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遗传性血色素沉着症铁耗竭后铁再蓄积的速率。对静脉切开术治疗的意义。

Rate of iron reaccumulation following iron depletion in hereditary hemochromatosis. Implications for venesection therapy.

作者信息

Adams P C, Kertesz A E, Valberg L S

机构信息

Department of Medicine, University Hospital, University of Western Ontario, London, Canada.

出版信息

J Clin Gastroenterol. 1993 Apr;16(3):207-10. doi: 10.1097/00004836-199304000-00008.

Abstract

Although venesection therapy is well established for the initial depletion of iron stores in hereditary hemochromatosis, the frequency of subsequent therapy has not been clearly defined. In this study, 21 homozygotes (16 male, five female; mean age of 58, with a range of 26 to 77 years) who had completed initial venesection therapy were followed without further venesections for a mean of 4.0 years (range of 1 to 10.4 years) with iron reaccumulation assessed by annual serum ferritin concentration. Over the follow-up period, the mean rise in serum ferritin was 99 (micrograms/l)/year (range of 1.2 to 241 micrograms/l). The mean interval for the ferritin to become elevated above the normal range in 10 patients was 3.8 years. Eleven of 21 patients required no further venesection therapy over the follow-up interval. There was no significant correlation between the annual rate of ferritin increase and the age or amount of iron removed by prior venesections. These data demonstrate that monitoring body iron stores annually and the selective use of venesections if iron stores reaccumulate is a safe alternative to lifelong venesections every 2-4 months. Many homozygotes will not require reinitiation of venesection therapy for > 4 years. Annual monitoring of body iron stores with reinstitution of weekly venesection when the serum ferritin exceeds the upper limit of normal was a safe alternative to long-term maintenance venesection.

摘要

尽管放血疗法在遗传性血色素沉着症中铁储备的初始耗竭方面已得到充分确立,但后续治疗的频率尚未明确界定。在本研究中,对21名完成初始放血疗法的纯合子(16名男性,5名女性;平均年龄58岁,范围为26至77岁)进行了随访,未进行进一步放血,平均随访4.0年(范围为1至10.4年),通过每年的血清铁蛋白浓度评估铁的重新蓄积情况。在随访期间,血清铁蛋白的平均年上升幅度为99(微克/升)/年(范围为1.2至241微克/升)。10名患者的铁蛋白升高至正常范围以上的平均间隔时间为3.8年。21名患者中有11名在随访期间无需进一步的放血治疗。铁蛋白年增长率与年龄或先前放血去除的铁量之间无显著相关性。这些数据表明,每年监测体内铁储备,并在铁储备重新蓄积时选择性地使用放血疗法,是每2 - 4个月进行终身放血疗法的一种安全替代方案。许多纯合子在超过4年的时间里不需要重新开始放血治疗。每年监测体内铁储备,当血清铁蛋白超过正常上限时重新开始每周放血,是长期维持放血疗法的一种安全替代方案。

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