PEDIATRICS Vol. 58 No. 4 October 1976, pp. 618-620
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Plasma Renin Activity in Familial Dysautonomia: Unresponsiveness to a beta-Adrenergic Blocker

Z. Hochberg M.D.1, L. Kahana Ph.D.1, A. Spindel M.D.1, and D. Diengott M.D.1

1 Rothschild University, Hospital, Haifa, Israel

In familial dysautonomia (FD) the autonomous nervous system, reflex control, superficial sensation, and motor coordination are all affected.1 Postural hypotension with periods of hypertension are common findings.

Rabinowitz et al.2 showed abnormal release of renin in seven patients with FD, six of them having high plasma renin activity (PRA). Abnormal values of PRA were observed in the upright position and on a low-salt diet.

Propranolol, a beta-adrenergic receptor blocker, inhibits PRA in man regardless of the previous PRA values.3

The effect of propranolol on PRA and blood pressure of children with FD was studied, since it was hoped that propranolol, by suppressing renin release, might stabilize their blood pressure.