Reassuring Data on the Cardiovascular Risk in Adults With X-linked Hypophosphatemia Receiving Conventional Therapy

Reassuring Data on the Cardiovascular Risk in Adults With X-linked Hypophosphatemia Receiving Conventional Therapy

Abstract Context X-linked hypophosphatemia (XLH) is a rare genetic disorder that results in increased plasma levels of fibroblast growth factor 23 (FGF23). Several studies have demonstrated a direct association between FGF23 and cardiovascular mortality in cohorts of patients with chronic renal fail...

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Published in:The journal of clinical endocrinology and metabolism Vol. 109; no. 2; pp. e488 - e494
Main Authors: Bouzemane, Alexandre, Vignot, Emmanuelle, Derain Dubourg, Laurence, De Mul, Aurélie, Molin, Arnaud, Chapurlat, Roland, Fontanges, Elisabeth, Delsart, Daphne, Akbari, Alireza, Huang, Shih Han Susan, McIntyre, Christopher W, Bacchetta, Justine, Lemoine, Sandrine
Format: Journal Article
Language:English
Published: US Oxford University Press 18-01-2024
Endocrine Society
Subjects:
Life Sciences
sodium
cardiovascular outcomes
X-linked hypophosphatemia (XLH)
MRI
Phosphates
Left Ventricular/diagnostic imaging/epidemiology/etiology
Familial Hypophosphatemic Rickets/complications
Pulse Wave Analysis
Mri
Hypertension/complications/epidemiology
Humans
Risk Factors
Cardiovascular Diseases/epidemiology/etiology
Sodium
Adult
Hypophosphatemia
Heart Disease Risk Factors
Fibroblast Growth Factors
Hypertrophy
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Summary:Abstract Context X-linked hypophosphatemia (XLH) is a rare genetic disorder that results in increased plasma levels of fibroblast growth factor 23 (FGF23). Several studies have demonstrated a direct association between FGF23 and cardiovascular mortality in cohorts of patients with chronic renal failure. However, in patients with XLH, studies on the cardiovascular impact of the disease are rare, with contradictory results. Objective The aim was to assess whether the disease led to an increased cardiovascular risk. Methods We conducted a single-center retrospective observational study on a local cohort of adult patients with XLH. The primary endpoint was a composite endpoint of the frequency of left ventricular hypertrophy (LVH) or presence of high blood pressure. Our secondary objectives were to assess echocardiographic, pulse wave velocity, and central blood pressure data as other markers of CV health. Independently of this cohort, tissue sodium content with magnetic resonance imaging was studied in 2 patients with XLH before and after burosumab. Results Twenty-two patients were included. Median serum phosphate was 0.57 (0.47-0.72) mmol/L and FGF23 94 pg/L (58-2226). Median blood pressure was 124 (115-130)/68 (65-80) mm Hg, with only 9% of patients being hypertensive. A majority of patients (69%) had no LVH, only 1 had a left ventricular mass >100 g/m² and 25% of patients had left ventricular remodeling. Pulse wave velocity was normal in all patients. No differences in skin and muscle sodium content were observed before and after burosumab in the 2 patients who underwent sodium magnetic resonance imaging. Conclusion We found no elevated risk of developing hypertension or LVH in patients with XLH.
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ISSN:0021-972X
1945-7197
DOI:10.1210/clinem/dgad608