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Baroreflex failure, sympathetic storm, and cerebral vasospasm in fibulin-4 cutis laxa.
- Source :
-
Pediatrics [Pediatrics] 2014 May; Vol. 133 (5), pp. e1396-400. Date of Electronic Publication: 2014 Apr 14. - Publication Year :
- 2014
-
Abstract
- Sudden, severe, and life-threatening, the crises associated with baroreflex failure are diagnostically challenging, particularly in children, a population in which it has rarely been described. The baroreflex failure syndrome results from impaired afferent baroreceptive input and manifests with autonomic stimulation-induced surges in blood pressure and heart rate accompanied by distinct signs, including thunderclap headache, diaphoresis, and emotional instability. Although the adult literature includes cases of severe headache in baroreflex failure,(1) (,) (2) we present the first case of a child with recurrent thunderclap headache and cerebral vasospasm with baroreflex failure secondary to vascular complications of a rare genetic connective tissue disorder.<br /> (Copyright © 2014 by the American Academy of Pediatrics.)
- Subjects :
- Autonomic Nervous System Diseases diagnosis
Carotid Artery, Internal abnormalities
Carotid Artery, Internal pathology
Cerebral Angiography
Child
Cutis Laxa diagnosis
DNA Mutational Analysis
Headache Disorders, Primary diagnosis
Headache Disorders, Primary genetics
Humans
Magnetic Resonance Angiography
Male
Syndrome
Tomography, X-Ray Computed
Autonomic Nervous System Diseases genetics
Baroreflex genetics
Cutis Laxa genetics
Extracellular Matrix Proteins genetics
Reflex, Abnormal genetics
Vasospasm, Intracranial genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1098-4275
- Volume :
- 133
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 24733866
- Full Text :
- https://doi.org/10.1542/peds.2012-3539