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Dopamine B Hydroxylase Deficiency Responsible for Severe Dysautonomic Orthostatic Hypotension in an Elderly Patient
Author(s) -
Gentric A.,
Fouilhoux A.,
Caroff M.,
Mottier D.,
Jouquan J.
Publication year - 1993
Publication title -
journal of the american geriatrics society
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.992
H-Index - 232
eISSN - 1532-5415
pISSN - 0002-8614
DOI - 10.1111/j.1532-5415.1993.tb01894.x
Subject(s) - medicine , orthostatic vital signs , dopamine , aromatic l amino acid decarboxylase , catecholamine , endocrinology , blood pressure , norepinephrine
We report the case of an elderly woman with severe dysautonomic orthostatic hypotension in whom a deficit in dopamine B hydroxylase has been established. In the literature, such a deficit has been described in six young adults with long standing symptoms of postural hypotension. This enzyme catalyses the conversion of dopamine to noradrenaline. In our elderly patient, noradrenaline and adrenaline were undetectable in the plasma, but plasma dopamine was detectable. Treatment with the synthetic amino acid, DL‐threodihydroxyphenylserine, which is converted to noradrenaline by dopa‐decarboxylase, resulted in a significant increase in blood pressure. The mechanism of this acquired deficit is not elucidated.