IMMUNE STATUS OF CHILDREN WITH PHENYLKETONURIA

Objective: To determine the effect of differences in plasma phenylalanine (Phe) concentrations (<363 umol/L, 363 to 605 umol/L, and >605 umol/L) on hematological and immunological parameters in 22 children with phenyllketonuria (PKU). Methods: Children with PKU were divided into one of three groups based on fasting plasma Phe levels. Hematologic and immunologic parameters of the children with PKU were compared between the groups and also compared with published values from age-matched children without PKU. Results: Hematologic and immunologic parameters did not differ among children with different plasma Phe concentrations. Specifically, no significant differences between groups of PKU children with differing plasma Phe levels were found for plasma levels of albumin, hemoglobin, amino acids, IgM, complement C3, interleukins 1 and 2, erythrocyte, leukocyte and differential cell counts, hematocrit, percentages and numbers of CD4(+), CD8(+), CD3(+) and total lymphocytes, or CD4 to CD8 ratio. Mean plasma IgG and IgA concentrations of the PKU children were, however, significantly lower than values from similar aged children. Moreover, positive correlations were obtained between plasma albumin and percentages and numbers of CD3(+) and CD4(+), between plasma IgG and interleukins 1 and 2, and between intakes of energy, protein, iron and plasma IgG levels. No correlations were found between plasma Phe and immunological parameters. Conclusion: While differences in plasma Phe concentrations up to concentrations of 866 umol/L do not appear to affect selected immune system parameters, further studies are needed to investigate the relationship between dietary nutrient intake, nutritional status, antibody biosynthesis and cytokine production. Assessment of plasma and cell membrane lipids and trace mineral status of PKU children would be helpful to determine if relationships exist between these nutrients and antibody production.