and diagnostic proficiency. Finally, we review molecular testing strategies that have evolved and outline their possible future impacts on disease detection and outcome improvement.
Subject(s)
Anemia, Sickle Cell/history , Neonatal Screening/history , Anemia, Sickle Cell/diagnosis , History, 20th Century , History, 21st Century , Humans , Infant, Newborn , Neonatal Screening/legislation & jurisprudence , Neonatal Screening/trends , Prenatal Diagnosis/history , Quality Assurance, Health Care , United StatesABSTRACT
OBJECTIVE: We evaluated the effect of oral and other hormonal contraceptive (HC) use on venous thromboembolism risk among African American women and investigated whether the association was modified by the sickle cell trait. STUDY DESIGN: We report the findings of a case-control study that included 60 African American women with an idiopathic, first episode of venous thromboembolism and 196 African American controls. RESULTS: The odds of current HC use compared with noncurrent use contrasting cases and controls is 3.8 (95% confidence interval [CI], 1.7-8.1; P < .001). Among subjects with sickle cell trait, the odds ratio is higher (odds ratio [OR], 6.7; 95% CI, 1.0-43) than the odds ratio among subjects without sickle cell trait (OR, 2.6; 95% CI, 1.1-6.2), but the difference is not statistically significant. CONCLUSION: This study provides persuasive evidence that hormonal contraceptive use increases venous thromboembolism risk among African American women and that the increase in risk may be larger among women with sickle cell trait.
Subject(s)
Black or African American , Contraceptives, Oral, Hormonal/adverse effects , Sickle Cell Trait/complications , Venous Thromboembolism/chemically induced , Adolescent , Adult , Case-Control Studies , Contraceptive Agents, Female/adverse effects , Female , Humans , Middle Aged , Risk Factors , Young AdultABSTRACT
People with sickle cell disease have a chronically activated coagulation system and display hemostatic perturbations, but it is unknown whether they experience an increased risk of venous thromboembolism. We conducted a case-control study of venous thromboembolism that included 515 hospitalized black patients and 555 black controls obtained from medical clinics. All subjects were assayed for hemoglobin S and hemoglobin C genotypes. The prevalence of the S allele was 0.070 and 0.032 for case patients and controls, respectively (P < .001). The odds that a patient had sickle cell trait were approximately twice that of a control, indicating that the risk of venous thromboembolism is increased approximately 2-fold among blacks with sickle cell trait compared with those with the wild-type genotype (odds ratio = 1.8 with 95% confidence interval, 1.2-2.9). The odds ratio for pulmonary embolism and sickle cell trait was higher, 3.9 (2.2-6.9). The prevalence of sickle cell disease was also increased among case patients compared with controls. We conclude that sickle cell trait is a risk factor for venous thromboembolism and that the proportion of venous thromboembolism among blacks attributable to the mutation is approximately 7%.
Subject(s)
Black or African American , Sickle Cell Trait/complications , Thromboembolism/etiology , Venous Thrombosis/etiology , Adult , Aged , Blood Coagulation , Genotype , Hemoglobin C/analysis , Hemoglobin C/genetics , Hemoglobin, Sickle/analysis , Hemoglobin, Sickle/genetics , Humans , Male , Middle Aged , Mutation , Prevalence , Risk Factors , Sickle Cell Trait/blood , Sickle Cell Trait/genetics , Thromboembolism/blood , Thromboembolism/genetics , Venous Thrombosis/blood , Venous Thrombosis/geneticsABSTRACT
BACKGROUND: This study examined children's experiences following a motor vehicle accident (MVA). METHODS: Approximately 9 months following the accident, children (n=50) and their parents (n=50) participated in extensive interviews about the accident and in comprehensive, structured diagnostic interviews concerning overall psychological functioning. Additional assessments included post-traumatic stress questionnaires, archival police report records, and emergency treatment medical records. RESULTS: Of the 50 children, 7 children (14%) met criteria for PTSD diagnosis, and an additional 5 children met criteria for specific phobia (10%) related to the automobile accident on the structured diagnostic interview (DICA-R-C; total of 24%). Degree of physical injury predicted more PTSD symptoms, and previous accident experiences predicted fewer symptoms, before and after controlling for other variables. Holding degree of physical injury and age constant revealed that social support predicted fewer PTSD symptoms. CONCLUSIONS: Findings suggest the possible inoculating role of previous accidents and the importance of social support following MVA injury.
