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1962~1986年,在 Naffield 矫形外科中心的1351例血友病患者中,81例是因伴有周围神经损伤住院。54例(全部为男性)中共有神经损害88处,对其中39例(61处病损)进行了随访(4月~18年,平均8.4年),以寻找导致神经损伤及影响其功能恢复的因素。这些病例中,44例为Ⅷ因子缺乏的甲型血友病,9例为Ⅸ因子缺乏的乙型血友病,1例为伴高水平循环抗体的获得性血友病。其中1例Ⅷ因子浓度在2%,另1例为13%,而其余病例(包括Ⅷ因子和Ⅸ因子缺乏者)的血中因子浓度均低于1%。除了上述的1例获得性血友病患者外,还有16例(36%)Ⅷ因子缺乏病人的血中见该因子的抗体滴度升高。作者发现本文病例神经病变的最常见原因为肌肉内出血,导致神经受压,而无神经内出血的证据。神经病变以股神经麻痹最常见。作者对Ⅷ因子抗体在神经恢复中的意义进行了研究。结果发现:16例有抗
From 1962 to 1986, out of 1351 hemophiliacs in the Naffield Orthopedic Center, 81 were hospitalized with peripheral nerve injury. A total of 88 neurological lesions were found in 54 (all men) and 39 of them (61 lesions) were followed up (April to 18 years, mean 8.4 years) in search of neurological damage and their functional recovery factor. Of these cases, 44 were Hemophilia A lacking Factor VIII, 9 were Hemophilia H deficient in Factor IX and 1 was acquired hemophilia with high circulating antibodies. One of them had a concentration of 2% of factor Ⅷ and 13% of the other, while blood levels of other cases (including factor Ⅷ and factor Ⅸ) were below 1%. In addition to the above 1 case of acquired hemophilia, 16 (36%) patients with a factor VIII deficiency had elevated antibody titers of this factor. The authors found that the most common cause of neuropathy in this case was intramuscular bleeding, leading to nerve compression without evidence of neurological hemorrhage. Neuropathy is the most common nerve palsy. The author of Ⅷ factor antibodies in the recovery of the significance of the study. The results showed that: 16 cases were resistant