Related Experiment Video
Updated: Jan 31, 2026
Real-time Analyses of Retinol Transport by the Membrane Receptor of Plasma Retinol Binding Protein
Published on: January 28, 2013
Clinical Observations, Plasma Retinol Concentrations, and In Vitro Lymphocyte Functions in Children With Sickle Cell
Solo R Kuvibidila1, Renée Gardner1, Maria Velez1
1Department of Pediatrics, Louisiana State University Health Sciences Center, New Orleans, LA.
Insights
Children with sickle cell disease (SCD) may have suboptimal vitamin A levels, impacting their immune function and health. Addressing vitamin A deficiency could improve immunity and reduce complications in these children.
Area of Science:
- Pediatric Hematology
- Nutritional Immunology
- Biochemistry
Background:
- Children with sickle cell disease (SCD) exhibit increased susceptibility to infections, growth deficits, and impaired immunity.
- These health issues mirror those seen in individuals with vitamin A deficiency, characterized by low plasma retinol concentrations (<20 μg/dL).
Purpose of the Study:
- To investigate the association between vitamin A status, overall health, and in vitro immune function in children with SCD.
Main Methods:
- Plasma retinol, retinol binding protein, C-reactive protein, and alpha-1-acid glycoprotein levels were measured in 59 children with SCD (ages 9 months to 18 years).
- In vitro immune function was assessed by measuring lymphocyte proliferation and interleukin-2 (IL-2) activity.
- Participants included children with SS, SC, and Sβthalassemia hemoglobin genotypes.
Main Results:
- Plasma retinol concentrations in children with SCD were comparable to those in healthy individuals but were lower in boys than girls and in children with inflammation.
- Seven children (11.9%) were vitamin A-deficient, and 9 (15.3%) had suboptimal vitamin A status.
- Children with vitamin A deficiency showed trends toward lower height and weight percentiles, reduced lymphocyte proliferation, and IL-2 activity, alongside higher C-reactive protein levels, pain crisis episodes, and inflammation.
Conclusions:
- Suboptimal vitamin A status is present in a significant proportion of children with SCD.
- Identification and correction of vitamin A deficiency may enhance immune responses.
- Improving vitamin A status holds potential for attenuating health complications associated with SCD.
Background:
Children with sickle cell disease (SCD) often have infections, growth deficits, and impaired immunity, problems that also are observed in individuals with a vitamin A deficiency (plasma retinol concentration <20 μg/dL). The goal of this study was to investigate the association between vitamin A, health status, and the in vitro immune function of children with SCD.
Methods:
Fifty-nine children (40 SS, 11 SC, and 8 Sβthalassemia [Sβthal] hemoglobin genotypes) 9 months to 18 years old were investigated for plasma levels of retinol, retinol binding protein, C-reactive protein, alpha-1-acid glycoprotein, lymphocyte proliferation, and interleukin (IL)-2 activity in supernatant of phytohemagglutinin-treated lymphocytes.
Results:
The plasma retinol concentrations of children with SCD (mean 57.6 μg/dL, range 4.6-116 μg/dL) were not different from those of 21 normal individuals (mean 62 μg/dL, range 28.7-162 μg/dL). Plasma retinol concentrations did not vary by hemoglobin genotype but were lower in boys than in girls (P < 0.05) and were also lower in children with inflammation (P = 0.1). Seven children (11.9%) (6 HbSS, 1 HbSβ0thal) were vitamin A-deficient, and 9 children (15.3%) had suboptimal vitamin A status (plasma retinol concentration of 20-29 μg/dL). Children with vitamin A deficiency had slightly lower height (P = 0.09) and weight mean percentiles, lymphocyte proliferative responses, and IL-2 activity (P > 0.1), but higher means of C-reactive protein (P = 0.05), pain crisis episodes and inflammation (P = 0.1), and health scores (P > 0.1) than children who were not vitamin A-deficient. Lymphocyte proliferative responses negatively correlated with health score, pain crisis episodes, and blood units received, but positively correlated with retinol binding protein (P < 0.05 to P = 0.1).
Conclusion:
Identification and correction of suboptimal vitamin A status in children with SCD may improve immunity and attenuate certain health complications associated with this disease.
More Related Videos
05:23Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
09:18Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Related Concept Videos
02:41Concentration Cells
Consider the following voltaic cell:
01:24Chronic Kidney Disease II: Clinical Manifestations
01:30Coronary Artery Disease III: Clinical Manifestations
01:29Gastroesophageal Reflux Disease II: Clinical Features and Management
Clinical Manifestations
GERD presents itself in a multitude of ways, with symptoms varying from person to person. The hallmark symptoms are...
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
01:21Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation