Patients with Congenital Dopamine -Hydroxylase Deficiency: A Lesson in Catecholamine Physiology
| العنوان: | Patients with Congenital Dopamine -Hydroxylase Deficiency: A Lesson in Catecholamine Physiology |
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| المؤلفون: | Joke H.M. Tulen, Theo Thien, Anton H. van den Meiracker, Steven W. J. Lamberts, Claude Julien, Maarten A.D.H. Schalekamp, Arie Man in 't Veld, Jacques Lenders, Peter Moleman, Frans Boomsma |
| المصدر: | American Journal of Hypertension. 1:231-238 |
| بيانات النشر: | Oxford University Press (OUP), 1988. |
| سنة النشر: | 1988 |
| مصطلحات موضوعية: | Adult, medicine.medical_specialty, Levodopa, Tyrosine hydroxylase, business.industry, Dopamine antagonist, Dopamine beta-Hydroxylase, Syndrome, medicine.disease, Hypoprolactinemia, Norepinephrine, Catecholamines, Endocrinology, Autonomic Nervous System Diseases, Dopamine, Internal medicine, Internal Medicine, medicine, Dopamine beta hydroxylase deficiency, Catecholamine, Humans, business, medicine.drug |
| الوصف: | We recently described a case of congenital dopamine beta-hydroxylase (DBH) deficiency. The syndrome is characterized by noradrenergic denervation, adrenomedullary failure, but intact baroreflex afferents, cholinergic innervation, and adrenocortical function. Norepinephrine, epinephrine, and their degradation products were undetectable in plasma, urine, and cerebrospinal fluid, whereas dopamine and its degradation products were elevated. Plasma DBH was not detectable. Studies in this novel syndrome showed evidence for the peripheral production of dopamine from sympathetic nerve terminals noradrenergic in nature. Tyrosine hydroxylase is probably induced in this syndrome, since plasma levels of L-DOPA were also elevated. Absence of hemodynamic effects of sympathicolytic agents in the face of an increase in blood pressure after dopamine antagonists suggest that intrasynaptic concentrations of dopamine are in the range of its plasma concentrations. Hypoprolactinemia, reduced REM sleep, increased slow wave sleep and sodium loss, despite low blood pressure, are further evidence for the biological role of dopamine in man. |
| تدمد: | 1941-7225 0895-7061 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c94b1d3dad6ad09ca8966ef8e50c6030 https://doi.org/10.1093/ajh/1.3.231 |
| رقم الأكسشن: | edsair.doi.dedup.....c94b1d3dad6ad09ca8966ef8e50c6030 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 19417225 08957061 |
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