Angiotensin II blockade and aortic-root dilation in Marfan's syndrome
Angiotensin II blockade and aortic-root dilation in Marfan's syndrome
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DOI：
10.1056/nejmoa0706585
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发表时间：
2008-06-26
影响因子：
158.5
通讯作者：
Dietz, Harry C., III
中科院分区：
文献类型：
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作者：
Brooke, Benjamin S.;Habashi, Jennifer P.;Dietz, Harry C., III
背景资料：主动脉根部的进行性扩大，导致夹层，是马凡氏综合征患者过早死亡的主要原因。马凡氏综合征小鼠模型的最新数据表明，牙根扩大是由转化生长因子（β）（TGF-β）过度信号传导引起的，可通过TGF-β拮抗剂（包括血管紧张素II受体阻滞剂（ARB））治疗减轻。我们评估了临床反应ARBs在小儿马凡氏综合征患者谁有严重的arteric-root enlargement.Methods：我们确定了18个小儿马凡氏综合征患者已被随访12至47个月的治疗ARBs后，其他药物治疗未能防止进行性arteric-root enlargement。17例患者的ARB为氯沙坦，1例患者为厄贝沙坦。我们通过比较ARB治疗前后牙根直径的变化率来评价ARB治疗的疗效。结果：ARB治疗前后牙根直径的平均（+/-SD）变化率从以前药物治疗期间的3.54 ± 2.87 mm/年显著下降到ARB治疗期间的0.46 ± 0.62 mm/年（P
Background: Progressive enlargement of the aortic root, leading to dissection, is the main cause of premature death in patients with Marfan's syndrome. Recent data from mouse models of Marfan's syndrome suggest that aortic-root enlargement is caused by excessive signaling by transforming growth factor (beta) (TGF-(beta)) that can be mitigated by treatment with TGF-(beta) antagonists, including angiotensin II-receptor blockers (ARBs). We evaluated the clinical response to ARBs in pediatric patients with Marfan's syndrome who had severe aortic-root enlargement.Methods: We identified 18 pediatric patients with Marfan's syndrome who had been followed during 12 to 47 months of therapy with ARBs after other medical therapy had failed to prevent progressive aortic-root enlargement. The ARB was losartan in 17 patients and irbesartan in 1 patient. We evaluated the efficacy of ARB therapy by comparing the rates of change in aortic-root diameter before and after the initiation of treatment with ARBs.Results: The mean (+/-SD) rate of change in aortic-root diameter decreased significantly from 3.54+/-2.87 mm per year during previous medical therapy to 0.46+/-0.62 mm per year during ARB therapy (P