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[Ondine's Curse and rare oculomotor abnormalities: a case report].

Ondine's Curse or congenital central hypoventilation syndrome (CCHS) is a neurocristopathy (failure of migration or differentiation of neural crest-derived precursor cells) and is characterized by hypoventilation or apnea, which is most pronounced during sleep, with no other abnormalities of the neuro-respiratory system. Because of respiratory distress soon after birth, patients must be intubated and ventilated for a long time. This disorder may be associated with other symptoms of neurocristopathy (Hirschsprung disease, neuroblastoma, neuroganglioma) and other abnormalities of the autonomic nervous system (vasomotor dysfunctions or ophthalmic abnormalities: abnormal pupils, insufficient convergence, strabismus, or ptosis). We report the original case of a CCHS patient who presented with alternative ptosis of both the right and left eyes and esotropia. The ocular findings should lead to earlier diagnosis and speedier adequate treatment.

Humans↗

Perspectives on the integrative functions of the 'sympatho-adrenomedullary system'.

An historic survey is given of the gradual change of views and concepts concerning how the sympatho-adrenomedullary system is organized and operates: While it for nearly a century was considered to merely exhibit more or less generalized activation-inhibition responses, experimental studies during the last 50 years have revealed how it instead constitutes a highly sophisticated instrument for control, engaged in a variety of differentiated response patterns by which the brain controls events in major organ systems, down to include their cellular-molecular levels of organisation.

Adrenal Medulla↗

Cerebral veins: fluorescence histochemistry, electron microscopy, and in vitro reactivity.

Pial veins, choroid plexus veins, and the cerebri magna vein were investigated with regard to their ultrastructural organization, adrenergic nerve supply, and in vitro reactivity. The vessel walls consisted of a continuous layer of endothelial cells, large amounts of collagenous material, and occasional pericytes. Smooth muscle cells were observed only in a few specimens from the cerebri magna vein. All veins were surrounded by adrenergic nerve fibres. Potassium (124 mM) and noradrenaline (10(-5) - 10(-4) M) induced small contractions (0.2-0.5 mN) of isolated veins during in vitro conditions. The magnitude of these responses was less than one-tenth of that obtained in small pial arteries.

Animals↗

Differentiation of vasoactive renal sympathetic nerve fibres.

Activation of renal sympathetic nerves produces marked changes in renal haemodynamics, tubular ion and water transport and renin secretion. This review examines information indicating that these effects are mediated by functionally specific groups of renal sympathetic nerve fibres separately innervating the renal vessels, tubules and juxtaglomerular granular cells.

Animals↗

Role of the suprachiasmatic nucleus in the pathogenesis of migraine attacks.

Neuroanatomic, morphometric, immunocytochemical, neurobiochemical and clinical data support the hypothesis that the suprachiasmatic nucleus of the hypothalamus might be the initial site of migraine attacks. The prodromal phase of a migraine attack could be considered a syndrome of functional suprachiasmatic nucleus insufficiency, and other phases a reactive denervation hypersensitivity with the affection of the visual, nociceptive, antinociceptive and cranial vasomotor system.

Biological Clocks↗

Inhibition of oestrone sulphatase activity by tibolone and its metabolites.

Tibolone is a 19-nortestosterone derivative commonly used in hormone replacement therapy. Although tibolone and its 3alpha/beta-hydroxy metabolites exert oestrogenic effects on bone and the vasomotor system, they do not appear to stimulate breast tissue proliferation. It has been proposed that the lack of an oestrogenic effect on breast tissues may result from the inhibition of oestrone sulphatase (E1-STS) in this tissue by tibolone and its metabolites. In this study we have examined the ability of tibolone and its metabolites to inhibit E1-STS activity in intact breast cancer cells, its effect on E1-STS activity in placental microsomes and also the expression of E1-STS mRNA in more detail. As the major proportion of hydroxytibolone metabolites circulate in a sulphated form, the ability of the 3alpha-sulphate and 3alpha,17beta-disulphate metabolites to inhibit E1-STS activity was also examined. In MCF-7 cells, tibolone and its 3beta-hydroxylated metabolite were relatively potent inhibitors; they inhibited activity by 48 % and 46 %, respectively. In these cells, the 3alpha-sulphate and 3alpha,17beta-disulphate metabolites of tibolone inhibited E1-STS activity by 95% and 79% at 10 microM, respectively. No effects of tibolone or its metabolites on the expression of E1-STS mRNA in MCF-7 cells were detected. Using T-47D breast cancer cells, evidence was obtained that the sulphated metabolites of tibolone could continue to inhibit E1-STS activity after removal of the drugs and extensive washing of cells. In placental microsomes, however, the 3beta-hydroxy metabolite was the most potent inhibitor with an IC50 of 20.5 microM; the sulphated metabolites were less potent. Neither tibolone nor its metabolites had any inhibitory effect on the conversion of oestrone to oestradiol in breast cancer cells. Results from this study have confirmed that tibolone and its metabolites can inhibit E1-STS activity. This may explain the absence of breast stimulation as observed in clinical studies.

Breast Neoplasms↗

[Idiopathic rhinitis].

Idiopathic Rhinitis (IR) is a term for a group of nasal diseases of unknown aetiology. Formerly, these diseases were called vasomotor rhinitis, but because there is no indication of a disorder in the vasomotor system, this term has been abandoned. Other terms, like non-infectious non-allergic rhinitis and non-allergic non-infectious perennial rhinitis are purely descriptive. Potential pathomechanisms in IR could be 1) neural dysfunction/dysregulation; 2) immunological inflammatory responses; and 3) changes of the permeability of the mucosa, leading to increase entrance of potential harmful substances. Idiopathic Rhinitis is a diagnosis by exclusion, meaning that a number of more or less defined nasal conditions as a cause of rhinitis have been excluded - not only allergic rhinitis. Although a considerable progress was made in the standardization of provocation tests or biomarkers in nasal secretions, there are today no specific test available to confirm the diagnosis rhinitis in many non allergic conditions. An evidence-based review of treatment outcomes shows topical sprays of steroids, antihistamines to be of benefit in idiopathic rhinitis. Furthermore, nasal saline solution is effective in many patients with IR. Multiple surgical procedures have been suggested for IR, but should be reserved for patients who fail medical therapy.

Adrenal Cortex Hormones↗

Vasodilator activity of human alpha-calcitonin gene-related peptide in the feline mesenteric vascular bed.

Human alpha-calcitonin gene-related peptide (CGRP) is a substance immunohistochemically present in a number of organ systems; however, the biologic significance of this peptide is uncertain. The present study was undertaken to investigate the effects of CGRP in the feline mesenteric vascular bed under conditions of controlled blood flow. Bolus injections of CGRP decreased mesenteric perfusion pressure in a dose-related fashion. When compared with nitroglycerin, CGRP possesses markedly greater vasodilator activity in the intestinal vascular bed of the cat. Because circulating levels of CGRP have been identified, the present data suggest CGRP may play a role in the regulation of systemic vasomotor tone and regional hemodynamics, as well as involve altered calcium metabolism seen with other calcium-regulating hormones in hypertensive states.

Animals↗

Clinically benign fever of unknown origin: a personal retrospective.

The purpose of this discussion has been to bring to the attention of physicians the fact that all instances of etiologically undefined persistent fever are not associated with potentially serious or life-threatening organic disease, regardless of the height of the temperature. As has been pointed out, many patients with FUO clearly have disorders that are clinically benign, and the cause of these disorders is defined much more frequently on the basis of information obtained from a detailed historic inquiry than on the basis of findings made during the most meticulous physical examination and extensive laboratory studies. These individuals are usually seen first in an outpatient setting and seldom, if ever, require hospitalization because the cause of their FUO can, with uncommon exceptions, be identified as a physiologic or emotional dysfunction, a reaction to a drug or a chemical, or a disorder that is genetically determined. Failure to recognize that even a high elevation of the temperature can represent a clinically benign situation may lead to unnecessary hospitalization, during which the many investigations that are usually carried out may serve only to reinforce the patient's concern about a serious disease. It is most important for both patients and physicians to be aware that temperature, like all other physiologic and chemical measurements in humans, is expressed by a range of values and that a temperature of 98.6 degrees F is not normal for all persons. It must also be appreciated that "normal" temperature varies with age. The newborn infant may develop high-grade fever in the absence of disease because of marked instability of the vasomotor system.(ABSTRACT TRUNCATED AT 250 WORDS)

Body Temperature↗

The role of endogenous nitric oxide in the sympathetic and hemodynamic response to platelet activating factor-induced hypotension in anesthetized dogs.

We previously demonstrated that platelet-activating factor (PAF) when injected intravenously decreases renal sympathetic nerve activity in anesthetized dogs. Recently, nitric oxide (NO) has been shown to inhibit renal sympathetic nerve activity. The present study was designed to determine, using the NO synthase inhibitor NG-nitro-L-arginine methyl ester (L-NAME), whether endogenous NO contributes to the PAF-induced renal sympatho-inhibition in anesthetized dogs. We also determined the role of NO in systemic and pulmonary hemodynamics during PAF-induced hypotension. In response to PAF (10 microg x kg(-1), intravenously), renal sympathetic nerve activity showed similar responses in animals pretreated with L-NAME (n = 7; 20 mg x kg(-1) bolus and .05 mg x kg(-1) x min[-1]), D-NAME (n = 7), and phenylephrine (n = 7), as characterized by an initial increase (230%) followed by a decrease (56%). The depressor response to PAF was also similar as early as 10 min after injection in all PAF-injected groups. In contrast, L-NAME pretreatment potentiated PAF-induced pulmonary hypertension. Pulmonary arterial pressure 10 min after PAF in the L-NAME group (25+/-2 mmHg) was significantly greater than that in the D-NAME group (12+/-3 mmHg). In conclusion, endogenously produced NO is not involved in PAF-induced renal sympathetic nerve response or hypotension but attenuates PAF-induced pulmonary hypertension at the early stage in anesthetized dogs.

Anesthetics↗