Document Detail


Respiratory and cerebrovascular responses to hypoxia and hypercapnia in familial dysautonomia.
MedLine Citation:
PMID:  12406829     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Although cardiorespiratory complications contribute to the high morbidity/mortality of familial dysautonomia (FD), the mechanisms remain unclear. We evaluated respiratory, cardiovascular, and cerebrovascular control by monitoring ventilation, end-tidal carbon dioxide (CO2-et), oxygen saturation, RR interval, blood pressure (BP), and midcerebral artery flow velocity (MCFV) during progressive isocapnic hypoxia, progressive hyperoxic hypercapnia, and during recovery from moderate hyperventilation (to simulate changes leading to respiratory arrest) in 22 subjects with FD and 23 matched control subjects. Subjects with FD had normal ventilation, higher CO2-et, lower oxygen saturation, lower RR interval, and higher BP. MCFV was also higher but depended on the higher baseline CO2-et. In the FD group, whereas hyperoxic hypercapnia induced normal cardiovascular and ventilatory responses, progressive hypoxia resulted in blunted increases in ventilation, paradoxical decreases in RR interval and BP, and lack of MCFV increase. Hyperventilation induced a longer hypocapnia-induced apneic period (51.5 +/- 9.9 versus 11.2 +/- 5.5 seconds, p < 0.008) with profound desaturation (to 75.8 +/- 3.5%), marked BP decrease, and RR interval increase. Subjects with FD develop central depression in response to even moderate hypoxia with lack of expected change in cerebral circulation, leading to hypotension, bradycardia, hypoventilation, and potentially respiratory arrest. Higher resting BP delays occurrence of syncope during hypoxia. Therapeutic measures preventing hypoxia/hypocapnia may correct cardiovascular accidents in patients with FD.
Authors:
Luciano Bernardi; Max Hilz; Brigitte Stemper; Claudio Passino; Goetz Welsch; Felicia B Axelrod
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2002-10-24
Journal Detail:
Title:  American journal of respiratory and critical care medicine     Volume:  167     ISSN:  1073-449X     ISO Abbreviation:  Am. J. Respir. Crit. Care Med.     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2003-01-13     Completed Date:  2003-02-13     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  9421642     Medline TA:  Am J Respir Crit Care Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  141-9     Citation Subset:  AIM; IM    
Affiliation:
New York University Medical Center, New York University School of Medicine, New York, USA. lbern1ps@unipv.it
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MeSH Terms
Descriptor/Qualifier:
Adaptation, Physiological*
Adolescent
Adult
Anoxia / etiology,  physiopathology*
Blood Pressure Determination
Case-Control Studies
Cerebrovascular Circulation / physiology
Chemoreceptor Cells / physiology*
Child
Dysautonomia, Familial / complications*,  diagnosis,  mortality
Female
Humans
Hypercapnia / etiology,  physiopathology*
Hypotension / diagnosis,  physiopathology
Male
Middle Aged
Oxygen Consumption / physiology*
Probability
Pulmonary Gas Exchange
Reference Values
Respiratory Mechanics
Risk Assessment
Survival Analysis

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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