Cellular and Molecular Alterations Underlying Abnormal Bone Growth in X-Linked Hypophosphatemia.
Int J Mol Sci
; 23(2)2022 Jan 15.
Article
em En
| MEDLINE
| ID: mdl-35055123
X-linked hypophosphatemia (XLH), the most common form of hereditary hypophosphatemic rickets, is caused by inactivating mutations of the phosphate-regulating endopeptidase gene (PHEX). XLH is mainly characterized by short stature, bone deformities and rickets, while in hypophosphatemia, normal or low vitamin D levels and low renal phosphate reabsorption are the principal biochemical aspects. The cause of growth impairment in patients with XLH is not completely understood yet, thus making the study of the growth plate (GP) alterations necessary. New treatment strategies targeting FGF23 have shown promising results in normalizing the growth velocity and improving the skeletal effects of XLH patients. However, further studies are necessary to evaluate how this treatment affects the GP as well as its long-term effects and the impact on adult height.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Raquitismo Hipofosfatêmico Familiar
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Endopeptidase Neutra Reguladora de Fosfato PHEX
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Fator de Crescimento de Fibroblastos 23
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Lâmina de Crescimento
Limite:
Animals
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Humans
Idioma:
En
Revista:
Int J Mol Sci
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Espanha