REPAIR OF ELBOW DEFECTS AND THE BIOCHEMICAL CHARACTERISTICS OF WERNERS SYNDROME

REPAIR OF ELBOW DEFECTS AND THE BIOCHEMICAL CHARACTERISTICS OF WERNERS SYNDROME
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DOI:
10.1097/00000637-198910000-00017
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发表时间:
1989-10-01
影响因子:
1.5
通讯作者:
SOEDA, S
SOEDA, S
中科院分区:
医学4区
文献类型:
--
作者:
KOSHIMA, I;SHOZIMA, M;SOEDA, S

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沃纳综合征是一种罕见的常染色体隐性遗传疾病,伴有加速衰老的特征。我们报告2例肘关节缺损,分别以肌瓣或远端尺侧返动脉皮瓣修复。对活检皮肤的蛋白质分析显示,分子量在40,000至60,000范围内的异常蛋白质显著增加。在患者血清中也检测到异常低分子量蛋白,但在尿液中未观察到异常蛋白。提示Werner氏营养性溃疡可用普通皮瓣修复,并提示Werner氏皮肤及血清中有异常蛋白产生。
Werner's syndrome is a rare condition of autosomal recessive inheritance associated with features of accelerated aging. We describe 2 patients whose elbow defects were repaired with muscle flap or distally based ulnar recurrent artery flap. Protein analysis for the biopsied skin showed a remarkable increase of abnormal proteins in the range from 40,000 to 60,000 molecular weight. An abnormally low-molecular-weight protein in serum was also detected in the patient, but no abnormal proteins were seen in the urine. It is suggested that Werner's trophic ulcer can be repaired by ordinary skin flap, and we conclude that some abnormal proteins are produced in the Werner's skin and serum.