在高氏病中对肺高血压的心声评估
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Updated: May 5, 2026

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Echocardiographic Assessment of the Right Heart in Mice
Published on: November 28, 2013
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D Elstein1, M W Klutstein, A Lahad
1Gaucher Clinic, Jerusalem, Israel.
Lancet (London, England)
|May 18, 1999
概括
对高氏病的酶疗法可能会增加肺高血压的风险. 研究人员发现患者中这种疾病的发生率为7%,建议对所有患有高氏病的人进行例行监测.
科学领域:
- 心脏病学 心脏病学
- 血液学 血液学 血液学
- 遗传学 是一个遗传学.
背景情况:
- 酶替代疗法 (ERT) 有效地管理高氏病的症状.
- 一部分接受ERT治疗的患者患有肺高血压 (PH).
- 在高氏病中PH的发生率需要进一步调查.
研究的目的:
- 为了确定高沙病患者肺高血压的频率.
- 评估ERT与PH发展之间的关系.
主要方法:
- 心声图被用来评估134名患有1型高氏病的成年人.
- 使用连续波多普勒测量了三管无能度 (TI) 梯度.
- 肺高血压被定义为一个TI梯度超过30mmHg.
主要成果:
- 9名患者 (7%) 被诊断出患有肺高血压.
- 所有PH患者正在接受治疗,其中6人先前接受了脊髓切除术.
- 胸部放射图证实了PH发现,并确定了TI梯度为25-29mmHg的患者的潜在PH.
结论:
- 在Gaucher病患者中观察到出乎意料的高PH率.
- 建议对所有接受治疗或未接受治疗的患者进行PH的常规心声监测.
- 如果TI梯度超过30 mm Hg,可能需要考虑退出ERT.
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