Department of Pediatrics and Adolescent Medicine, Division of Pediatric Pulmonology, Allergology and Endocrinology, Medical University of Vienna, Vienna, Austria.
Vienna Bone and Growth Center, Vienna, Austria.
Curr Osteoporos Rep. 2023 Dec;21(6):787-805. doi: 10.1007/s11914-023-00822-6. Epub 2023 Oct 28.
Metabolic and genetic bone disorders affect not only bone mass but often also the bone material, including degree of mineralization, matrix organization, and lacunar porosity. The quality of juvenile bone is moreover highly influenced by skeletal growth. This review aims to provide a compact summary of the present knowledge on the complex interplay between bone modeling and remodeling during skeletal growth and to alert the reader to the complexity of bone tissue characteristics in children with bone disorders.
We describe cellular events together with the characteristics of the different tissues and organic matrix organization (cartilage, woven and lamellar bone) occurring during linear growth. Subsequently, we present typical alterations thereof in disorders leading to over-mineralized bone matrix compared to those associated with low or normal mineral content based on bone biopsy studies. Growth spurts or growth retardation might amplify or mask disease-related alterations in bone material, which makes the interpretation of bone tissue findings in children complex and challenging.
代谢和遗传骨骼疾病不仅影响骨量，而且常常影响骨的物质特性，包括矿化程度、基质组织和陷窝孔隙率。青少年骨骼的质量还受到骨骼生长的强烈影响。本综述旨在提供一个关于骨骼生长过程中骨建模和重塑之间复杂相互作用的简明概述，并提醒读者注意骨骼疾病患儿的骨组织特征的复杂性。
我们描述了线性生长过程中发生的细胞事件以及不同组织和有机基质组织（软骨、编织骨和板层骨）的特征。随后，我们根据骨活检研究，介绍了导致矿化过度的骨基质的疾病与那些与低或正常矿物质含量相关的疾病的典型改变。生长突增或生长迟缓可能会放大或掩盖与疾病相关的骨物质改变，这使得儿童的骨组织发现的解释变得复杂和具有挑战性。
