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

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

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.

Original languageEnglish
Pages (from-to)159-63
Number of pages5
JournalQJM: An International Journal of Medicine
Issue number3
Publication statusPublished - 1 Mar 2002

Keywords / Materials (for Non-textual outputs)

  • Adult
  • Endothelin Receptor Antagonists
  • Female
  • Forearm
  • Hirschsprung Disease
  • Humans
  • Male
  • Oligopeptides
  • Piperidines
  • Receptor, Endothelin B
  • Receptors, Endothelin
  • Regional Blood Flow
  • Vasoconstrictor Agents
  • Vasomotor System
  • Viper Venoms


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