Clinical Observations, Plasma Retinol Concentrations, and In Vitro Lymphocyte Functions in Children With Sickle Cell...

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 Sβ^thalassemia 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.