Background: Tricuspid regurgitation velocity (TRV), measured by echocardiography, is a surrogate marker for pulmonary hypertension. Limited pediatric studies have considered the association between TRV and surrogate markers of end-organ disease. Methods: Therefore, we conducted a cross-sectional study that evaluated the prevalence of elevated TRV ≥ 2.5 m/s and its associations with renal and cerebrovascular outcomes in children with SCD 1-21 years of age in two large sickle cell cohorts, the University of Alabama at Birmingham (UAB) sickle cell cohort, and the Sickle Cell Clinical and Research Intervention Program (SCCRIP) cohort at St. Jude Children’s Research hospital. We hypothesized that patients with sickle cell disease with elevated TRV would have higher odds of having either albuminuria or cerebrovascular disease. Results: We identified 166 children from the UAB cohort (mean age: 13.49 ± 4.47 years) and 325 children from the SCCRIP cohort (mean age: 13.41 ± 3.99 years) with echocardiography. The prevalence of an elevated TRV was 21% in both UAB and SCCRIP cohorts. Elevated TRV was significantly associated with cerebrovascular disease (OR 1.88 (95% CI: 1.12- 3.15)) and persistent albuminuria (OR: 1.81 (95% CI: 1.07– 3.06)) after adjusting for age, sex, treatment, and site. Conclusion: This cross-sectional, multicenter study identifies associations between surrogate markers of pulmonary hypertension with kidney disease and cerebrovascular disease. A prospective study should be performed to evaluate the longitudinal outcomes for patients with multiple surrogate markers of end-organ disease.

Background: Children with sickle cell disease (SCD) have higher resting basal metabolic rates than healthy peers and have historically been underweight. Current treatments increase patients’ hemoglobin (Hb) level and decrease reticulocytosis, which may in turn decrease patients’ basal metabolic rates. Objective: To examine the rates of overweight and obesity in children with SCD compared to state and national norms and to assess the correlation between Hb and weight status. Methods: We conducted a retrospective chart review of patients with SCD between the ages of 2-19 years from October 2013 until April 2019. Data were collected from the most recent clinic visit and Body Mass Index (BMI) percentiles were calculated using Center for Disease Control growth charts. Mississippi and national weight status estimates for youth 10-17 years were obtained from the 2016-2017 National Survey of Children’s Health. Results: Data were available for 787 patients. For children 10-17 years (n = 480), 24.5% of patients were overweight/obese compared to Mississippi and national rates, 39.2% and 31%, respectively. The prevalence of 10-17 year olds who were overweight (12.7%) was not significantly different from Mississippi (13.1%) or national (15.2%) (p = 0.13). Hb were different among patients who were underweight (8.80g/dL), normal weight (9.2g/dL), and overweight/obese (10.5g/dL) (p < 0.001). Conclusions: Children with SCD in Mississippi have similar rates of overweight compared to state and national norms and have higher Hb than patients with SCD who are normal weight/underweight. The impact of increased BMI in SCD is unknown and additional longitudinal studies are needed.