Tieder M, Blonder J, Strauss S, Shaked U, Maor J, Gabizon D, Manor H, Sela B A
Pediatric Nephrology Unit, Assaf Harofeh Medical Center, Zerifin, Israel.
Nephron. 1993;64(4):526-31. doi: 10.1159/000187395.
The treatment of X-linked hypophosphatemia (XLH) consists of phosphate and vitamin D3 derivatives. Transient hypercalciuria and hypercalcemia are well-known signs of vitamin D intoxication. Despite urinary calcium excretion control, the danger of nephrocalcinosis in treated patients has been emphasized. It has recently been suggested that hyperoxaluria might be a causative factor of nephrocalcinosis other than calcium in phosphate-treated XLH patients. We measured urinary oxalate and phosphate excretion in 12 patients with the syndrome of hereditary hypophosphatemic rickets with hypercalciuria (HHRH) receiving only oral phosphates and in 5 XLH patients receiving both oral phosphates and vitamin D. No correlation was found between the dosage of phosphate supplements or urinary phosphate excretion and urinary oxalate excretion, in either group of patients. Nephrocalcinosis, presenting as hyperechogenicity of the medullary pyramids, was found in 2 of the 5 XLH patients and only in 2 HHRH patients who had been treated with excessive doses of vitamin D2 and calcium, prior to the true diagnosis being established. We conclude: (1) hyperoxaluria is not a cause of nephrocalcinosis in phosphate-treated patients with hereditary hypophosphatemic rickets; (2) prolonged phosphate treatment alone does not induce nephrocalcinosis in HHRH patients, and (3) we believe that in XLH patients, nephrocalcinosis is essentially due to vitamin D overdosage at some stage, or noncompliance in phosphate intake, leading to repeated undetected hypercalciuric periods.
X连锁低磷血症（XLH）的治疗包括磷酸盐和维生素D3衍生物。短暂性高钙尿症和高钙血症是维生素D中毒的已知体征。尽管对尿钙排泄进行了控制，但已强调了接受治疗的患者发生肾钙质沉着症的风险。最近有人提出，高草酸尿症可能是磷酸盐治疗的XLH患者中除钙之外导致肾钙质沉着症的一个病因。我们测量了12例仅接受口服磷酸盐治疗的遗传性低磷性佝偻病伴高钙尿症（HHRH）综合征患者以及5例同时接受口服磷酸盐和维生素D治疗的XLH患者的尿草酸和磷酸盐排泄情况。在两组患者中，均未发现磷酸盐补充剂剂量或尿磷酸盐排泄与尿草酸排泄之间存在相关性。在5例XLH患者中有2例发现肾钙质沉着症，表现为髓质锥体回声增强，而在2例HHRH患者中仅在确诊前曾接受过大剂量维生素D2和钙治疗的患者中发现肾钙质沉着症。我们得出以下结论：（1）高草酸尿症不是磷酸盐治疗的遗传性低磷性佝偻病患者发生肾钙质沉着症的原因；（2）单独长期使用磷酸盐治疗不会在HHRH患者中诱发肾钙质沉着症；（3）我们认为，在XLH患者中，肾钙质沉着症主要是由于在某个阶段维生素D过量或磷酸盐摄入不依从，导致反复出现未被发现的高钙尿期。
