Spindler A, Berman A, Mautalen C, Ubios J, Santini A E
Department of Internal Medicine, Hospital Padilla, College of Medicine, University of Tucumán, Argentina.
J Rheumatol. 1992 Apr;19(4):642-5.
We describe the case of a 38-year-old woman with 24 years of progressive skeletal deformities. Radiologic survey showed evidence of generalized skeletal involvement. She presented with secondary osteoarthritis, and her hands showed Heberden's and Bouchard's nodes. Light microscopic examination showed many reversed lines in the trabeculae and increased bone resorption and bone forming areas. On electron microscopic study there were intranuclear inclusions in the osteoclasts. To our knowledge, she is the oldest patient described in the English literature, and the first treated with (3-amino-1-hydroxypropilidene)-1,1 biphosphonate (Pamidronate). She showed a decrease in serum alkaline phosphatase, urinary hydroxyproline. Clinical variables showed good response to medication.
我们描述了一位患有24年进行性骨骼畸形的38岁女性病例。放射学检查显示有全身骨骼受累的证据。她患有继发性骨关节炎，双手可见赫伯登结节和布夏尔结节。光镜检查显示小梁中有许多反转线，骨吸收和骨形成区域增加。电子显微镜研究发现破骨细胞中有核内包涵体。据我们所知，她是英文文献中描述的年龄最大的患者，也是首例接受（3-氨基-1-羟丙基）-1,1-二膦酸盐（帕米膦酸盐）治疗的患者。她的血清碱性磷酸酶和尿羟脯氨酸水平下降。临床指标显示对药物治疗反应良好。
