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

Abstract

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.