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

Lancet. 1987 Nov 21;2(8569):1170-2. doi: 10.1016/s0140-6736(87)91317-1.

Abstract

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.

Publication types

  • Clinical Trial
  • Controlled Clinical Trial
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Blood Pressure / drug effects
  • Clinical Trials as Topic
  • Dopamine beta-Hydroxylase / deficiency*
  • Dose-Response Relationship, Drug
  • Droxidopa / administration & dosage
  • Droxidopa / therapeutic use*
  • Female
  • Humans
  • Hypotension, Orthostatic / drug therapy
  • Hypotension, Orthostatic / etiology
  • Norepinephrine / metabolism*
  • Serine / analogs & derivatives*

Substances

  • Serine
  • Dopamine beta-Hydroxylase
  • Droxidopa
  • Norepinephrine