Harrison J E, Cumming W A, Fornasier V, Fraser D, Kooh S W, McNeill K G
Metabolism. 1976 Jan;25(1):33-40. doi: 10.1016/0026-0495(76)90157-8.
Seven adults with familial hypophosphatemia have been investigated by histologic and radiographic examination of bone, and estimates of bone mineral status by in vivo neutron activation analysis (IVNAA). Histological examination showed severe osteomalacia and osteosclerosis in all cases. Radiography showed skeletal deformities and other sequelae of severe rickets of childhood in five of the seven cases, with, in addition, thickened well-mineralized bones; the other two showed normal radiographs. IVNAA measurements showed that the first five had greater than normal bone calcium and that the other two had normal values. Thus, in all cases there is a greater than normal total bone tissue (osteoid and mineralized bone together). The quantitative body calcium measurements show clearly that osteosclerosis occurs in familial hypophosphatemia, confirming the opinions based on histological and radiological data. Although there has been occasional reference to this sclerosis in the literature, up to the present it has received little attention.
对7名患有家族性低磷血症的成年人进行了骨组织学和放射学检查，并通过体内中子活化分析（IVNAA）评估骨矿物质状态。组织学检查显示所有病例均有严重的骨软化症和骨硬化症。放射学检查显示，7例中有5例存在骨骼畸形和儿童期严重佝偻病的其他后遗症，此外还有骨矿化良好的骨骼增厚；另外2例放射学检查结果正常。IVNAA测量显示，前5例骨钙高于正常，另外2例数值正常。因此，在所有病例中，总的骨组织（类骨质和矿化骨）均高于正常。体内钙定量测量清楚地表明，家族性低磷血症会出现骨硬化症，这证实了基于组织学和放射学数据得出的观点。尽管文献中偶尔提及这种硬化症，但迄今为止它很少受到关注。
