Long R G, Varghese Z, Meinhard E A, Skinner R K, Wills M R, Sherlock S
Br Med J. 1978 Jan 14;1(6105):75-7. doi: 10.1136/bmj.1.6105.75.
Despite regular long-term parenteral vitamin D2 treatment, four patients with biliary cirrhosis had multiple symptoms of bone disease and bone biopsy specimens showed osteomalacia without osteoporosis. Three patients also had a proximal myopathy. Plasma calcium values (after correction for albumin), phosphorus, magnesium, and serum 25-hydroxy-vitamin D were within normal limits. Treatment with 1,25-dihydroxy-cholecalciferol (1,25-(OH)2D3) relieved symptoms in three of the four patients and improved those in the fourth. Histological examination of bone showed improvement in all four patients, but serum and urinary biochemical changes were not pronounced. We conclude that 1,25-(0H)2D3 treatment has a beneficial effect on bone and muscle in hepatic osteomalacia, either because vitamin D 1-hydroxylation fails in biliary cirrhosis or because hepatic osteomalacia is resistant to vitamin D2 metabolites.
尽管长期定期接受肠外维生素D2治疗,但4例胆汁性肝硬化患者仍有多种骨病症状,骨活检标本显示为骨软化症而非骨质疏松症。3例患者还患有近端肌病。血浆钙值(校正白蛋白后)、磷、镁以及血清25-羟基维生素D均在正常范围内。用1,25-二羟胆钙化醇(1,25-(OH)2D3)治疗使4例患者中的3例症状得到缓解,第4例患者症状有所改善。骨组织学检查显示所有4例患者均有改善,但血清和尿液生化变化不明显。我们得出结论,1,25-(OH)2D3治疗对肝性骨软化症的骨骼和肌肉有有益作用,这可能是因为胆汁性肝硬化中维生素D 1-羟化作用失败,或者是因为肝性骨软化症对维生素D2代谢产物有抵抗性。