Endogenous restoration of noradrenaline by precursor therapy in dopamine-beta-hydroxylase deficiency.

Biaggioni I, Robertson D
Lancet. 1987 2 (8569): 1170-2

PMID: 2890806 · DOI:10.1016/s0140-6736(87)91317-1

DL-dihydroxyphenylserine was given by mouth in a single-blind, placebo-controlled trial to two patients with orthostatic hypotension due to dopamine-beta-hydroxylase deficiency, in the hope of providing noradrenaline by endogenous decarboxylation. Dose-dependent increases in blood pressure were obtained over the range 150-600 mg. After 600 mg mean arterial pressure rose 33 and 19 mm Hg and these rises were tightly correlated with an increase in plasma noradrenaline (r = 0.995, p less than 0.001 and r = 0.88, p less than 0.05). Urinary noradrenaline increased from undetectable levels to 338 and 511 micrograms/24 h. Standing time (a correlate of functional capacity) also increased significantly in both patients. No side-effects were noted.

MeSH Terms (11)

Adult Blood Pressure Clinical Trials as Topic Dopamine beta-Hydroxylase Dose-Response Relationship, Drug Droxidopa Female Humans Hypotension, Orthostatic Norepinephrine Serine

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