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Clinical Autonomic Research

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01-06-2008 | RESEARCH ARTICLE

Cardiac sympathetic hypo-innervation in familial dysautonomia

Authors: David S. Goldstein, MD, PhD, Basil Eldadah, MD, PhD, Yehonatan Sharabi, MD, Felicia B. Axelrod, MD

Published in: Clinical Autonomic Research | Issue 3/2008

Abstract

Objective

Familial dysautonomia (FD) involves incomplete development of the sympathetic nervous system. Whether such loss extends to sympathetic innervation of the heart has been unknown. This study used 6-[¹⁸F]fluorodopamine neuroimaging to assess cardiac sympathetic innervation and function in FD.

Methods

Six adult FD patients underwent thoracic PET scanning for 30 minutes after i.v. 6-[¹⁸F]fluorodopamine injection, as did healthy volunteers without (N = 21) or with (N = 10) pre-treatment by desipramine, which interferes with neuronal uptake and thereby simulates effects of noradrenergic denervation. Effective rate constants for uptake and loss were calculated using a single compartment pharmacokinetic model.

Results

FD patients had decreased uptake and accelerated loss of 6-[¹⁸F]fluorodopamine-derived radioactivity in the interventricular myocardial septum (P = 0.009, P = 0.05) and ventricular free wall (P = 0.007, P < 0.001), compared to untreated controls. Desipramine-treated subjects had decreased uptake but normal loss of 6-[¹⁸F]fluorodopamine-derived radioactivity.

Conclusions

FD involves cardiac noradrenergic hypo-innervation. Since accelerated loss of 6-[¹⁸F]fluorodopamine-derived radioactivity cannot be explained by decreased neuronal uptake alone, FD may also involve augmented NE loss from extant terminals.

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Title: Cardiac sympathetic hypo-innervation in familial dysautonomia
Authors: David S. Goldstein, MD, PhD
Basil Eldadah, MD, PhD
Yehonatan Sharabi, MD
Felicia B. Axelrod, MD
Publication date: 01-06-2008
Publisher: D. Steinkopff-Verlag
Published in: Clinical Autonomic Research / Issue 3/2008
Print ISSN: 0959-9851
Electronic ISSN: 1619-1560
DOI: https://doi.org/10.1007/s10286-008-0464-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Cardiac sympathetic hypo-innervation in familial dysautonomia

Abstract

Objective

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

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