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[Bone metabolism in adults with phenylketonuria – Hungarian data].

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[Bone metabolism in adults with phenylketonuria – Hungarian data].

Orv Hetil. 2017 Nov;158(47):1868-1872

Authors: Barta AG, Sumánszki C, Reismann P

Summary
INTRODUCTION: Sufferers with phenylketonuria have decrease bone mineral density in comparison with wholesome folks, nevertheless, the ethiology of those alterations is just not clear. Hungarian knowledge had been lacking on this subject.
AIM: The principle intention of our research was to survey the correlation between metabolic management and alter of bone mineral density in early handled Hungarian grownup sufferers with phenylketonuria.
METHOD: On this monocentric research bone mineral density of 59 grownup PKU sufferers have been repeatedly measured in a Four-year interval utilizing dual-energy X-ray absorptiometry. Two subgroups have been established primarily based on common blood phenylalanine ranges. The correlation between the change in bone mineral density and common phenylalanine, tyrosine concentrations have been decided whereas preliminary bone mineral density and alter have additionally been examined within the subgroups.
RESULTS: Imply phenylalanine focus was 614 (182-1222) micromol/L, whereas imply tyrosine focus was 49 (24-99) micromol/L and the calculated ratio was 16 (Four,5-35). Three sufferers have had severely decreased bone mineral density in both localisation whereas 22 have had delicate lower. Low bone mineral density in comparison with cronological age has been discovered by 9 affected person. The imply change was +Zero.0380 (-Zero.1550-Zero.7800) g/cm2 in femur, and +Zero.0120 (-Zero.57300-Zero.3130) g/cm2 within the lumbar backbone. There was a correlation within the change in Z-score neither with imply phenylalanine nor with tyrosine focus.
CONCLUSIONS: Bone mineral density was not modified and hardly influenced by the metabolic management in early-treated younger grownup phenylketonuria sufferers in a number of years interval. Orv Hetil. 2017; 158(47): 1868-1872.

PMID: 29153023 [PubMed – indexed for MEDLINE]

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