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Inherited fibroblast growth factor 23 excess.
Cherian, Kripa Elizabeth; Paul, Thomas Vizhalil.
Afiliação
  • Cherian KE; Department of Endocrinology, Christian Medical College, Vellore, India.
  • Paul TV; Department of Endocrinology, Christian Medical College, Vellore, India. Electronic address: thomasvpaul@yahoo.com.
Best Pract Res Clin Endocrinol Metab ; 38(2): 101844, 2024 Mar.
Article em En | MEDLINE | ID: mdl-38044258
Syndromes of inherited fibroblast growth factor 23 (FGF-23) excess encompass a wide spectrum that includes X-linked hypophosphataemia (XLH), autosomal dominant and recessive forms of rickets as well as various syndromic conditions namely fibrous dysplasia/McCune Albright syndrome, osteoglophonic dysplasia, Jansen's chondrodysplasia and cutaneous skeletal hypophosphataemia syndrome. A careful attention to patient symptomatology, family history and clinical features, supported by appropriate laboratory tests will help in making a diagnosis. A genetic screen may be done to confirm the diagnosis. While phosphate supplements and calcitriol continue to be the cornerstone of treatment, in recent times burosumab, the monoclonal antibody against FGF-23 has been approved for the treatment of children and adults with XLH. While health-related outcomes may be improved by ensuring adherence and compliance to prescribed treatment with a smooth transition to adult care, bony deformities may persist in some, and this would warrant surgical correction.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hipofosfatemia / Raquitismo Hipofosfatêmico Familiar Idioma: En Revista: Best Pract Res Clin Endocrinol Metab Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Índia

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hipofosfatemia / Raquitismo Hipofosfatêmico Familiar Idioma: En Revista: Best Pract Res Clin Endocrinol Metab Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Índia