<?xml version="1.0" encoding="UTF-8"?><xml><records><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Papadimas, G K</style></author><author><style face="normal" font="default" size="100%">Terzis, G.</style></author><author><style face="normal" font="default" size="100%">Methenitis, S</style></author><author><style face="normal" font="default" size="100%">Spengos, K.</style></author><author><style face="normal" font="default" size="100%">Papadopoulos, C.</style></author><author><style face="normal" font="default" size="100%">Vassilopoulou, S.</style></author><author><style face="normal" font="default" size="100%">Kavouras, S</style></author><author><style face="normal" font="default" size="100%">Michelakakis, H</style></author><author><style face="normal" font="default" size="100%">Manta, P</style></author></authors></contributors><titles><title><style face="normal" font="default" size="100%">Body composition analysis in late-onset Pompe disease.</style></title><secondary-title><style face="normal" font="default" size="100%">Mol Genet Metab</style></secondary-title><alt-title><style face="normal" font="default" size="100%">Mol. Genet. Metab.</style></alt-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">Absorptiometry, Photon</style></keyword><keyword><style  face="normal" font="default" size="100%">Adolescent</style></keyword><keyword><style  face="normal" font="default" size="100%">Adult</style></keyword><keyword><style  face="normal" font="default" size="100%">Aged</style></keyword><keyword><style  face="normal" font="default" size="100%">Body Composition</style></keyword><keyword><style  face="normal" font="default" size="100%">Bone Density</style></keyword><keyword><style  face="normal" font="default" size="100%">Female</style></keyword><keyword><style  face="normal" font="default" size="100%">Femur Neck</style></keyword><keyword><style  face="normal" font="default" size="100%">Glycogen Storage Disease Type II</style></keyword><keyword><style  face="normal" font="default" size="100%">Humans</style></keyword><keyword><style  face="normal" font="default" size="100%">Male</style></keyword><keyword><style  face="normal" font="default" size="100%">Middle Aged</style></keyword><keyword><style  face="normal" font="default" size="100%">Young Adult</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2011</style></year><pub-dates><date><style  face="normal" font="default" size="100%">2011 Jan</style></date></pub-dates></dates><volume><style face="normal" font="default" size="100%">102</style></volume><pages><style face="normal" font="default" size="100%">41-3</style></pages><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">Pompe disease is an inherited metabolic disorder caused by α-glycosidase deficiency. The adult onset form is mainly characterized by progressive proximal muscle weakness and respiratory dysfunction. The aim of the present study is to evaluate body composition in adult patients before and after enzyme replacement therapy (ERT). Body composition was examined at baseline by means of dual x-ray absorptiometry (DXA) in nine adult patients and after different time periods in six of them who received ERT. Total BMD (bone mineral density) was initially mildly decreased in two patients, while femoral neck BMD was decreased in five patients. On the other hand fat mass was increased in the majority of patients, while body mass index (BMI) was high in four. ERT administration did not seem to induce obvious BMD changes in any patient. Conclusively, the greater femoral neck BMD involvement may be attributed to the lower mechanical load applied by the selectively weakened muscles, whereas the increased fat mass may be the result of metabolic and nutritional derangement.</style></abstract><issue><style face="normal" font="default" size="100%">1</style></issue><custom1><style face="normal" font="default" size="100%">http://www.ncbi.nlm.nih.gov/pubmed/20926325?dopt=Abstract</style></custom1></record></records></xml>