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Journal of Inherited Metabolic Disease

, Volume 35, Issue 5, pp 817–821 | Cite as

Free asymmetric dimethylarginine (ADMA) is low in children and adolescents with classical phenylketonuria (PKU)

  • M. Huemer
  • B. Simma
  • D. Mayr
  • D. Möslinger
  • A. Mühl
  • I. Schmid
  • H. Ulmer
  • O.A. Bodamer
Original Article

Abstract

Introduction

Free asymmetric dimethylarginine (ADMA) is a competitive inhibitor of the nitric oxide synthases (NOS). Suppression of nitric oxide (NO) synthesis increases the risk of atherosclerosis. Nevertheless, in the condition of oxidative stress, NOS blockade by ADMA may exert protective effects. Protein metabolism is altered in patients with phenylketonuria (PKU) on dietary treatment and as shown recently, oxidative stress is high in PKU. Since free ADMA concentrations are determined by both protein metabolism and oxidative stress we hypothesized, that free ADMA levels may be elevated in PKU patients.

Design

Sixteen patients with PKU on dietary treatment (mean age 10.1 ± 5.2 yrs), and 91 healthy children (mean age 11.6 ± 3.7 yrs) participated in a cross sectional study.

Results

ADMA, total homocysteine (tHcy) and blood glucose were lower and the L-arginine/ADMA ratio was higher in PKU patients compared to controls. No significant correlation was present between phenylalanine (Phe) concentrations, protein intake, and lipid profile, history of cardiovascular disease or ADMA.

Discussion

In contrast to our hypothesis, ADMA was lower and the L-arginine/ADMA ratio was higher in PKU patients. Therefore, in PKU patients, the regulating function of ADMA on NO synthesis is altered and may thus contribute to oxidative stress.

Keywords

Nitric Oxide Nitric Oxide Synthases ADMA Level Phenylalanine Hydroxylase ADMA Concentration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Abbreviations

ADMA

asymmetric dimethylarginine

CVD

cardiovascular disease

DDAH

dimethylarginine dimethylaminohydrolase

HDL

high density lipoprotein

NOS

nitric oxide synthase(s)

NO

nitric oxide

Phe

phenylalanine

PKU

phenylketonuria

tHcy

total homocysteine

Notes

Acknowledgements

We are indebted to all study participants and their families, to the Dept. of Anesthesia and ENT at the Landeskrankenhaus Feldkirch, to the participating Pediatricians and Mrs. Helbok-Blum for their support. We gratefully acknowledge the Federal Government of Vorarlberg for financial support of our work.

Details of funding

The work has been funded by the Federal Government of Vorarlberg, Austria. The authors confirm independence from the sponsors; the content of the article has not been influenced by the sponsors.

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Copyright information

© SSIEM and Springer 2012

Authors and Affiliations

  • M. Huemer
    • 1
  • B. Simma
    • 2
  • D. Mayr
    • 1
  • D. Möslinger
    • 3
  • A. Mühl
    • 4
  • I. Schmid
    • 5
  • H. Ulmer
    • 6
  • O.A. Bodamer
    • 7
    • 8
  1. 1.Department of PediatricsLandeskrankenhaus BregenzBregenzAustria
  2. 2.Department of PediatricsLandeskrankenhaus FeldkirchFeldkirchAustria
  3. 3.University Children’s HospitalViennaAustria
  4. 4.CentogeneViennaAustria
  5. 5.University Institute for Clinical ChemistrySalzburgAustria
  6. 6.Department of Medical Statistics, Informatics and Health EconomicsInnsbruck Medical UniversityInnsbruckAustria
  7. 7.University Children’s HospitalSalzburgAustria
  8. 8.Institute for Inherited Metabolic DiseasesParacelsus Medical UniversitySalzburgAustria

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