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Cellular and Molecular Alterations Underlying Abnormal Bone Growth in X-Linked Hypophosphatemia.
Fuente, Rocío; García-Bengoa, María; Fernández-Iglesias, Ángela; Gil-Peña, Helena; Santos, Fernando; López, José Manuel.
Afiliação
  • Fuente R; Division of Pediatrics, Department of Medicine, Faculty of Medicine, University of Oviedo, 33006 Oviedo, Spain.
  • García-Bengoa M; Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), 33011 Oviedo, Spain.
  • Fernández-Iglesias Á; Institute of Physiology, Center for Integrative Human Physiology (ZIHP), University of Zurich, Winterthurerstrasse 190, CH-8057 Zurich, Switzerland.
  • Gil-Peña H; Division of Pediatrics, Department of Medicine, Faculty of Medicine, University of Oviedo, 33006 Oviedo, Spain.
  • Santos F; Research Center for Emerging Infections and Zoonoses (RIZ), University of Veterinary Medicine Hannover, 30559 Hanover, Germany.
  • López JM; Division of Pediatrics, Department of Medicine, Faculty of Medicine, University of Oviedo, 33006 Oviedo, Spain.
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Raquitismo Hipofosfatêmico Familiar / Endopeptidase Neutra Reguladora de Fosfato PHEX / Fator de Crescimento de Fibroblastos 23 / Lâmina de Crescimento Limite: Animals / Humans Idioma: En Revista: Int J Mol Sci Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Espanha

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Raquitismo Hipofosfatêmico Familiar / Endopeptidase Neutra Reguladora de Fosfato PHEX / Fator de Crescimento de Fibroblastos 23 / Lâmina de Crescimento Limite: Animals / Humans Idioma: En Revista: Int J Mol Sci Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Espanha