Ignite Creation Date:
2024-05-11 @ 8:31 AM
Last Modification Date:
2024-10-26 @ 3:29 PM
Study NCT ID:
NCT06408805
Status:
COMPLETED
Last Update Posted:
2024-05-10
First Post:
2024-05-07
Brief Title:
Autonomic Nervous System Profile in Hereditary Angioedema
Sponsor:
Istituti Clinici Scientifici Maugeri SpA
Organization:
Istituti Clinici Scientifici Maugeri SpA
Study Overview
Official Title:
Hereditary Angioedema Due to C1 Inhibitor Deficiency Evaluation of the Cardiac Autonomic Nervous System Profile in Patients With and Without Prophylactic Treatment
Status:
COMPLETED
Status Verified Date:
2024-05
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
ANS-HAE
Brief Summary:
The goal of this study is to compare the autonomic nervous system control of patients with hereditary angioedema to healthy individuals
The main questions it aims to answer are
Are there differences in the autonomic nervous system control between patients with hereditary angioedema and healthy individuals during short-term resting period and during orthostatic challenges
Are there differences in the autonomic nervous system control recorded over long-term periods ie 24 hours
The main questions it aims to answer are
Are there differences in the autonomic nervous system control between patients with hereditary angioedema and healthy individuals during short-term resting period and during orthostatic challenges
Are there differences in the autonomic nervous system control recorded over long-term periods ie 24 hours
Detailed Description:
Angioedema AE without wheals is a localized self-limiting edema associated with different mechanisms The best-known form is hereditary angioedema HAE due to C1 inhibitor deficiency C1-INH a rare disease with a prevalence of 165000 in Italy Symptoms include swelling of the extremities genitals bowel mucosa face and upper airway including the larynx Laryngeal attacks if not treated can lead to death AE attacks are unpredictable and occur episodically upon release of the main mediator of the attack namely bradykinin resulting from hyperactivation of the contact system lacking its main control protein C1-INH
The overall result is an impairment of the endothelial function associated with increased vascular permeability The release of bradykinin occurs locally in an unpredictable way at times facilitated by trauma and different triggers such as stress
In C1-INH-HAE patients the autonomic nervous system ANS has a role in the regulation of vascular permeability for example via the baroreflex mechanism It is also known that sympathetic nervous system inhibition by the α2 agonist clonidine reduces microvascular permeability in endotoxemic animals suggesting that antagonizing the sympathetic nervous system might prove beneficial in stabilizing capillary leakage during inflammation Similarly the vagus nerve has a protective role in models of inflammation such as ischemia-reperfusion injury The parasympathetic tone acting on the B2-receptors in the nucleus ambiguous can also be modulated by bradykinin The ANS could be investigated using the tilt test and Holter ECG monitoring in current clinical practice when its impairment is suspected Power spectral analysis of heart rate variability HRV ie the analysis of the spontaneous fluctuations of the heart period is exploited to analyze data collected during the tilt test and Holter-ECG deriving indices of the autonomic control
In C1-INH-HAE patients the cardiac ANS was investigated during a remission period during the AE attack and its prodromal phase
It has been demonstrated that HRV analysis extended to multiday ECG recordings may furnish an early marker of an angioedema attack
In this perspective the identification of markers like HRV parameters could play a crucial role in helping patients and physicians to characterize the ANS control in each patient and individualize treatment based on the ANS balance
This study aims to compare the cardiovascular ANS control in healthy subjects and HAE patients by head-up tilt test monitoring and evaluate the differences in long-term ANS between healthy controls and HAE patients by Holter ECG monitoring
The overall result is an impairment of the endothelial function associated with increased vascular permeability The release of bradykinin occurs locally in an unpredictable way at times facilitated by trauma and different triggers such as stress
In C1-INH-HAE patients the autonomic nervous system ANS has a role in the regulation of vascular permeability for example via the baroreflex mechanism It is also known that sympathetic nervous system inhibition by the α2 agonist clonidine reduces microvascular permeability in endotoxemic animals suggesting that antagonizing the sympathetic nervous system might prove beneficial in stabilizing capillary leakage during inflammation Similarly the vagus nerve has a protective role in models of inflammation such as ischemia-reperfusion injury The parasympathetic tone acting on the B2-receptors in the nucleus ambiguous can also be modulated by bradykinin The ANS could be investigated using the tilt test and Holter ECG monitoring in current clinical practice when its impairment is suspected Power spectral analysis of heart rate variability HRV ie the analysis of the spontaneous fluctuations of the heart period is exploited to analyze data collected during the tilt test and Holter-ECG deriving indices of the autonomic control
In C1-INH-HAE patients the cardiac ANS was investigated during a remission period during the AE attack and its prodromal phase
It has been demonstrated that HRV analysis extended to multiday ECG recordings may furnish an early marker of an angioedema attack
In this perspective the identification of markers like HRV parameters could play a crucial role in helping patients and physicians to characterize the ANS control in each patient and individualize treatment based on the ANS balance
This study aims to compare the cardiovascular ANS control in healthy subjects and HAE patients by head-up tilt test monitoring and evaluate the differences in long-term ANS between healthy controls and HAE patients by Holter ECG monitoring
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None