Coe J D, Murphy W A, Whyte M P
J Bone Joint Surg Am. 1986 Sep;68(7):981-90.
Ten pseudofractures and six complete fractures occurred in five patients with adult hypophosphatasia. The four pseudofractures that were treated non-operatively progressed to complete fracture. The six pseudofractures and six complete fractures that were treated by internal fixation united. New fractures occurred in four patients after the removal of the internal fixation. We recommend that complete fractures, symptomatic pseudofractures, and progressive asymptomatic pseudofractures in patients with adult hypophosphatasia be treated with load-sharing internal-fixation devices, such as rigid intramedullary nails. These should not be removed unless it is absolutely essential. If removal is necessary, however, they should be replaced, preferably during the same anesthetic session. In our opinion, the guidelines developed from this study may be applied reasonably to patients with osteomalacia from causes other than hypophosphatasia, especially if medical therapy has been unsuccessful.
5例成人低磷酸酯酶症患者发生了10处假性骨折和6处完全骨折。4例接受非手术治疗的假性骨折进展为完全骨折。6例假性骨折和6处完全骨折接受内固定治疗后愈合。4例患者在取出内固定后发生了新的骨折。我们建议，成人低磷酸酯酶症患者的完全骨折、有症状的假性骨折和进行性无症状假性骨折，应采用分担负荷的内固定装置治疗，如坚固的髓内钉。除非绝对必要，否则不应取出这些内固定装置。然而，如果有必要取出，应进行更换，最好在同一次麻醉过程中进行。我们认为，本研究制定的指南可合理应用于低磷酸酯酶症以外其他原因导致的骨软化症患者，尤其是在药物治疗失败的情况下。
