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Heart:特纳综合征的冠脉解剖vs单纯二叶主动脉瓣

2018-10-28 xiangting MedSci原创

TS-BAV和单纯BAV患者中无中缝RL BAV左侧优势的均等分布表明左侧优势的存在是无中缝BAVs的特征,与TS无关。

在特纳综合征(TS)和二叶主动脉瓣(BAV)患者中已经描述了冠脉解剖存在变异,如左主干和左冠脉系统缺失。目前尚不清楚TS的冠脉变异是否与BAV和特定的BAV亚型有关。

这项研究旨在比较伴和不伴BAV的TS患者vs单纯BAV患者的冠脉解剖,并研究这些组中的BAV形态学亚型。

使用CT对86例TS患者(20例TS-BAV,66例TS-三叶主动脉瓣)和86例单纯BAV患者进行冠脉解剖和BAV形态学研究(37±13岁vs 42±15岁)。

伴和不伴BAV的TS患者冠脉优势无显著差异(25% vs 21%,p=0.933)。无中缝左右冠脉瓣(RL BAV)融合的BAVs显示TS-BAV和单纯BAV中左冠脉优势的患病率高(均为38%)。与单纯BAV相比,TS-BAV中的左主干缺失更常见(10%vs 0%)。所有左主干缺失的TS-BAV患者都有无中缝的RL BAV。

TS-BAV和单纯BAV患者中无中缝RL BAV左侧优势的均等分布表明左侧优势的存在是无中缝BAVs的特征,与TS无关。无中缝的TS和RL BAV与左主干缺失独立相关。认识到左主干缺失的发病率较高对于避免低温灌注期间的并发症很重要。

原始出处:

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    2018-10-30 zhaojie88
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    2018-10-30 slcumt
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由此可见,目前使用生长激素和雌激素治疗的TS患儿并不会增加骨质疏松性骨折的风险。TS患者较低的BMD可通过增加骨骼半径来抵消,使四肢骨骼得到正常的骨强度。

2016 临床实践指南:特纳综合征患者的管理

2016年在辛辛那提国际特纳综合征会议上发布了特纳综合征管理指南,该指南的主要内容涉及特纳综合征的诊断和基因诊断,患者成长和青春期管理,生育能力、辅助生殖技术和妊娠,特纳综合征患者心血管健康问题,从儿科到成人管理过渡,健康和并发症监测,神经认识和行为方面等。

生长激素,几多欢喜几多愁

激素对机体有重要作用。它们参与调节包括生长发育在内的几乎所有主要的身体活动。如果机体不能产生足够的激素,则需要注射生长激素。 人类生长激素(HGH)影响机体的身高,参与构建身体的骨骼和肌肉。 HGH对儿童的生长发育至关重要。此外,它有助于增加蛋白质和脂肪分解以提供组织生长所需的能量。活动水平可影响生长激素的水平,运动、睡眠、压力和较低的血糖水平会导致HGH水平上升。 HGH水平显著影响身体的变化。

JCEM:SHOX缺乏患者生长激素治疗有效

矮小同源盒基因(SHOX)缺失和特纳综合征的生长障碍有相似的病因。因为在特纳综合征患者生长激素(GH)治疗对身高有确定的疗效,假释生长激素治疗在SHOX缺乏患者也可以刺激生长。为了评估在SHOX缺乏的身材矮小患者,生长激素治疗的长期疗效,并比较生长激素对SHOX缺乏患者和特纳综合征患者最终(成年)身高(FH)的影响。来自美国宾夕法尼亚州托马斯杰弗逊大学的Werner F Blum教授及其团队进行了

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