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Craniosynostosis in vitamin D-resistant rickets. A mouse model
Abstract:
Craniosynostosis and associated craniofacial deformities, such as frontal bossing, often occur as symptoms of vitamin D-resistant rickets in children. Similar skull deformities develop in mice with X-linked dominant hypophosphatemia, the most common form of vitamin D-resistant rickets. These mice have a short, wide, high neurocranium, which suggested an inhibition of coronal suture growth. To study this question, we compared histologically the postnatal development of the coronal sutures in normal and hypophosphatemic mice between 1 and 13 weeks of age. Premature fusion of the coronal suture occurred in hypophosphatemic mice by 4 weeks of age. The proportion of the suture obliterated by bone varied among individual animals, but craniosynostosis was present in all animals studied at 4 weeks and older. Fusion of the coronal suture did not occur through 13 weeks of age in any of the normal mice studied. The x-linked hypophosphatemic mouse is an animal model that can be used to study the role of vitamin D-resistant rickets in the development of craniosynostosis, to relate craniosynostosis to the development of associated skull deformities, and to test new treatment procedures.
Insights
Vitamin D-resistant rickets in mice causes premature coronal suture fusion, leading to craniosynostosis. This mouse model aids research into rickets-related skull deformities and treatments.
Area of Science:
- Pediatric Endocrinology
- Developmental Biology
- Craniofacial Surgery
Background:
- Craniosynostosis and frontal bossing are common in children with vitamin D-resistant rickets.
- X-linked dominant hypophosphatemia, a form of vitamin D-resistant rickets, causes similar skull deformities in mice.
Purpose of the Study:
- To investigate the effect of X-linked dominant hypophosphatemia on coronal suture development in mice.
- To establish the x-linked hypophosphatemic mouse as a model for studying rickets-induced craniosynostosis.
Main Methods:
- Histological comparison of coronal suture development in normal and hypophosphatemic mice.
- Age range: postnatal weeks 1 to 13.
Main Results:
- Premature fusion of the coronal suture observed in hypophosphatemic mice by 4 weeks of age.
- Craniosynostosis was present in all studied hypophosphatemic mice aged 4 weeks and older.
- Normal mice did not exhibit coronal suture fusion up to 13 weeks of age.
Conclusions:
- The x-linked hypophosphatemic mouse model is suitable for studying craniosynostosis associated with vitamin D-resistant rickets.
- This model can help elucidate the relationship between rickets and craniofacial deformities.
- It provides a platform for testing novel therapeutic interventions for craniosynostosis.