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Immune status of children with phenylketonuria
S S Gropper1, H C Chaung, L E Bernstein
1Department of Nutrition and Food Science, Auburn University, Alabama.
Insights
Plasma phenylalanine levels up to 866 umol/L did not impact most immune markers in children with phenylketonuria (PKU). However, lower IgG and IgA were observed, suggesting further research into nutrient intake and immune function is needed.
Area of Science:
- Biochemistry
- Immunology
- Pediatrics
Background:
- Phenylketonuria (PKU) is a genetic disorder requiring dietary management of phenylalanine (Phe).
- The impact of varying Phe levels on immune function in PKU patients remains incompletely understood.
Purpose of the Study:
- To investigate the relationship between plasma Phe concentrations and hematological and immunological parameters in children with PKU.
- To compare these parameters with age-matched controls without PKU.
Main Methods:
- Children with PKU were stratified into three groups based on plasma Phe levels (< 363, 363–605, and > 605 umol/L).
- Hematologic and immunologic markers were analyzed and compared between groups and with published data from healthy children.
Main Results:
- No significant differences in most hematological and immunological parameters were found among PKU children with varying plasma Phe levels.
- PKU children exhibited significantly lower mean plasma IgG and IgA concentrations compared to controls.
- Positive correlations were observed between plasma albumin and lymphocyte counts, and between plasma IgG and nutrient intake, but not between plasma Phe and immune parameters.
Conclusions:
- Plasma Phe concentrations up to 866 umol/L do not appear to affect selected immune parameters in children with PKU.
- Further research is warranted to explore the interplay of dietary intake, nutritional status, and immune responses, including antibody and cytokine production.
- Assessing plasma and cell membrane lipids and trace mineral status may elucidate relationships with antibody production in PKU.
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 phenylketonuria (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.