PDF(Pubmed)
Abstract：
UNASSIGNED: Sickle cell disease, a common genetic disorder in African Americans, manifests an increased risk of sudden death, the basis of which is incompletely understood. Prolongation of heart rate-corrected QT (QTc) interval on the electrocardiogram, a standard clinical measure of cardiac repolarization, may contribute to sudden death by predisposing to torsades de pointes ventricular tachycardia.
UNASSIGNED: We established a cohort study of 293 adult and 121 pediatric sickle cell disease patients drawn from the same geographic region as the Jackson Heart Study (JHS) cohort, in which significant correlates of QT duration have been characterized and quantitatively modeled. Herein, we establish clinical and laboratory correlates of QTc duration in our cohort using stepwise multivariate linear regression analysis. We then compared our adult sickle cell disease data to effect-size predictions from the published JHS statistical model of QT interval duration.
UNASSIGNED: In adult sickle cell disease, gender, diuretic use, QRS duration, serum ALT levels, anion gap, and diastolic blood pressure show positive correlation; hemoglobin levels show inverse correlation; in pediatric sickle cell disease, age, hemoglobin levels, and serum bicarbonate and creatinine levels show inverse correlation. The mean QTc in our adult sickle cell disease cohort is 7.8 milliseconds longer than in the JHS cohort, even though the JHS statistical model predicts that the mean QTc in our cohort should be > 11 milliseconds shorter than in the much older JHS cohort, a differential of > 18 milliseconds.
UNASSIGNED: Sickle cell disease patients have substantial QTc prolongation relative to their age, driven by factors some overlapping, in adult and pediatric sickle cell disease, and distinct from those that have been defined in the general African American community.
UNASSIGNED: We established a cohort study of 293 adult and 121 pediatric sickle cell disease patients drawn from the same geographic region as the Jackson Heart Study (JHS) cohort, in which significant correlates of QT duration have been characterized and quantitatively modeled. Herein, we establish clinical and laboratory correlates of QTc duration in our cohort using stepwise multivariate linear regression analysis. We then compared our adult sickle cell disease data to effect-size predictions from the published JHS statistical model of QT interval duration.
UNASSIGNED: In adult sickle cell disease, gender, diuretic use, QRS duration, serum ALT levels, anion gap, and diastolic blood pressure show positive correlation; hemoglobin levels show inverse correlation; in pediatric sickle cell disease, age, hemoglobin levels, and serum bicarbonate and creatinine levels show inverse correlation. The mean QTc in our adult sickle cell disease cohort is 7.8 milliseconds longer than in the JHS cohort, even though the JHS statistical model predicts that the mean QTc in our cohort should be > 11 milliseconds shorter than in the much older JHS cohort, a differential of > 18 milliseconds.
UNASSIGNED: Sickle cell disease patients have substantial QTc prolongation relative to their age, driven by factors some overlapping, in adult and pediatric sickle cell disease, and distinct from those that have been defined in the general African American community.
摘要：
■镰状细胞病，非洲裔美国人常见的遗传疾病，表明猝死的风险增加，其基础是不完全理解。心电图上心率校正QT（QTc）间期延长，心脏复极化的标准临床测量，可能会导致尖端扭转室性心动过速而导致猝死。
■我们建立了一项队列研究，纳入了293名成人和121名儿童镰状细胞病患者，这些患者来自与杰克逊心脏研究（JHS）队列相同的地理区域。其中QT持续时间的显著相关性已被表征和定量建模。在这里，我们使用逐步多元线性回归分析,在我们的队列中建立了QTc持续时间的临床和实验室相关性.然后，我们将我们的成人镰状细胞疾病数据与已发布的QT间期JHS统计模型的效应大小预测进行了比较。
■在成人镰状细胞病中，性别,利尿剂的使用，QRS持续时间,血清ALT水平，阴离子间隙,与舒张压呈正相关；血红蛋白水平呈负相关；在小儿镰状细胞病中，年龄,血红蛋白水平，血清碳酸氢盐和肌酐水平呈负相关。我们的成人镰状细胞病队列的平均QTc比JHS队列长7.8毫秒，尽管JHS统计模型预测我们队列中的平均QTc应该比更老的JHS队列短>11毫秒，>18毫秒的差分。
■镰状细胞病患者相对于他们的年龄有相当大的QTc延长，在一些重叠的因素的驱动下，成人和儿童镰状细胞病，并且不同于一般非裔美国人社区中定义的那些。
■我们建立了一项队列研究，纳入了293名成人和121名儿童镰状细胞病患者，这些患者来自与杰克逊心脏研究（JHS）队列相同的地理区域。其中QT持续时间的显著相关性已被表征和定量建模。在这里，我们使用逐步多元线性回归分析,在我们的队列中建立了QTc持续时间的临床和实验室相关性.然后，我们将我们的成人镰状细胞疾病数据与已发布的QT间期JHS统计模型的效应大小预测进行了比较。
■在成人镰状细胞病中，性别,利尿剂的使用，QRS持续时间,血清ALT水平，阴离子间隙,与舒张压呈正相关；血红蛋白水平呈负相关；在小儿镰状细胞病中，年龄,血红蛋白水平，血清碳酸氢盐和肌酐水平呈负相关。我们的成人镰状细胞病队列的平均QTc比JHS队列长7.8毫秒，尽管JHS统计模型预测我们队列中的平均QTc应该比更老的JHS队列短>11毫秒，>18毫秒的差分。
■镰状细胞病患者相对于他们的年龄有相当大的QTc延长，在一些重叠的因素的驱动下，成人和儿童镰状细胞病，并且不同于一般非裔美国人社区中定义的那些。
