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FGF23 and Associated Disorders of Phosphate Wasting.
Pediatr Endocrinol Rev 2019; 17(1):17-34PE

Abstract

Fibroblast growth factor 23 (FGF23), one of the endocrine fibroblast growth factors, is a principal regulator in the maintenance of serum phosphorus concentration. Binding to its cofactor αKlotho and a fibroblast growth factor receptor is essential for its activity. Its regulation and interaction with other factors in the bone-parathyroid-kidney axis is complex. FGF23 reduces serum phosphorus concentration through decreased reabsorption of phosphorus in the kidney and by decreasing 1,25 dihydroxyvitamin D (1,25(OH)2D) concentrations. Various FGF23-mediated disorders of renal phosphate wasting share similar clinical and biochemical features. The most common of these is X-linked hypophosphatemia (XLH). Additional disorders of FGF23 excess include autosomal dominant hypophosphatemic rickets, autosomal recessive hypophosphatemic rickets, fibrous dysplasia, and tumor-induced osteomalacia. Treatment is challenging, requiring careful monitoring and titration of dosages to optimize effectiveness and to balance side effects. Conventional therapy for XLH and other disorders of FGF23-mediated hypophosphatemia involves multiple daily doses of oral phosphate salts and active vitamin D analogs, such as calcitriol or alfacalcidol. Additional treatments may be used to help address side effects of conventional therapy such as thiazides to address hypercalciuria or nephrocalcinosis, and calcimimetics to manage hyperparathyroidism. The recent development and approval of an anti-FGF23 antibody, burosumab, for use in XLH provides a novel treatment option.

Authors+Show Affiliations

Indiana University School of Medicine, Riley Hospital for Children, Fellow, Endocrinology and Diabetes, 705 Riley Hospital Drive, Room 5960, Indianapolis, IN 46202, USA, E-mail: agohil@iu.edu.Indiana University School of Medicine, Riley Hospital for Children, Associate Professor of Medicine and Pediatrics, 1120 West Michigan Street, CL 459, Indianapolis, IN 46202, USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

31599133

Citation

Gohil, Anisha, and Erik A. Imel. "FGF23 and Associated Disorders of Phosphate Wasting." Pediatric Endocrinology Reviews : PER, vol. 17, no. 1, 2019, pp. 17-34.
Gohil A, Imel EA. FGF23 and Associated Disorders of Phosphate Wasting. Pediatr Endocrinol Rev. 2019;17(1):17-34.
Gohil, A., & Imel, E. A. (2019). FGF23 and Associated Disorders of Phosphate Wasting. Pediatric Endocrinology Reviews : PER, 17(1), pp. 17-34. doi:10.17458/per.vol17.2019.gi.fgf23anddisordersphosphate.
Gohil A, Imel EA. FGF23 and Associated Disorders of Phosphate Wasting. Pediatr Endocrinol Rev. 2019;17(1):17-34. PubMed PMID: 31599133.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - FGF23 and Associated Disorders of Phosphate Wasting. AU - Gohil,Anisha, AU - Imel,Erik A, PY - 2019/10/11/entrez PY - 2019/10/11/pubmed PY - 2019/11/2/medline KW - 1,25(OH)2D KW - Burosumab KW - FGF23 KW - Klotho KW - Phosphorus KW - Rickets KW - XLH SP - 17 EP - 34 JF - Pediatric endocrinology reviews : PER JO - Pediatr Endocrinol Rev VL - 17 IS - 1 N2 - Fibroblast growth factor 23 (FGF23), one of the endocrine fibroblast growth factors, is a principal regulator in the maintenance of serum phosphorus concentration. Binding to its cofactor αKlotho and a fibroblast growth factor receptor is essential for its activity. Its regulation and interaction with other factors in the bone-parathyroid-kidney axis is complex. FGF23 reduces serum phosphorus concentration through decreased reabsorption of phosphorus in the kidney and by decreasing 1,25 dihydroxyvitamin D (1,25(OH)2D) concentrations. Various FGF23-mediated disorders of renal phosphate wasting share similar clinical and biochemical features. The most common of these is X-linked hypophosphatemia (XLH). Additional disorders of FGF23 excess include autosomal dominant hypophosphatemic rickets, autosomal recessive hypophosphatemic rickets, fibrous dysplasia, and tumor-induced osteomalacia. Treatment is challenging, requiring careful monitoring and titration of dosages to optimize effectiveness and to balance side effects. Conventional therapy for XLH and other disorders of FGF23-mediated hypophosphatemia involves multiple daily doses of oral phosphate salts and active vitamin D analogs, such as calcitriol or alfacalcidol. Additional treatments may be used to help address side effects of conventional therapy such as thiazides to address hypercalciuria or nephrocalcinosis, and calcimimetics to manage hyperparathyroidism. The recent development and approval of an anti-FGF23 antibody, burosumab, for use in XLH provides a novel treatment option. SN - 1565-4753 UR - https://www.unboundmedicine.com/medline/citation/31599133/FGF23_and_Associated_Disorders_of_Phosphate_Wasting DB - PRIME DP - Unbound Medicine ER -