24-hour ambulatory blood pressure monitoring in children with familial dysautonomia

Familial dysautonomia (Riley Day syndrome) is a genetic disease. The present study of 24-hour ambulatory blood pressure monitoring in children with familial dysautonomia was carried out to investigate the pattern of blood pressure in this syndrome. To the best of our knowledge, this is the only desc...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Pediatric nephrology (Berlin, West) West), 2005-04, Vol.20 (4), p.507-511
Hauptverfasser: Nussinovitch, Naomi, Nussinovitch, Moshe, Peleg, Edna, Rosenthal, Talma
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
Beschreibung
Zusammenfassung:Familial dysautonomia (Riley Day syndrome) is a genetic disease. The present study of 24-hour ambulatory blood pressure monitoring in children with familial dysautonomia was carried out to investigate the pattern of blood pressure in this syndrome. To the best of our knowledge, this is the only description of patients with 24-hour blood pressure monitoring. Vasomotor instability reflected in extreme hypertension and hypotension was recorded by 24-hour ambulatory blood pressure monitoring in three patients with familial dysautonomia: a 16-year old girl, a 14-year old boy and a 3-year old boy. Recordings were taken on a routine school day in the first two patients and during hospitalization in the third. Patients 1 and 2 displayed circadian rhythm but with significantly higher than normal blood pressure and heart rate. Patient 3 exhibited these fluctuations to a lesser degree. Postural hypotension without compensatory tachycardia was frequently seen in all three patients. Unusual variability in blood pressure was recorded during routine activities in patients 1 and 2 and during an acute attack in patient 3. Close monitoring of antihypertensive therapy should be considered in familial dysautonomia patients in whom blood pressure reaches excessive levels.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-004-1743-2