Hypertension, Hyperlipidemia and Hyperglycemia in Pediatric Sickle Cell Disease

Children with sickle cell disease (SCD) have historically weighed less than their healthy peers but now have similar rates of raised body mass indices (BMIs), compared to national norms. Recent adult studies in SCD have shown increasing rates of metabolic syndrome. The aim of this study was to evaluate if weight status in pediatric patients with SCD contributed to increased risk of hypertension, hyperlipidemia and hyperglycemia.

A prospective cross sectional study was conducted by enrolling 100 children with SCD from ages 10-19 years, half who have BMI's below the 85th percentile (underweight and normal weight) and half who have BMIs greater than the 85th percentile (overweight and obese). Blood pressure was measured by sphygmomanometer. Venous blood sample was analyzed to detect blood glucose, fructosamine, total cholesterol, triglycerides, and low and high density lipoprotein after 8 hour overnight fasting. Two questionnaires were completed by parents to assess youth nutrition and exercise, as well as family history of potential risk factors for outcome variables (e.g., maternal overweight/obesity, family type II diabetes).

Results revealed that patients with HbSS/B0 were more likely to be on hydroxyurea. Patients with HbSC/SB+ had significantly higher BMI percentile, were younger, and had higher Hb/Hct compared to those with HbSS/SB0. Across genotypes, patients with a raised BMI had significantly higher systolic (Median (IQR) 118 [12.00]) and diastolic blood pressure (Median (IQR) 71 [14.00]). Fasting glucose (101 [13.00] vs 96 [9.00]) was higher in participants with raised BMI although fructosamine levels were not significantly higher. Patients with raised BMI also had significantly higher LDL levels compared to those with a normal BMI (73 [36] vs 63.5 [30]). Specifically, there were 7 participants with LDL in the borderline range, all in the raised BMI category. HbSC/SB genotype and higher Hb increased patient's odd of raised BMI by 40% and 7% respectively (OR=1.41, p< 0.001, OR= 1.07, p= 0.015). No differences were reported by parents regarding family nutrition and physical activity between BMI groups. However, family history of type II diabetes was a significant predictor of raised BMI (OR= 1.27, p 0.021).

Children with less severe genotypes of SCD are more likely to have raised BMI's. Raised BMI is associated with higher systolic and diastolic blood pressure, fasting glucose and LDL levels across genotypes. Current results raise concerns for future development of metabolic syndrome and type II diabetes in youth with SCD.

No relevant conflicts of interest to declare.