Cohen I J, Kornreich L, Mekhmandarov S, Katz K, Zaizov R
Schneider Children's Medical Centre of Israel, Petah Tiqva, Israel.
Arch Dis Child. 1996 Sep;75(3):218-22. doi: 10.1136/adc.75.3.218.
In type I Gaucher's disease, episodes of severe disabling bone pain, the so called bone crises, may be resistant to all analgesics, including narcotics. The demonstration of subperiosteal oedema on magnetic resonance imaging (MRI) led to an attempt to use steroids to relieve the oedema and thereby the pain. On eight occasions, five patients with documented bone crises received conventional dose steroids (20 mg/m2/day) with considerable shortening of the attacks. On six occasions five further patients received high dose methylprednisolone (30 mg/kg intravenously or 1 g/m2 orally daily for two days), which was followed by oral prednisone for three to five days on the last four occasions. In this later group, pain relief was evident within several hours. Three treatments were given on an ambulatory basis. The MRI scan of one of these patients showed no subperiosteal fluid collection five days after high dose steroids had been started, and on subsequent x ray examination, there was no periosteal elevation. This treatment should be considered in cases of Gaucher's disease with bone crises.
在I型戈谢病中，严重致残性骨痛发作，即所谓的骨危象，可能对包括麻醉药在内的所有镇痛药均无反应。磁共振成像（MRI）显示的骨膜下水肿促使人们尝试使用类固醇来减轻水肿，从而缓解疼痛。有8次，5例确诊为骨危象的患者接受了常规剂量的类固醇治疗（20mg/m²/天），发作时间显著缩短。另有6次，5例患者接受了大剂量甲泼尼龙治疗（静脉注射30mg/kg或口服1g/m²，连续两天），后4次在随后的3至5天口服泼尼松。在这组后期患者中，数小时内疼痛即明显缓解。3次治疗是在门诊进行的。其中1例患者在开始使用大剂量类固醇5天后，MRI扫描显示骨膜下无液体积聚，随后的X线检查也未发现骨膜抬高。对于患有骨危象的戈谢病患者，应考虑这种治疗方法。
