title: Homocysteine and other vascular risk factors in patients with phenylketonuria on a diet.
abstract: The aim of this study was to investigate the known risk factors, such as lipids, homocysteine and endothelin, for the development of coronary artery disease (CAD) in phenylketonuria (PKU) patients, depending on their diet. The PKU patients (n = 74) were divided into two groups. Group A (n = 34; mean age 6.78 +/- 1.5 y) adhered strictly to a diet and group B (n = 40; mean age 8.0 +/- 3.2 y) did not comply with the diet. The control group comprised 50 healthy non-PKU children. All groups were evaluated for blood levels of homocysteine and vitamin B6 by high-performance liquid chromatography, vitamin B12 and folate in serum by a radioassay, lipids by a routine method, and lipoprotein(a) and endothelin-1 with an immunoassay. Homocysteine levels (28.65 +/- 3.3 micromol l(-1)) were increased in group A compared with group B (6.86 +/- 1.6 micromol l(-1)) and the controls (6.9 +/- 2.0 micromol l(-1)) (p < 0.001). Vitamin B6 (10.7 +/- 10.9 nmol l(-1)), vitamin B12 (98.5 +/- 22.3 pmol l(-1)), folate (2.35 +/- 1.3 nmol l(-1)) and lipids were decreased in group A. The other vascular risk factors, which were not dependent on diet [lipoprotein(a) and endothelin-1], did not differ among the three groups.
  
  
  CONCLUSIONS
  PKU patients on a strict diet had low vitamin B6, vitamin B12 and folate levels resulting in moderate hyperhomocysteinaemia. The evaluation of these vitamins at short intervals and their supplementation could be an early measure in the prevention of CAD.
authors.affiliation: Institute of Child Health, Aghia Sophia Children's Hospital, Athens, Greece.
authors.forename: K H
authors.initials: KH
authors.lastname: Schulpis
authors.forename: G A
authors.initials: GA
authors.lastname: Karikas
authors.forename: E
authors.initials: E
authors.lastname: Papakonstantinou
mesh_terms: D002648:Child; D002675:Child, Preschool; D003324:Coronary Artery Disease / Q000097:blood* / Q000209:etiology*; D018753:Diet, Protein-Restricted / Q000009:adverse effects*; D019332:Endothelin-1 / Q000097:blood; D005492:Folic Acid / Q000097:blood; D006710:Homocysteine / Q000009:adverse effects* / Q000097:blood*; D006801:Humans; D008055:Lipids / Q000097:blood; D015596:Nutrition Assessment; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000150:complications* / Q000178:diet therapy*; D012307:Risk Factors; D014805:Vitamin B 12 / Q000097:blood; D025101:Vitamin B 6 / Q000097:blood
chemical_list: D019332:Endothelin-1; D008055:Lipids; D006710:Homocysteine; D010649:Phenylalanine; D025101:Vitamin B 6; D005492:Folic Acid; D014805:Vitamin B 12