el-Labban N G, Hopper C, Barber P
Institute of Dental Surgery, Eastman Dental Hospital, London, England.
J Oral Pathol Med. 1995 Mar;24(3):125-9. doi: 10.1111/j.1600-0714.1995.tb01152.x.
The masseter and temporalis muscles were investigated ultrastructurally in 2 patients having fibrodysplasia ossificans progressiva (FOP). Results showed atrophy and necrosis of muscle fibres. Both cases contained cartilage which was calcified in the second case and was associated with degenerating chondrocytes. The blood vessels showed various changes, with haematoma formation and large fibrin deposits. Some of the fibrin deposits seen in the second case were surrounded with cartilage. This suggests that the cartilage probably forms as a reaction to an old haemorrhage. It is possible that the effect of the mutant gene found in FOP is on blood vessels rather than the normal regulatory mechanisms of the inducible osteogenic precursor cells. These degenerating blood vessels often result in haemorrhage and fibrin deposits; the fibrosis, cartilage or bone formation are probably secondary changes. Amianthoid fibres found in the tendon of the temporalis muscles of the second case, together with the other changes of fibrosis and cartilage formation, account for the severe restricted mouth opening suffered by these patients.
对2例进行性骨化性纤维发育不良（FOP）患者的咬肌和颞肌进行了超微结构研究。结果显示肌纤维萎缩和坏死。两例均有软骨，第二例软骨发生钙化，并伴有软骨细胞退变。血管呈现各种变化，有血肿形成和大量纤维蛋白沉积。在第二例中可见一些纤维蛋白沉积物被软骨包围。这表明软骨可能是对陈旧性出血的一种反应形成的。FOP中发现的突变基因的作用可能是针对血管，而非诱导性成骨前体细胞的正常调节机制。这些退变的血管常导致出血和纤维蛋白沉积；纤维化、软骨或骨形成可能是继发性变化。第二例患者颞肌肌腱中发现的石棉样纤维，连同纤维化和软骨形成的其他变化，解释了这些患者严重的张口受限。
