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

Naomi Nussinovitch, Moshe Nussinovitch, Edna Peleg, Talma Rosenthal

Research output: Contribution to journalArticlepeer-review

Abstract

Background: 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. Objective: To the best of our knowledge, this is the only description of patients with 24-hour blood pressure monitoring. Study design: 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. Results: 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. Conclusions: Close monitoring of antihypertensive therapy should be considered in familial dysautonomia patients in whom blood pressure reaches excessive levels.

Original languageEnglish
Pages (from-to)507-511
Number of pages5
JournalPediatric Nephrology
Volume20
Issue number4
DOIs
StatePublished - Apr 2005

Keywords

  • Genetic
  • Hypertension
  • Hypotension
  • Riley Day syndrome
  • Tachycardia

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