XLHRX-Linked Hypophosphataemic Rickets
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In children younger than 10 years with XLHR, femoral and tibial hemiepiphysiodesis are recommended to correct lower extremity deformities, which is a relatively minor surgical procedure to allow appropriate growth (114).
However, some patients with XLHR do not achieve the desired height velocity despite appropriate treatment (115,108).
In a study of 28 adults with XLHR who received monthly KRN23, a significant increase in serum phosphate, 1,25(OH)2D and maximum renal tubular threshold for phosphate reabsorption (TmP/GFR) has been observed after four or twelve months of treatment (121).
Oral phosphate alone is sufficient for patients with HHRH in contrast to patients with XLHR, ADHR or ARHP, who are usually treated with high doses of alphacalcidol or calcitriol and multiple daily doses of oral phosphate, low-sodium diet and hydration are recommended for the disease (5,26).
Although XLHR has been recognized since the 1930s, it was not until 1960, that Harris and Sullivan [1960] reported the dental abnormalities characteristic of the disease.
Other oral pathologies in XLHR reported in the literature include dental abrasion and periodontal disease [Pereira et al., 2004], absent or poorly defined lamina dura [Larmas et al., 1991], taurodontism and ectopic eruption of permanent canines [Seow et al., 1995].
Different clinical approaches have been proposed for the dental management of patients with XLHR: Breen [1986] and Rakocz et al.
McWhorter and Seale [1991] reported that 25% of patients with XLHR manifested abscesses in the primary dentition.