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Hypocholesterolemia in Nigerian children with sickle cell disease
Dorothy J VanderJagt1, Jaimie Shores, Anthony Okorodudu
1Department of Biochemistry and Molecular Biology, School of Medicine, University of New Mexico, Albuquerque 87131, USA.
Insights
Children with sickle cell disease (SCD) in Nigeria show low cholesterol levels but are not at increased risk for cardiovascular disease (CVD). However, low cholesterol may impact overall health and mortality.
Area of Science:
- Pediatric Hematology
- Cardiovascular Health
- Nutritional Biochemistry
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Cardiovascular disease (CVD) risk in Nigerian children with SCD is debated.
- Lipid profiles and body composition in these children require investigation.
Purpose of the Study:
- To analyze serum lipids and homocysteine levels in Nigerian children with SCD.
- To assess body composition in children with SCD.
- To determine the risk of CVD in this population.
Main Methods:
- Serum analysis of total cholesterol, HDL-cholesterol, LDL-cholesterol, triglycerides, and homocysteine.
- Bioelectrical impedance analysis for body composition (fat-free mass and body fat).
- Comparison of SCD patients (40 females, 37 males, ages 5-19) with age-matched controls.
Main Results:
- Children with SCD exhibited significantly reduced fat-free mass and increased body fat.
- Marked hypocholesterolemia (100-102 mg/dl) and low LDL-cholesterol (54 mg/dl) were observed.
- HDL-cholesterol levels were below the reference range, but LDL/HDL ratios were normal. Homocysteine was at the high end of normal.
Conclusions:
- Children with SCD in northern Nigeria do not appear to be at increased risk of CVD.
- The observed hypocholesterolemia warrants concern regarding overall mortality and well-being.
- Further research into the long-term health implications of lipid abnormalities in pediatric SCD is recommended.
Abstract:
Reports of circulating lipids of children with sickle cell disease (SCD) in Nigeria disagree on the question of whether these children are at increased risk of cardiovascular disease (CVD). We therefore analyzed the serum of 40 females and 37 males with SCD, age 5-19 years, and equal numbers of age-matched controls for total cholesterol, HDL-cholesterol, LDL-cholesterol, triglycerides, and homocysteine. Using bioelectrical impedance analysis, we documented a significant reduction in the per cent fat-free mass in the SCD males and increases in the per cent body fat in both the male and female children with SCD. Marked hypocholesterolemia was present in both genders (means, 100-102 mg/dl) and the LDL-cholesterol levels of the male and female subjects with SCD (54 mg/dl) were below the lower limit of the reference range (59-137 mg/dl). Serum triglycerides in the SCD subjects were in the middle of the reference range for children. Although the mean HDL-cholesterol levels of the SCD males (23.1 mg/dl) and females (24.5 mg/dl) were well below the lower limit of the reference range (35-84 mg/dl), respectively, the LDL-cholesterol/HDL-cholesterol ratios of the SCD subjects were not abnormal. The mean serum homocysteine concentrations of the male and female SCD subjects (9.4-9.6 micromol/l) were at the high end of the normal range. Collectively, these results indicate that children with SCD in northern Nigeria are not at increased risk of CVD. However, their marked hypocholesterolemia should be a cause of concern about the overall mortality and general well-being.