Cohen R B, Hahn G V, Tabas J A, Peeper J, Levitz C L, Sando A, Sando N, Zasloff M, Kaplan F S
Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania, Philadelphia 19104.
J Bone Joint Surg Am. 1993 Feb;75(2):215-9. doi: 10.2106/00004623-199302000-00008.
Forty-four patients who had fibrodysplasia ossificans progressiva responded by mail to a questionnaire regarding the age at the onset of heterotopic ossification at fifteen commonly involved anatomical sites. The average age of the patients when they responded to the questionnaire was twenty-seven years (range, three to sixty-nine years). The average age at the onset of ossification was five years (range, birth to twenty-five years). The most common sites of early heterotopic ossification were the neck, spine, and shoulder girdle. Thirty-five (80 per cent) of the patients had had some restrictive heterotopic ossification by the age of seven years. By the age of fifteen years, forty-two (more than 95 per cent) of the patients had severely restricted mobility of the upper limbs. In these patients, heterotopic ossification proceeded in a direction that was axial to appendicular, cranial to caudad, and proximal to distal; this pattern appeared typical for fibrodysplasia ossificans progressiva.
44例进行性骨化性纤维发育不良患者通过邮件回复了一份关于15个常见受累解剖部位异位骨化发病年龄的问卷。患者回复问卷时的平均年龄为27岁（范围为3至69岁）。骨化开始的平均年龄为5岁（范围为出生至25岁）。早期异位骨化最常见的部位是颈部、脊柱和肩胛带。35例（80%）患者在7岁时就出现了一些限制性异位骨化。到15岁时，42例（超过95%）患者上肢活动严重受限。在这些患者中，异位骨化的发展方向是从躯干到四肢、从头部到尾部、从近端到远端；这种模式在进行性骨化性纤维发育不良中似乎是典型的。
