Endocrinology and Metabolism Division, AUTH, Thessaloniki, Greece.
Hormones (Athens). 2010 Jul-Sep;9(3):274-8. doi: 10.14310/horm.2002.1277.
X-linked dominant hypophosphatemic rickets (XLH) is the most prevalent genetic form of hypophosphatemic rickets. Standard treatment of XLH patients includes long-term administration of phosphate and calcitriol. Treated patients usually respond well to the conventional therapy and demonstrate amelioration of rachitic symptoms and improved growth. However, long-term administration of phosphate and vitamin D preparations is sometimes complicated with nephrocalcinosis, secondary or tertiary hyperparathyroidism and arterial hypertension. We describe a patient with XLH, caused by a rare missense mutation of the PHEX gene. The patient, while under treatment with alphacalcidol and oral phosphate, developed hypercalciuria, nephrocalcinosis, secondary hyperparathyroidism and arterial hypertension. Cinacalcet was added to the therapeutic regimen and the long-term effects on calciotropic parameters and FGF23 levels are herein reported.
X 连锁显性低磷血症性佝偻病 (XLH) 是最常见的低磷血症性佝偻病的遗传形式。XLH 患者的标准治疗包括长期服用磷酸盐和骨化三醇。接受治疗的患者通常对常规治疗反应良好,佝偻病症状得到改善,生长得到改善。然而,长期服用磷酸盐和维生素 D 制剂有时会导致肾钙质沉着症、继发性或 tertiary 甲状旁腺功能亢进和动脉高血压。我们描述了一名 XLH 患者,其病因是 PHEX 基因突变。该患者在接受阿尔法骨化醇和口服磷酸盐治疗时出现高钙尿症、肾钙质沉着症、继发性甲状旁腺功能亢进和动脉高血压。添加西那卡塞到治疗方案中,并报告了对钙调节参数和 FGF23 水平的长期影响。