Comparison of atherogenic risk factors among poorly controlled and well-controlled adolescent phenylketonuria patients.
BACKGROUND
Previous studies investigating the known risk factors of atherosclerosis in phenylketonuria patients have shown conflicting results. The primary aim of our study was to investigate the serum atherogenic markers in adolescent classical phenylketonuria patients and compare these parameters with healthy peers. The secondary aim was to compare these atherogenic markers in well-controlled and poorly controlled patients.


METHODS
A total of 59 patients (median age: 12.6 years, range: 11-17 years) and 44 healthy controls (median age: 12.0 years, range: 11-15 years) were enrolled in our study. Phenylketonuria patients were divided into two groups: well-controlled (serum phenylalanine levels below 360 µmol/L; 24 patients) and poorly controlled patients (serum phenylalanine levels higher than 360 µmol/L).


RESULTS
The mean high-density lipoprotein cholesterol levels of well-controlled patients (1.0±0.2 mmol/L) were significantly lower compared with poorly controlled patients and controls (1.1±0.2 mmol/L, p=0.011 and 1.4±0.2 mmol/L, p<0.001, respectively). Poorly controlled patients had lower high-density lipoprotein cholesterol levels than healthy controls (p=0.003). Homocysteine levels of both well-controlled (9.8±6.4 µmol/L) and poorly controlled (9.2±5.6 µmol/L) patients were higher compared with controls (5.8±1.8 µmol/L, p<0.01). The mean platelet volume of well-controlled patients (9.5±1.1 fL) was higher than that of poorly controlled patients and controls (8.9±0.8 fL, p=0.024 and 7.7±0.6 fL, p<0.001, respectively).


CONCLUSIONS
Lower high-density lipoprotein cholesterol and higher homocysteine and mean platelet volume levels were detected in phenylketonuria patients. In particular, these changes were more prominent in well-controlled patients. We conclude that phenylketonuria patients might be at risk for atherosclerosis, and therefore screening for atherosclerotic risk factors should be included in the phenylketonuria therapy and follow-up in addition to other parameters.
1Division of Metabolism,Ankara Children Education and Research Hospital,Ankara,Turkey.
Mehmet
M
Gündüz
2Department of Pediatrics, Faculty of Medicine,Dokuz Eylul University,Izmir,Turkey.
Sevim
S
Çakar
2Department of Pediatrics, Faculty of Medicine,Dokuz Eylul University,Izmir,Turkey.
Pınar
P
Kuyum
2Department of Pediatrics, Faculty of Medicine,Dokuz Eylul University,Izmir,Turkey.
Balahan
B
Makay
2Department of Pediatrics, Faculty of Medicine,Dokuz Eylul University,Izmir,Turkey.
Nur
N
Arslan
D000293:Adolescent; D050197:Atherosclerosis / Q000175:diagnosis*; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D008076:Cholesterol, HDL / Q000097:blood*; D005260:Female; D006710:Homocysteine / Q000097:blood*; D006801:Humans; D008297:Male; D063847:Mean Platelet Volume; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000097:blood*; D012307:Risk Factors; D014421:Turkey
D015415:Biomarkers; D008076:Cholesterol, HDL; D006710:Homocysteine; D010649:Phenylalanine
Phenylketonuria; high-density lipoprotein cholesterol; homocysteine; low-density lipoprotein cholesterol; mean platelet volume