Hyperparathyroidism and parathyroidectomy in X-linked hypophosphatemia patients

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dc.contributor.authorDeLacey, Sean
dc.contributor.authorLiu, Ziyue
dc.contributor.authorBroyles, Andrea
dc.contributor.authorEl-Azab, Sarah A.
dc.contributor.authorGuandique, Cristian F.
dc.contributor.authorJames, Benjamin C.
dc.contributor.authorImel, Erik A.
dc.contributor.departmentMedicine, School of Medicineen_US
dc.date.accessioned2021-07-23T15:52:17Z
dc.date.available2021-07-23T15:52:17Z
dc.date.issued2019-10-01
dc.description.abstractBackground X-linked hypophosphatemia (XLH) causes rickets, osteomalacia, skeletal deformities and growth impairment, due to elevated fibroblast growth factor 23 and hypophosphatemia. Conventional therapy requires high doses of phosphate salts combined with active vitamin D analogues. Risks of this regimen include nephrocalcinosis and secondary hyperparathyroidism or progression to tertiary (hypercalcemic) hyperparathyroidism. Methods The primary goals were to estimate the prevalence of hyperparathyroidism and to characterize parathyroidectomy outcomes regarding hypercalcemia among XLH patients. XLH patients attending our center from 1/2000 to 12/2017 were included in a retrospective chart review. Prevalence of nephrocalcinosis and eGFR<60 mL/min/1.732 was also assessed. Results Of 104 patients with XLH, 84 had concurrent measurements of calcium and PTH (40 adults and 44 children). Of these, 70/84 (83.3%), had secondary or tertiary hyperparathyroidism at any time point. Secondary hyperparathyroidism was persistent in 62.2% of those with data at multiple timepoints. Tertiary hyperparathyroidism had an overall prevalence of 14/84 (16.7%) patients. Parathyroidectomy was performed in 8/84 (9.5%) of the total population. After parathyroidectomy, persistent or recurrent tertiary hyperparathyroidism was detected in 6/8 (75%) patients at a median of 6 years (from 0 to 29 years). One patient had chronic postoperative hypoparathyroidism and one patient remained normocalcemic 4 years after surgery. Nephrocalcinosis was more prevalent in patients with tertiary hyperparathyroidism than those without (60.0% vs 18.6%). Chronic kidney disease (eGFR<60 mL/min/1.732) was also more prevalent in patients with tertiary hyperparathyroidism than those without (35.7% vs 1.5%). Conclusion The majority of patients with XLH develop secondary hyperparathyroidism during treatment with phosphate and active vitamin D. A significant proportion develops tertiary hyperparathyroidism and most have recurrence or persistence of hypercalcemia after surgery.en_US
dc.eprint.versionAuthor's manuscripten_US
dc.identifier.citationDeLacey, S., Liu, Z., Broyles, A., El-Azab, S. A., Guandique, C. F., James, B. C., & Imel, E. A. (2019). Hyperparathyroidism and parathyroidectomy in X-linked hypophosphatemia patients. Bone, 127, 386–392. https://doi.org/10.1016/j.bone.2019.06.025en_US
dc.identifier.issn8756-3282en_US
dc.identifier.urihttps://hdl.handle.net/1805/26284
dc.language.isoen_USen_US
dc.publisherElsevieren_US
dc.relation.isversionof10.1016/j.bone.2019.06.025en_US
dc.relation.journalBoneen_US
dc.sourcePMCen_US
dc.subjectHyperparathyroidismen_US
dc.subjectKidneyen_US
dc.subjectParathyroidectomyen_US
dc.subjectFamilial Hypophosphatemic Ricketsen_US
dc.titleHyperparathyroidism and parathyroidectomy in X-linked hypophosphatemia patientsen_US
dc.typeArticleen_US
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