Csaba Sumánszki1, Erika Kiss2, Erika Simon2, Péter Reismann1.
1: 2nd Department of Medicine, Semmelweis University, Budapest; 2: 1st Department of Pediatrics, Semmelweis University;
Introduction: Phenylketonuria, is the most common inborn error of aminoacid metabolism. The risk of low bone density in phenylketonuria has widely been acknowledged, although the pathological mechanism is still unclear.
Aims: We aimed to determine the relationship between bone mass, nutritional status, and body composition parameters in adults patients with PKU.
Method: 80 patients, 41 premenopausal women and 39 male (age 18-49) subjects with classical PKU where included in our cross-sectional study. Lumbar spine and femoral neck bone mineral density (BMD) was assessed using dual X-ray absorptiometry (DXA), body composition was assessed by bioimpedance and pre-albumin levels where measured from blood.
Results: No significant difference in lumbar spine and femoral neck BMD Z-scores has been found between female and male subjects. Females subject significantly higher body fat percentage compared to males (means ± SD, females 35.6 ± 8.7; males 23.4 ± 11.1; p<0.001). In female subjects both lumbar spine and femoral BMD correlated positively with fat mass (r = 0.39; p=0.003, r = 0.47; p=0.002 respectively) and lean mass (r = 0.53; p<0.001, r = - 0.31; p=0.042 respectively). In male subjects lumbar spine BMD correlated positively fat mass (r = 0.31; p<0.001). Pre-albumin levels were higher in males compared to female subjects (means ± SD, females 28 ± 4.1; males 30.5 ± 3.8; p=0.008), but it did not correlate with BMD.
Conclusion: There was no difference in the BMD in adult female and male subjects. In female patients lean mass had a greater effect on BMD than fat mass. In male subjects only fat mass had effect on lumbar spine BMD. Lean mass is an important component in the prevention of bone loss, however this finding suggests that in PKU it has a stronger effect in premenopausal women than in male patients.
Doctoral School: Clinical Medicine
Program: Hormonal Regulations
Supervisor: Péter Reismann
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