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Bone Material Properties in Bone Diseases Affecting Children
Adalbert Raimann1,2, Barbara M Misof2,3, Peter Fratzl4
1Department of Pediatrics and Adolescent Medicine, Division of Pediatric Pulmonology, Allergology and Endocrinology, Medical University of Vienna, Vienna, Austria.
Insights
Metabolic and genetic bone disorders impact bone quality and structure in children. Skeletal growth complicates interpretation of bone tissue findings in these pediatric patients.
Area of Science:
- Pediatric Endocrinology
- Bone Biology
- Skeletal Dysplasias
Background:
- Metabolic and genetic bone disorders affect bone mass, mineralization, matrix organization, and porosity.
- Juvenile bone quality is significantly influenced by skeletal growth processes.
- Understanding these factors is crucial for diagnosing and managing pediatric bone diseases.
Purpose of the Study:
- To summarize the complex interplay between bone modeling and remodeling during skeletal growth.
- To highlight the intricate bone tissue characteristics in children with metabolic and genetic bone disorders.
- To alert clinicians to the challenges in interpreting bone findings in pediatric patients.
Main Methods:
- Review of cellular events and tissue characteristics during linear skeletal growth.
- Analysis of bone biopsy studies in children with various bone disorders.
- Comparison of bone alterations in conditions with high, low, or normal mineral content.
Main Results:
- Skeletal growth involves distinct cellular events and tissue organization (cartilage, woven bone, lamellar bone).
- Disease-specific alterations in bone matrix mineralization and organization are observed.
- Growth spurts or retardation can amplify or mask underlying disease-related bone changes.
Conclusions:
- Bone modeling and remodeling during growth are complex and disease-dependent.
- Interpreting bone tissue findings in children with metabolic or genetic disorders requires careful consideration of growth influences.
- Accurate assessment of pediatric bone disorders necessitates understanding the interaction between disease and skeletal maturation.
Purpose Of Review:
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.
Recent Findings:
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.
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