Abstract.
Hypertension has been anecdotally reported in children with familial hypophosphatemic rickets (XLH). To better identify and characterize the clinical and laboratory features of hypertensive XLH children, we reviewed the medical records of 41 XLH children, all treated with phosphate and vitamin D analogues. Eight children, who were originally normotensive, developed hypertension during the 2nd decade of life. At diagnosis of hypertension all had persistent secondary/tertiary hyperparathyroidism (HPTD), defined as high serum parathyroid hormone (PTH) for 12 months or longer. Seven had nephrocalcinosis (NC). Analysis of data showed that of 11 children with HPTD, 8 developed hypertension compared with 0 among 30 without HPTD (P<0.001). Of 40 children studied, 18 had NC that was significantly associated with both HPTD (P<0.01) and hypertension (P<0.025). At diagnosis of hypertension, serum calcium was elevated in 2. Plasma renin activity was high in 3 of 4 patients in whom it was measured. Doppler ultrasonography or renal scan was normal in the 5 children studied. Early echocardiography showed left ventricular hypertrophy in only 2 of 5 children studied. In 3 patients who underwent parathyroidectomy, hypertension persisted and 1 progressed to renal failure. Serum creatinine remained normal in all others. Successful treatment of hypertension consisted of β-adrenergic blockers, angiotensin converting enzyme inhibitors, and Ca channel blockers as monotherapy or in combination. We conclude that hypertension in treated XLH children is closely associated with HPTD. Emphasis should therefore be placed on prevention of the development of HPTD as a complication of XLH treatment, and close monitoring for hypertension in those who do develop HPTD.
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Acknowledgements.
This study was supported by The Sam and Helen Kaplan Research Fund in Pediatric Nephrology. The authors thank Ms. Andrea Hurst for her administrative assistance. Presented in part at the 36th ESPN meeting, Bilbao, 20–23 September 2002, and at the American Society of Nephrology 35th Annual Meeting, Philadelphia, 1–4 November 2002. Dedicated to the late Eric McClure.
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Alon, U.S., Monzavi, R., Lilien, M. et al. Hypertension in hypophosphatemic rickets—role of secondary hyperparathyroidism. Pediatr Nephrol 18, 155–158 (2003). https://doi.org/10.1007/s00467-002-1044-6
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DOI: https://doi.org/10.1007/s00467-002-1044-6