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Comparison of atherogenic risk factors among poorly controlled and well-controlled adolescent phenylketonuria patients

Published online by Cambridge University Press:  17 August 2015

Mehmet Gündüz ,
Sevim Çakar ,
Pınar Kuyum ,
Balahan Makay  and
Nur Arslan
Mehmet Gündüz
Affiliation:
Division of Metabolism, Ankara Children Education and Research Hospital, Ankara, Turkey
Sevim Çakar
Affiliation:
Department of Pediatrics, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey
Pınar Kuyum
Affiliation:
Department of Pediatrics, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey
Balahan Makay
Affiliation:
Department of Pediatrics, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey
Nur Arslan*
Affiliation:
Department of Pediatrics, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey Department of Molecular Medicine, Izmir, Turkey
*
Correspondence to: Dr N. Arslan, MD, PhD, Department of Pediatrics, Division of Pediatric Gastroenterology, Nutrition and Metabolism, Faculty of Medicine, Department of Molecular Medicine, Dokuz Eylul University, Izmir, Turkey. Tel: +0090 2324126107; Fax: +0090 2324126005; E- mail: [email protected]
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Abstract

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).

Conclusion

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.

Keywords

Phenylketonurialow-density lipoprotein cholesterolhigh-density lipoprotein cholesterolmean platelet volumehomocysteine
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 5 , June 2016 , pp. 901 - 908
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Copyright
© Cambridge University Press 2015 

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