Kitagawa T, Akatsuka A, Owada M, Mano T
Contrib Nephrol. 1980;22:107-19. doi: 10.1159/000385993.
The plasma 1,25-(OH)2VD3 level in Fanconi syndrome was low but rose rapidly following the administration of 1 alpha-OHVD3. 1 alpha-OHVD3 was administered to 6 patients with different types of Fanconi syndrome. The effect of 1 alpha-OHVD3 proved to be 200-250 times as great as that of vitamin D2. From our results it was suggested that Ca malabsorption due to failure of 1 alpha-hydroxylation of vitamin D in the kidney, together with renal tubular acidosis and hypophosphatemia, is involved in the pathogenesis of the rickets of Fanconi syndrome.
范科尼综合征患者的血浆1,25-(OH)2VD3水平较低,但在给予1α-OHVD3后迅速升高。对6例不同类型范科尼综合征患者给予1α-OHVD3。结果证明,1α-OHVD3的作用比维生素D2大200 - 250倍。从我们的结果来看,提示由于肾脏中维生素D的1α-羟化功能障碍导致的钙吸收不良,连同肾小管酸中毒和低磷血症,参与了范科尼综合征佝偻病的发病机制。