Reduced bone mineral density in glycogen storage disease type III: evidence for a possible connection between metabolic imbalance and bone homeostasis
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- 作者：Daniela Melisa ; daniela.melis@unina.it" class="auth_mail" title="E-mail the corresponding author ; Alessandro Rossia ; ale.ro_ar@libero.it" class="auth_mail" title="E-mail the corresponding author ; Rosario Pivonellob ; rosario.pivonello@unina.it" class="auth_mail" title="E-mail the corresponding author ; Antonio Del Puentec ; delpuente@unina.it" class="auth_mail" title="E-mail the corresponding author ; Claudia Pivonellob ; cpivonello@gmail.com" class="auth_mail" title="E-mail the corresponding author ; Giuliana Cangemid ; giuliana.cangemi@gmail.com" class="auth_mail" title="E-mail the corresponding author ; Mariarosaria Negrib ; negrimariarosaria@yahoo.it" class="auth_mail" title="E-mail the corresponding author ; Annamaria Colaob ; colao@unina.it" class="auth_mail" title="E-mail the corresponding author ; Generoso Andriaa ; andria@unina.it" class="auth_mail" title="E-mail the corresponding author ; Giancarlo Parentia ; parenti@unina.it" class="auth_mail" title="E-mail the corresponding author
- 关键词：GDE ; Glycogen debranching enzyme ; BMD ; bone mineral density ; DXA ; dual-emission-X-ray absorptiometry ; QUS ; quantitative ultrasound ; OC ; osteocalcin ; CTX ; C-terminal cross-linking telopeptide of type I collagen ; IPAQ ; International Physical Activity Questionnaire
- 刊名：Bone
- 出版年：2016
- 出版时间：May 2016
- 年：2016
- 卷：86
- 期：Complete
- 页码：79-85
- 全文大小：513 K
文摘
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The pathogenesis of osteopenia in glycogen storage disease type III (GSDIII) is still unclear
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Several endocrine and metabolic factors are known to affect BMD as well as physical activity
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We found reduced levels of IGF-1, IGF-1/IGFBP3 molar ratio, insulin, calcitonin and osteocalcin and increased serum levels of CTX
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DXA Z-score correlated inversely with cholesterol and triglycerides levels and directly with IGF-1/IGFBP3 molar ratio
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Results shed light on possible factors leading to osteopenia and may be relevant in the management of GSDIII patients