Dopamine /8‐Hydroxylase Deficiency A Genetic Disorder of Cardiovascular Regulation
作者:
David Robertson,
Virginia Haile,
Sharon Perry,
Rose Robertson,
John Phillips,
Italo Biaggioni,
期刊:
Hypertension
(OVID Available online 1991)
卷期:
Volume 18,
issue 1
页码: 1-8
ISSN:0194-911X
年代: 1991
出版商: OVID
数据来源: OVID
摘要:
Dopamine /3-hydroxyIase (DBH) deficiency is a genetic disorder in which affected patients cannot synthesize norepinephrine, epinephrine, and octopamine in either the central nervous system or the peripheral autonomic neurons. Dopamine acts as a false neurotransmitter in their noradrenergic neurons. Neonates with DBH deficiency have had episodic hypothermia, hypoglycemia, and hypotension, but survivors sometimes cope relatively well until late childhood when overwhelming orthostatic hypotension profoundly limits their activities. The hypotension may be so severe that clonic seizures supervene. Most currently recognized patients are young or middle-aged adults. The diagnosis is established by the observation of severe orthostatic hypotension in a patient whose plasma norepinephrine/dopamine ratio is much less than one.
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