Abnormal endothelin B receptor vasomotor responses in patients with Hirschsprung's disease.
Abnormal endothelin B receptor vasomotor responses in patients with Hirschsprung's disease.
复制标题
先天性巨结肠患者内皮素 B 受体血管舒缩反应异常。
DOI：
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复制
发表时间：
2002
期刊：
影响因子：
--
通讯作者：
D. Webb
中科院分区：
文献类型：
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作者：
D. Newby;F. Strachan;D. Webb
背景
先天性巨结肠与内皮素-3和内皮素B受体基因缺陷有关。
目的
目的：评价先天性巨结肠患者体内血管对内皮素B受体刺激的反应。
方法
前臂血流量测定使用静脉闭塞体积描记法在10例先天性巨结肠病和10名匹配的健康对照组在肱动脉内输注的高选择性内皮素B受体激动剂，sarafotoxin S6 c。为了模拟内皮素B受体功能障碍，将sarafotoxin S6 c与高选择性内皮素B受体拮抗剂BQ-788共同输注到6名健康对照中。
结果
Sarafotoxin S6 c引起短暂的初始血管舒张，随后是缓慢发作的持续血管收缩（p<0.001）。与对照组相比，先天性巨结肠患者的初始血管舒张功能明显受损，而随后的血管收缩更为明显（p<0.001）。在健康对照组中，BQ-788和sarafotoxin S6 c的共输注引起了与先天性巨结肠患者相似的反应模式：消除了最初的血管舒张，增强了随后的血管收缩（p<0.001）。
讨论
在大多数先天性巨结肠患者中，存在血管内皮素B受体的功能缺陷。
BACKGROUND
Hirschsprung's disease is associated with defects in the endothelin-3 and endothelin B receptor genes.
AIM
To assess the in vivo vasomotor responses to endothelin B receptor stimulation in patients with Hirschsprung's disease.
METHODS
Forearm blood flow was measured using venous occlusion plethysmography in 10 patients with Hirschsprung's disease and 10 matched healthy controls during intra-brachial infusion of the highly selective endothelin B receptor agonist, sarafotoxin S6c. To simulate endothelin B receptor dysfunction, sarafotoxin S6c was co-infused with the highly selective endothelin B receptor antagonist, BQ-788, in six of the healthy controls.
RESULTS
Sarafotoxin S6c caused a brief initial vasodilatation followed by a slow-onset, sustained vasoconstriction (p<0.001). Compared to control subjects, patients with Hirschsprung's disease had a substantial impairment of the initial vasodilatation whilst producing a more pronounced subsequent vasoconstriction (p<0.001). In healthy controls, co-infusion of BQ-788 and sarafotoxin S6c caused a similar pattern of responses to those obtained in patients with Hirschsprung's disease: abolition of the initial vasodilatation and augmentation of subsequent vasoconstriction (p<0.001).
DISCUSSION
In the majority of patients with Hirschsprung's disease, there is a functional defect of the vascular endothelin B receptor.
先天性巨结肠中内皮素受体介导的信号传导。
DOI：
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发表时间：
1996
期刊：
Human molecular genetics.
影响因子：
--
作者：
Chakravarti,A
通讯作者：
Chakravarti,A