Liens D, Delmas P D, Meunier P J
Institut National de la Santé et de la Recherche Médicale (INSERM) Unit 403, Edouard Herriot Hospital, Lyon, France.
Lancet. 1994 Apr 16;343(8903):953-4. doi: 10.1016/s0140-6736(94)90069-8.
Nine patients with symptomatic and severe fibrous dysplasia were treated with intravenous pamidronate (60 mg per day over 3 days every sixth month), and were followed up for 18-48 months. The major effect was decreased bone pain (complete remission in 12 of 14 sites). Radiological changes were seen in four patients, with thickening of cortices, refilling of osteolytic lesions, or both. The initial increased bone remodelling was reduced, as shown by decrease of raised serum alkaline phosphatase and urinary hydroxyproline. The treatment was well tolerated, but a 13-year-old patient showed widening of knee growth-plates which is consistent with a transient mineralisation defect.
9例有症状的重度骨纤维发育不良患者接受了静脉注射帕米膦酸盐治疗（每6个月，连续3天，每天60毫克），并随访18至48个月。主要疗效为骨痛减轻（14个部位中的12个完全缓解）。4例患者出现影像学改变，表现为皮质增厚、溶骨性病变重新填充或两者皆有。血清碱性磷酸酶和尿羟脯氨酸升高有所下降，表明最初增加的骨重塑有所减少。该治疗耐受性良好，但一名13岁患者出现膝关节生长板增宽，这与短暂性矿化缺陷一致。
