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Transport proteins and acute phase reactant proteins in children with sickle cell anemia
R P Warrier1, S Kuvibidila, L Gordon
1Department of Pediatrics, Louisiana State University Medical Center, New Orleans 70112.
Insights
Sickle cell disease (SCD) children show significant weight and height deficits, with lower retinol-binding protein (RBP) and prealbumin levels. These nutritional markers are linked to growth retardation in SCD.
Area of Science:
- Pediatric Hematology
- Nutritional Biochemistry
- Growth and Development
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with chronic illness and potential growth impairments.
- Nutritional status, including transport proteins and acute-phase reactant proteins (APRP), plays a crucial role in the health outcomes of children with SCD.
Purpose of the Study:
- To investigate anthropometric measurements, hematological parameters, and levels of key transport and APRP proteins in children with SCD compared to healthy controls.
- To identify potential nutritional deficiencies and their correlation with growth deficits in pediatric SCD patients.
Main Methods:
- A cross-sectional study involving 34 children with SCD and 27 healthy controls, aged 0.5 to 16.5 years.
- Measurements included anthropometry (weight, height), hematology (WBC, hematocrit, Hb), and serum levels of albumin, transferrin, APRP, retinol-binding protein (RBP), and prealbumin.
- Statistical analysis was used to compare parameters between SCD children and controls, and between growth-retarded and growth-normal SCD children.
Main Results:
- SCD children exhibited significantly higher white blood cell counts and lower hematocrit and hemoglobin levels compared to controls.
- Weight and height deficits were observed in 41% and 25% of SCD boys, and 25% of SCD girls, respectively.
- SCD children had significantly lower mean levels of RBP and prealbumin, with abnormal RBP levels in 53% of cases. Growth-retarded SCD children showed reduced RBP and prealbumin levels.
Conclusions:
- Children with SCD experience significant growth deficits and altered hematological profiles.
- Lower levels of retinol-binding protein (RBP) and prealbumin are prevalent in SCD children, particularly those with growth retardation, suggesting potential nutritional deficiencies.
- Further research is needed to elucidate the role of protein-energy malnutrition in growth retardation among children with SCD.
Abstract:
Transport proteins, acute-phase reactant proteins (APRP), hematology, and anthropometry were studied in 34 sickle cell disease (SCD) children (20 boys, 14 girls) and 27 controls without growth deficits (13 boys, 14 girls) [corrected]. The age range was 1/2 to 16 1/2 years. Weight deficits (< 80%) by Waterlow's classification were observed in 41% of SCD boys and 25% of SCD girls, and height deficits (< 90%) were observed in 25% SCD boys and 25% girls. Mean white blood cell counts were significantly higher (P < .001) and hematocrit and hemoglobin (Hb) lower (P < .005) in SCD children than in controls. Although both groups had similar mean levels of albumin, transferrin, and APRP, SCD children had significantly lower mean levels of retinol-binding protein (RBP) (P < .001) and retinol-prealbumin (P < .001). Retinol-binding protein levels were abnormal in 18 (53%) SCD children and in only 23% controls (chi 2 = 14.06; P < 0.005); transferrin levels were abnormal in 20% of SCD children and in none of the controls. Children with SC and SF Hb phenotype had normal mean levels of RBP, whereas those with S beta thal and SS phenotype had levels below normal. Growth-retarded children by weight and height had reduced mean levels of RBP and prealbumin compared with growth-normal SCD children. The implication of primary protein-energy malnutrition on growth retardation in SCD children is under study.