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Serum paraoxonase/arylesterase activities in phenylketonuric patients on diet

European Journal of Clinical Nutrition volume 61, pages 803–808 (2007)Cite this article

Abstract

Aim:

To compare serum paraoxonase/arylesterase (PON-aryl) activities in phenylketonuric (PKU) patients with high and low phenylalanine (Phe) blood concentration.

Patients and methods:

Seventeen poorly controlled PKU children (off diet) underwent clinical and laboratory examinations before and after 30 days adhering to their special diet (on diet), whereas controls (N=24) were examined once. Lipid, lipoprotein levels and paraoxonase (PON 1) activities were measured with the Bayer Advia 1650 Clinical Chemistry System. Apolipoprotein AI (Apo AI) levels were determined by the Dade Behring BN ProSpec nephelometer, whereas total antioxidant capacity (TAC), PON-aryl and Phe levels were measured spectrophotometrically.

Results:

Phe significantly differed among the groups. Lipids and lipoproteins, except high-density-lipoprotein-cholesterol (HDL-C) and Apo AI, were higher when off diet than those on diet. HDL-C and Apo AI were similar in patients and controls. TAC (0.99±0.19 mmol/l) was significantly lower when the patients were off diet than when they adhered to diet and controls (1.71±0.20 and 1.81±0.20 mmol/l P<0.001 respectively). PON 1 and PON-aryl activities (68±2 U/min/ml, 88±26 KU (min/ml) in children with high Phe were reduced as compared with those with low blood Phe levels (152±41 U/min/ml, 107±23 KU/min/ml P<0.001) and controls (146±43 U/min/ml, 109±41 KU/min/ml P<0.001). The enzyme activities positively correlated with HDL-C and Apo AI when PKU patients were on diet and controls as well as with TAC in all the groups, whereas negatively correlated with Phe levels.

Conclusions:

PON-aryl activities are strongly related to the dietary control of PKU patients.

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Acknowledgements

We are highly indebted to Mrs Anna Stamatis for her careful typing of this manuscript.

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Authors and Affiliations

  1. Institute of Child Health, ‘Aghia Sophia’ Children's Hospital, Athens, Greece

    K H Schulpis

  2. Department of Clinical Biochemistry, ‘Aghia Sophia’ Children's Hospital, Athens, Greece

    A Bartzeliotou & I Papassotiriou

  3. Department of Experimental Physiology, Athens University Medical School, Athens, Greece

    S Tsakiris

  4. ‘G Papanikolaou’ Research Center, ‘Saint Savas’ Regional Oncological Hospital of Athens, Athens, Greece

    A Gounaris

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Correspondence to I Papassotiriou.

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Schulpis, K., Bartzeliotou, A., Tsakiris, S. et al. Serum paraoxonase/arylesterase activities in phenylketonuric patients on diet. Eur J Clin Nutr 61, 803–808 (2007). https://doi.org/10.1038/sj.ejcn.1602591

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