Thivolet J, Perrot H, Meunier F, Bouchet B
Nouv Presse Med. 1975 Nov 15;4(39):2779-82.
Clotting factor XIII was used in 13 patients suffering from scleroderma, 17 with generalised scleroderma and 3 with localised scleroderma. The duration of treatment varied between 15 days and 6 months. The effect was marked in 7 cases and more moderate in 5 others. It took the form of increased suppleness of the skin, improved joint mobility and, to a lesser degree, an improvement in vasomotor disturbances of the extremities. Oesophageal abnormalities on X-ray were never altered. The return to normal of alveolar-capillary diffusion seen in two cases requires futher confirmation. The action of factor XIII in scleroderma may be explicable by the formation of transamidation bonds between the alpha chains of the collagen molecule, similar to those obtained on fibrin.
凝血因子 XIII 用于 13 例硬皮病患者,其中 17 例为全身性硬皮病,3 例为局限性硬皮病。治疗持续时间在 15 天至 6 个月之间。7 例效果显著,另外 5 例效果较为中等。表现为皮肤柔韧性增加、关节活动度改善,以及程度较轻的四肢血管舒缩功能障碍改善。X 线检查发现的食管异常从未改变。两例出现的肺泡 - 毛细血管弥散恢复正常情况尚需进一步证实。凝血因子 XIII 在硬皮病中的作用可能是由于在胶原分子的α链之间形成了转酰胺键,类似于在纤维蛋白上形成的键。