Bard H, Kuntz D, Molle D, Witvoet J, Ryckewaert A
Rev Rhum Mal Osteoartic. 1984 Feb;51(2):63-8.
Hyperphosphataemia with levels of 65 mg/l was found in a black African aged 20 with a 10-year history of tumour calcinosis. Levels of blood calcium, plasma ionised calcium, serum alkaline phosphatase, 24-hour urinary calcium and phosphate were all normal, as was renal function. Tubular phosphate reabsorption (TmP) was greater than 90 mg per litre of glomerular filtrate (N = 22-42). Levels of circulating parathyroid hormone, nephrogenic cAMP and serum vitamin D metabolites [25 OH D3, 24,25 (OH)2 D3 and 1,25 (OH)2 D3] were normal. The TmP fell by 36% on exogenous PTH stimulation (N = 30.25 +/- 6.7), and by 7.9% 120 min. after injection of acetazolamide. Our results confirm the conclusions of recent studies: patients with tumour calcinosis have disordered renal phosphate excretion with normal PTH secretion, normal PTH action on the renal tubule and normal vitamin D metabolism. In fact in these hyperphosphataemic patients, circulating 1,25 (OH)2 D3 levels would be expected to be low, whereas they were normal in our patient. An attempt at treatment with acetazolamide and phosphate chelating agents gave no significant results.
在一名20岁、有10年肿瘤性钙化病史的非洲黑人中发现血磷水平为65mg/l。血钙、血浆离子钙、血清碱性磷酸酶、24小时尿钙和磷水平均正常,肾功能也正常。肾小管磷重吸收(TmP)大于每升肾小球滤过液90mg(正常范围22 - 42)。循环甲状旁腺激素、肾源性环磷酸腺苷和血清维生素D代谢产物[25 - OH D3、24,25 - (OH)2 D3和1,25 - (OH)2 D3]水平正常。外源性甲状旁腺激素刺激后TmP下降36%(正常范围30.25±6.7),注射乙酰唑胺120分钟后下降7.9%。我们的结果证实了近期研究的结论:肿瘤性钙化患者存在肾磷排泄紊乱,但甲状旁腺激素分泌正常、甲状旁腺激素对肾小管的作用正常且维生素D代谢正常。实际上,在这些高磷血症患者中,预期循环中的1,25 - (OH)2 D3水平会较低,但我们的患者该水平正常。尝试用乙酰唑胺和磷酸盐螯合剂治疗未取得显著效果。