Department of Endocrinology and Metabolic Diseases, Leiden University Medical Center, Leiden, The Netherlands.
J Bone Miner Res. 2010 Dec;25(12):2796-800. doi: 10.1002/jbmr.151.
A 23-year-old man with the rare sclerosing bone disorder van Buchem disease presented with progressively worsening headaches that eventually became persistent and associated with papilledema. Increased intracranial pressure was diagnosed, and the patient had a ventriculoperitoneal drain inserted as well as simultaneously receiving treatment with prednisone. Before starting treatment, there was biochemical evidence for increased bone turnover and for steady increases in bone mineral density (BMD) at the spine and total hip despite the patient having reached his peak height of 197 cm at the age of 19 years. Treatment with prednisone for 2 years resulted in biochemical and histologic suppression of bone formation as well as of bone resorption and arrest of further bone accumulation. Our data suggest that glucocorticoids (GCs) may represent an attractive alternative to the high-risk surgical approaches used in the management of patients with progressive sclerosing bone disorders. Our findings also suggest that whereas sclerostin may not be required for the action of GCs on bone formation, it may well be important for the action of GCs on bone resorption. The exact mechanism by which sclerostin may be involved in the regulation of bone resorption is as yet to be explored.
一位 23 岁的男性患有罕见的硬化性骨病范布吕根病，逐渐出现头痛，并最终持续存在且伴有视盘水肿。诊断为颅内压升高，患者接受了脑室-腹腔分流术，并同时接受泼尼松治疗。在开始治疗前，有生化证据表明骨转换增加，尽管患者在 19 岁时已达到 197 厘米的身高峰值，但脊柱和全髋关节的骨密度（BMD）仍持续稳定增加。用泼尼松治疗 2 年后，骨形成、骨吸收以及进一步的骨积累都得到了生化和组织学抑制。我们的数据表明，糖皮质激素（GCs）可能是治疗进展性硬化性骨病患者的高风险手术方法的一种有吸引力的替代方法。我们的发现还表明，尽管硬化素可能不是 GCs 对骨形成作用所必需的，但它可能对 GCs 对骨吸收的作用很重要。硬化素如何参与骨吸收的调节，其确切机制仍有待探索。
