Iron and fibroblast growth factor 23 in X-linked hypophosphatemia

dc.contributor.authorImel, Erik A.
dc.contributor.authorGray, Amie
dc.contributor.authorPadgett, Leah
dc.contributor.authorEcons, Michael J.
dc.contributor.departmentDepartment of Medicine, IU School of Medicineen_US
dc.date.accessioned2016-03-03T17:51:54Z
dc.date.available2016-03-03T17:51:54Z
dc.date.issued2014-03
dc.description.abstractBackground Excess fibroblast growth factor 23 (FGF23) causes hypophosphatemia in autosomal dominant hypophosphatemic rickets (ADHR) and X-linked hypophosphatemia (XLH). Iron status influences C-terminal FGF23 (incorporating fragments plus intact FGF23) in ADHR and healthy subjects, and intact FGF23 in ADHR. We hypothesized that in XLH serum iron would inversely correlate to C-terminal FGF23, but not to intact FGF23, mirroring the relationships in normal controls. Methods Subjects included 25 untreated outpatients with XLH at a tertiary medical center and 158 healthy adult controls. Serum iron and plasma intact FGF23 and C-terminal FGF23 were measured in stored samples. Results Intact FGF23 was greater than the control mean in 100% of XLH patients, and >2SD above the control mean in 88%, compared to 71% and 21% respectively for C-terminal FGF23. In XLH, iron correlated negatively to log-C-terminal FGF23 (r= −0.523, p<0.01), with a steeper slope than in controls (p<0.001). Iron was not related to log-intact FGF23 in either group. The log-ratio of intact FGF23 to C-terminal FGF23 was higher in XLH (0.00 ± 0.44) than controls (−0.28 ± 0.21, p<0.01), and correlated positively to serum iron (controls r= 0.276, p<0.001; XLH r= 0.428, p<0.05), with a steeper slope in XLH (p<0.01). Conclusion Like controls, serum iron in XLH is inversely related to C-terminal FGF23 but not intact FGF23. XLH patients are more likely to have elevated intact FGF23 than C-terminal FGF23. The relationships of iron to FGF23 in XLH suggest altered regulation of FGF23 cleaving may contribute to maintaining hypophosphatemia around an abnormal set-point.en_US
dc.eprint.versionAuthor's manuscripten_US
dc.identifier.citationImel, E. A., Gray, A., Padgett, L., & Econs, M. J. (2014). Iron and fibroblast growth factor 23 in X-linked hypophosphatemia. Bone, 60, 87–92. http://doi.org/10.1016/j.bone.2013.12.001en_US
dc.identifier.urihttps://hdl.handle.net/1805/8676
dc.language.isoen_USen_US
dc.publisherElsevier B.V.en_US
dc.relation.isversionof10.1016/j.bone.2013.12.001en_US
dc.relation.journalBoneen_US
dc.rightsPublisher Policyen_US
dc.sourcePMCen_US
dc.subjectFibroblast growth factor 23en_US
dc.subjectFGF23en_US
dc.subjectX-linked hypophosphatemiaen_US
dc.subjectironen_US
dc.subjectphosphateen_US
dc.titleIron and fibroblast growth factor 23 in X-linked hypophosphatemiaen_US
dc.typeArticleen_US
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