Abstract | OBJECTIVE: METHODS: RESULTS: In all 14 patients with CIPA, plasma norepinephrine levels were very low or undetectable and failed to increase when the patient was upright, yet upright blood pressure was well maintained. Plasma epinephrine levels were normal and increased when the patient was upright. Plasma renin activity also increased appropriately when the patient was upright and after furosemide-induced volume depletion. Nitric oxide-mediated endothelial function was intact. Patients with pure autonomic failure also had very low levels of plasma norepinephrine both supine and upright, but in contrast to patients with CIPA failed to maintain blood pressure upright. INTERPRETATION: The results indicate that postganglionic sympathetic neurons are severely depleted in CIPA, but chromaffin cells of the adrenal medulla are spared. This confirms the differential effect of NGF signaling for sympathetic neural and chromaffin cell development. The finding that patients with CIPA maintain blood pressure well on standing challenges current concepts of the role of norepinephrine in the regulation of arterial pressure.
|
Authors | Lucy Norcliffe-Kaufmann, Stuart D Katz, Felicia Axelrod, Horacio Kaufmann |
Journal | Annals of neurology
(Ann Neurol)
Vol. 77
Issue 5
Pg. 743-52
(May 2015)
ISSN: 1531-8249 [Electronic] United States |
PMID | 25627679
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
|
Copyright | © 2015 The Authors Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association. |
Chemical References |
|
Topics |
- Adolescent
- Adult
- Aged
- Aged, 80 and over
- Blood Pressure
(physiology)
- Child
- Child, Preschool
- Female
- Hereditary Sensory and Autonomic Neuropathies
(blood, diagnosis, physiopathology)
- Humans
- Infant
- Male
- Middle Aged
- Norepinephrine
(blood, deficiency)
- Young Adult
|