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Q J Med 2002; 95: 159-163
© 2002 Association of Physicians

Abnormal endothelin B receptor vasomotor responses in patients with Hirschsprung's disease

D.E. Newby, F.E. Strachan and D.J. Webb

From the Clinical Pharmacology Unit and Research Centre, University of Edinburgh, Edinburgh, UK

Received 2 November 2001 and in revised form 6 December 2001

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.

Address correspondence to Dr D.E. Newby, Cardiovascular Research, Department of Cardiology, Royal Infirmary, Lauriston Place, Edinburgh EH3 9YW. e-mail: d.e.newby{at}ed.ac.uk


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