The Effects Of Bronchodilator Therapy On Respiratory And Autonomic Function In Patients With Familial Dysautonomia
Status:
Completed
Trial end date:
2014-12-01
Target enrollment:
Participant gender:
Summary
Evaluate the effects of bronchodilator therapy on respiratory function. Our overall goal is
to determine whether, in patients with familial dysautonomia (FD), there is a component of
airway obstruction that is reversible. To this end, we will evaluate airway resistance before
and after receiving the anti-cholinergic ipratropium (Atrovent ®) and the beta-2-agonist
albuterol (ProVentil®/Ventolin®). We predict that the response to either drug will depend on
the underlying level of sympathetic and parasympathetic activity and airway tone. We will
then determine the cardiovascular effects of inhaled ipratropium and albuterol in patients
with FD. Because patients with FD have fewer sympathetic neurons and denervation
supersenstivity, we predict that following albuterol inhalation, there will be non-selective
activation of alpha-1-adrenergic receptors. Furthermore, because of a congenital defect in
the afferent baroreceptor neurons that sense blood pressure, we suspect that the resulting
vasoconstriction will be unopposed leading to a pressor effect. We hypothesize that
inhalation of the anti-cholinergic ipratopium will produce little rise in heart rate, due to
the extent of parasympathetic denervation to the heart.
Phase:
Phase 3
Details
Lead Sponsor:
New York University School of Medicine NYU Langone Health