家族性低磷血症性佝偻病和骨软化症的发病机制。
Pathogenesis of rickets and osteomalacia in familial hypophosphataemia.
作者信息
Condon J R, Nassim J R, Rutter A
出版信息
Arch Dis Child. 1971 Jun;46(247):269-72. doi: 10.1136/adc.46.247.269.
Calcium and/or phosphate tolerance tests were performed on patients with familial hypophosphataemia, normal control subjects, and patients with vitamin D deficient osteomalacia. Intestinal calcium absorption was similar in patients with familial hypophosphataemia and control subjects. The phosphate tolerance test, which is known to be `flat' in patients with familial hypophosphataemia, was normal in patients with vitamin D deficient osteomalacia. These findings suggest that rickets and osteomalacia in familial and some cases of non-familial hypophosphataemia are unrelated to abnormal metabolism of vitamin D. This hypothesis is supported by the fact that intestinal calcium absorption as measured by calcium tolerance test is normal in familial hypophosphataemia. It is suggested that the primary abnormality in familial hypophosphataemia is a partial metabolic block in the intestinal absorption and renal tubular reabsorption of phosphate.
对家族性低磷血症患者、正常对照者以及维生素D缺乏性骨软化症患者进行了钙和/或磷耐量试验。家族性低磷血症患者的肠道钙吸收与对照者相似。已知家族性低磷血症患者的磷耐量试验呈“平坦”状态，而维生素D缺乏性骨软化症患者的该试验结果正常。这些发现提示，家族性以及某些非家族性低磷血症中的佝偻病和骨软化症与维生素D的异常代谢无关。这一假说得到了以下事实的支持：通过钙耐量试验测定，家族性低磷血症患者的肠道钙吸收正常。提示家族性低磷血症的主要异常在于肠道对磷的吸收以及肾小管对磷的重吸收存在部分代谢障碍。
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