title: Cardiovascular disease biomarkers in patients with inborn errors of protein metabolism: a pilot study.
abstract: BACKGROUND
  Limited data exist so far on cardiovascular disease biomarkers in patients maintained on a protein-restricted diet for inborn errors of protein metabolism. The present study aimed to analyse plasma cholesterol, lipoproteins, triglycerides and total homocysteine in patients with various inborn errors of protein metabolism in comparison with healthy controls.
  
  
  METHODS
  A cross-sectional study of cardiovascular disease biomarkers was conducted in a cohort of patients with inborn errors of protein metabolism: nine phenylketonuria, nine urea cycle defect, six branched chain organic acidaemia and two tyrosinaemia type I patients compared to 30 healthy controls. All patients were on a strict natural protein diet for a mean (SD) period of 5.37 (2.30) years (range 2-9 years). Dietary assessment, plasma cholesterol, triglycerides, lipoproteins and total homocysteine levels were obtained.
  
  
  RESULTS
  There were no significant differences in blood lipid studies and total homocysteine levels between patients and controls.
  
  
  CONCLUSIONS
  The results obtained in this pilot study suggest that cardiovascular disease biomarkers are not increased in patients with inborn errors of protein metabolism. This may be explained by the possible protective effect of a mono- and polyunsaturated fat rich Mediterranean diet. Additional studies with a larger number of patients are needed to confirm this finding.
authors: Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
authors: P E
authors: PE
authors: Karam
authors: Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
authors: M N
authors: MN
authors: Majdalani
authors: Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
authors: R T
authors: RT
authors: Daher
authors: Department of Nutrition, American University of Beirut Medical Center, Beirut, Lebanon.
authors: A
authors: A
authors: Barhoumi
authors: Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
authors: N
authors: N
authors: Yazbeck
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000097:blood*; D002318:Cardiovascular Diseases / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D003430:Cross-Sectional Studies; D004044:Dietary Proteins / Q000008:administration & dosage; D005260:Female; D006710:Homocysteine / Q000097:blood; D006801:Humans; D007223:Infant; D008074:Lipoproteins / Q000097:blood; D008297:Male; D008661:Metabolism, Inborn Errors / Q000097:blood* / Q000150:complications / Q000178:diet therapy; D015596:Nutrition Assessment; D010865:Pilot Projects; D012307:Risk Factors; D014280:Triglycerides / Q000097:blood
chemical_list: D015415:Biomarkers; D004044:Dietary Proteins; D008074:Lipoproteins; D014280:Triglycerides; D006710:Homocysteine; D002784:Cholesterol
keywords: homocysteine; lipids; low-protein diet; mediterranean diet