Kooh S W, Jones G, Reilly B J, Fraser D
J Pediatr. 1979 Jun;94(6):870-4. doi: 10.1016/s0022-3476(79)80204-8.
To investigate whether hepatobiliary rickets is caused by defective intestinal absorption of vitamin D or by impaired hepatic hydroxylation of the vitamin, we studied three children who developed severe rickets, hypocalcemia, and hypophosphatemia, two despite having received 400 to 800 IU vitamin D per day by mouth, and one despite prolonged treatment with 10,000 IU daily. On oral vitamin D therapy, plasma vitamin D and 25-hydroxyvitamin D levels were low. When two children were treated with weekly intravenous doses of 3,000 IU vitamin D to approximate the recommended prophylactic allowance, their plasma calcium and phosphate values improved promptly, the radiographic lesions healed, and the plasma concentrations of vitamin D and 25-hydroxyvitamin D became normal. Our studies indicate that the primary cause of hepatobiliary rickets is intestinal malabsorption of vitamin D, not impairment of the hepatic metabolism of the vitamin.
为了研究肝胆性佝偻病是由维生素D肠道吸收缺陷还是由维生素肝脏羟化受损引起，我们研究了三名患有严重佝偻病、低钙血症和低磷血症的儿童，其中两名儿童尽管每天口服400至800国际单位维生素D，另一名儿童尽管每天接受10,000国际单位的长期治疗。接受口服维生素D治疗时，血浆维生素D和25-羟基维生素D水平较低。当两名儿童接受每周静脉注射3000国际单位维生素D以接近推荐的预防剂量时，他们的血浆钙和磷值迅速改善，放射学病变愈合，维生素D和25-羟基维生素D的血浆浓度恢复正常。我们的研究表明，肝胆性佝偻病的主要原因是维生素D的肠道吸收不良，而非维生素肝脏代谢受损。
