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首页> 外文期刊>Seminars in Thrombosis and Hemostasis >Vascular complications of severe hyperhomocysteinemia in patients with homocystinuria due to cystathionine beta-synthase deficiency: effects of homocysteine-lowering therapy.
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Vascular complications of severe hyperhomocysteinemia in patients with homocystinuria due to cystathionine beta-synthase deficiency: effects of homocysteine-lowering therapy.

机译：同型半胱氨酸尿症患者由于胱硫醚β-合酶缺乏症引起的严重高同型半胱氨酸血症的血管并发症：降低同型半胱氨酸治疗的效果。

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摘要

Homocystinuria (HCU) due to cystathionine beta-synthase (CBS) deficiency leads to severe hyperhomocysteinemia (HHcy). Vascular events (VE) remain the major cause of morbidity and mortality in the untreated patients with HCU. The study on the natural history of untreated HCU disclosed that, at the time of maximal risk, in other words beyond 10 years old, there was one event per 25 years. Recent studies from Australia (n = 32), The Netherlands (n = 28), and Ireland (n = 24) have documented the effects of long-term treatment on the vascular outcome of a total of 84 patients with 1314 patient-years of treatment for HCU. The mean (range) age was 27.8 (2.5 to 70) years. Five VE were recorded during treatment; one pulmonary embolism, two myocardial infarctions, and two abdominal aneurysms. All five VE occurred in B6-responsive patients at a mean (range) age of 48.8 (30 to 60) years. In 1314 patient-years of treatment, 53 VE would have been expected if they remained untreated; instead only 5 were documented, relative risk = 0.091 (95% confidence interval [CI] 0.043 to 0.190; p < 0.001). Appropriate homocysteine-lowering therapy for severe HHcy significantly reduced the vascular risk in patients with HCU. VE were rare with treatment despite the fact that the post-treatment homocysteine levels were several times higher than the cutoff point for homocysteine in the normal population. The present findings may have relevance to the current concept of "mild HHcy" as a risk factor for vascular disease, with elevated plasma homocysteine levels considerably lower than that of the post-treatment levels in this group of reported patients.
机译：由于胱硫醚β-合酶（CBS）缺乏引起的高半胱氨酸尿症（HCU）导致严重的高同型半胱氨酸血症（HHcy）。在未经治疗的HCU患者中，血管事件（VE）仍然是发病率和死亡率的主要原因。对未经治疗的HCU的自然病史的研究表明，在最大风险时，换句话说，超过10岁的人，每25年发生一次事件。澳大利亚（n = 32），荷兰（n = 28）和爱尔兰（n = 24）的最新研究表明，长期治疗对总共84例患者的血管结局有影响，其中1314病人年。 HCU的治疗。平均（范围）年龄为27.8（2.5到70）岁。治疗期间记​​录了五次VE; 1例肺栓塞，2例心肌梗塞和2例腹部动脉瘤。所有五种VE均发生在B6反应性患者中，平均（范围）年龄为48.8（30至60）岁。在1314个患者年的治疗年中，如果不进行治疗，预计会达到53 VE。相反，仅记录了5个，相对风险= 0.091（95％置信区间[CI] 0.043至0.190; p <0.001）。对重症HHcy适当降低同型半胱氨酸疗法可显着降低HCU患者的血管风险。尽管在正常人群中，治疗后的同型半胱氨酸水平比同型半胱氨酸的临界点高出几倍，但在治疗中很少见到VE。目前的发现可能与“轻度HHcy”作为血管疾病的危险因素的当前概念有关，血浆高半胱氨酸水平的升高远低于该组报道患者的治疗后水平。

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