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门腔分流术治疗家族性杂合子高胆固醇血症。

Portacaval shunt for familial heterozygous hypercholesterolemia.

作者信息

Madras P N

出版信息

Surg Gynecol Obstet. 1981 Feb;152(2):187-90.

PMID:7209760
Abstract

Two patients with heterozygous familial type 2 hypercholesterolemia are reported upon following treatment with end-to-side portacaval shunting. In both, the portacaval shunt decreased the cholesterol level by about 40 per cent. No adverse effects of the operation could be seen in either patient at 30 months, and no return of cardiovascular disease is evident in either patient. In one patient with large xanthelasmas, the lesions became a much duller yellow color and regressed from an elevated to flat appearance. However, the margins of the lesions did not change significantly. In young patients with heterozygous type 2 hypercholesterolemia in whom cardiovascular complications pose a major threat to life, a portacaval shunt is an effective means of reducing the cholesterol level. The benefits to the patient outweight the risks of operation. In one patient, an anxiety reaction developed which her psychiatrist related to a stressful situation. There were no clinical signs of encephalopathy, no response to protein restriction and a minimal elevation of the serum ammonia level, 86 versus 70 micrograms per cent as the upper limit of normal.

摘要

报告了两例杂合子家族性2型高胆固醇血症患者接受端侧门腔分流术后的情况。在这两例患者中,门腔分流使胆固醇水平降低了约40%。在30个月时,两名患者均未出现手术的不良反应,且均未出现心血管疾病复发。在一名患有大型睑黄瘤的患者中,病变颜色变得明显更暗淡,从隆起变为扁平状。然而,病变边缘没有明显变化。在心血管并发症对生命构成重大威胁的杂合子2型高胆固醇血症年轻患者中,门腔分流是降低胆固醇水平的有效方法。对患者的益处大于手术风险。在一名患者中出现了焦虑反应,其精神科医生认为这与压力状况有关。没有脑病的临床体征,对蛋白质限制无反应,血清氨水平略有升高,正常上限为每毫升86微克,而该患者为70微克。

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Portacaval shunt for familial heterozygous hypercholesterolemia.门腔分流术治疗家族性杂合子高胆固醇血症。
Surg Gynecol Obstet. 1981 Feb;152(2):187-90.
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[Pathogenetic bases and surgical alternatives in the treatment of familial hypercholesterolemia].[家族性高胆固醇血症治疗中的发病机制基础与手术选择]
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Portacaval shunt surgery and liver transplantation in the treatment of homozygous familial hypercholesterolemia.门腔分流术和肝移植治疗纯合子家族性高胆固醇血症。
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引用本文的文献

1
Hepatocyte ultrastructure in rats with portacaval shunt. A morphometric study of acinar zones.门腔分流大鼠的肝细胞超微结构。腺泡区的形态计量学研究。
Dig Dis Sci. 1982 Nov;27(11):1003-10. doi: 10.1007/BF01391746.
2
Portacaval shunt in patients with familial hypercholesterolemia.家族性高胆固醇血症患者的门腔分流术。
Ann Surg. 1983 Sep;198(3):273-83. doi: 10.1097/00000658-198309000-00004.
3
The Eck fistula in animals and humans.动物和人类的艾克瘘管。
Curr Probl Surg. 1983 Nov;20(11):687-752. doi: 10.1016/s0011-3840(83)80010-0.