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Biomedical subjects

A Moser

Publications and source records attributed to A Moser.

At least 127 records · Page 7Linked to original sources

Effects of somatostatin on dopamine sensitive adenylate cyclase activity in the caudate-putamen of the rat.

The effect of somatostatin-14 (SRIF) on dopamine-sensitive adenylate cyclase in caudate-putamen pellets was studied in naive female rats, and in rats with chemical lesions of the nigrostriatal dopaminergic tract produced by injection of 6-hydroxydopamine, or of the caudate-putamen itself produced by injection of kainic acid 3 week earlier. In unlesioned rats somatostatin at a concentration of 10(-7) moles/l inhibited adenylate cyclase activation by submaximal concentrations of dopamine, increasing the apparent Km but not altering Emax. In 6-hydroxydopamine lesioned rats somatostatin no longer influenced adenylate cyclase activity, whereas in kainic acid lesioned rats somatostatin still increased the apparent Km for dopamine activation. The effect of somatostatin in untreated and lesioned rats is compatible with a partial competitive antagonism to dopamine. Although the data from the lesioned rats present preliminary results, the dose response characteristics and the effects in lesioned animals suggest a more complex interaction, possibly by binding of somatostatin to an inhibitory subunit of regulatory adenylate cyclase components.

Adenylyl Cyclases↗

First trimester prenatal diagnosis of adrenoleukodystrophy by determination of very long chain fatty acid levels and by linkage analysis to a DNA probe.

A first trimester prenatal diagnosis of adrenoleukodystrophy has been done on chorionic villi biopsy in the pregnancy of a carrier woman. Two different approaches allowed one to determine that the male fetus was affected: the linkage analysis of DNA from chorionic villi using the highly polymorphic probe St 14 and the determination of very long chain fatty acid levels in cultured chorionic villi.

Adrenoleukodystrophy↗

Early manifestations of multiple sulfatase deficiency.

We describe two boys, presenting by 1 year of age, with developmental delay from birth, mildly coarse facial features, and hepatomegaly. These clinical features were most suggestive of a mucopolysaccharidosis, particularly MPS II. Biochemical studies, including sulfate incorporation in fibroblasts and lysosomal enzyme analyses in fibroblasts, leukocytes, and serum, showed abnormalities in both sulfatide and mucopolysaccharide metabolism and led to the diagnosis of multiple sulfatase deficiency. With time, both patients developed an ichthyotic rash and profound intellectual deterioration. We conclude that findings in the first year of life in some patients with MSD may closely resemble those in patients with a MPS disorder rather than the late infantile form of metachromatic leukodystrophy, as is classically described. Thus, MSD should be considered in the young patient suspected of having a MPS disorder.

Cerebroside-Sulfatase↗

[Modification of psychosocial adjustment by terminal disease].

Psychosocial adaption during or after somatic illness is relatively little studied--quite in difference to the better known psychosocial predictors of somatic illness. This study is concerned with a group of 31 female patients who suffered from a terminal vascular disease (primary vascular pulmonary hypertension)--an iatrogenic disease which with high probability is the consequence of a drug for weight reducing (Menocil), prescribed by their family doctors to these moderate overweight middle aged women. Psychosocial adaptions was assessed on a 5-point-scale, including the following dimensions: work-situation, socio-economic conditions, family adaption, social activities. Psychosocial adaption before illness was very good (just 10% below maximal values). Two or three years after illness started, however, there was a highly significant drop in all dimensions (most significant in "work" and "social activities"). To some extent success or failure of psychosocial adaption had a predictive power for the terminal course of this illness. Possible interaction between course of illness and psychosocial adaption is discussed and illustrated. There was also a significant correlation between psychosocial adaption and defense-processes.

Adult↗

[Overview of the epidemiology of stonefish poisonings, their treatment and preventive measures].

A review is presented of work on envenomation by stonefish (Synanceja spp.), which represent not only a danger for the inhabitants of tropical coasts but also for tourists. Stonefish are common in shallow water of reef areas by the shores of the Indian and Indopacific Ocean. The bizarrely shaped fish is often taken for a weed-covered stone and accidents occur when swimmers, divers or fishermen step on the stings of the dorsal fin. These stings are provided with poison glands. The venom has neurotoxic, myotoxic and hemorrhagic effects. The case of a 39-year-old diver is cited who suffered a stonefish stab which lasted for several weeks. Generally envenomations by Synanceja cause severe local pain and enormous swelling of the limb; systemic symptoms as usually found with neurotoxins are common; death may occur by shock, by paralysis of the diaphragm or cardiac arrest. For first aid bathing of the limb in hot water is recommended. Clinical measures are local analgesia, local neutralization of the venom, if possible antiserum therapy and intensive care with symptomatic treatment of systemic complications. The most effective prevention is adequate foot protection when wading in the sea.

Adult↗

Defense mechanisms and coping behavior in terminal illness. An overview.

Psychosomatic medicine is increasingly concerned with illness behavior, a concept which includes defense mechanisms and coping styles. An attempt was made to compare defensive processes and social adaptation in a group of 31 patients suffering from a probable terminal illness of iatrogenic origin. The surprising degree of conformity between the defense profiles towards the illness and those towards general life stress was found. Reaction-formation and stoicism were the defense mechanisms most often associated with successful defense. Interdependence of several ego measures such as defense against general life stress, defense against illness and social adaptation could be shown in various evaluations. Surprisingly, however, aggravators of illness turned out to be psychologically more stable than minimizers, a result which is in contrast to behavior in nonterminal illness. Theoretical implications of the results are discussed.

Adaptation, Psychological↗

Eye movement dysfunction in dementia of the Alzheimer type.

Patients with dementia of the Alzheimer type (DAT) are described to have disturbances in basic visual, complex visual and oculomotor functions. In order to study and quantify dysfunction of eye movements under several paradigms, they were measured with infrared photoelectric techniques and analyzed by a digital computer. The study included 12 normal subjects and 10 patients with mild to moderate DAT (DSM-III-R criteria). The authors' results could demonstrate both an attentional deficit to externally triggered, unpredictable targets and an impaired systematic, voluntary, internally organized scanning of the environment due to motivational and perceptional deficits.

Aged↗

Effect of interleukin-1 on lipid metabolism in the rat. Similarities to and differences from tumor necrosis factor.

Infection and inflammation are associated with hypertriglyceridemia, which is thought to be mediated by cytokines. Previous studies at our laboratory and others have shown that tumor necrosis factor acutely increases serum triglyceride levels primarily by stimulating hepatic lipid synthesis and secretion. The role of interleukin-1 (IL-1), a cytokine that is also secreted by stimulated macrophages and that has many actions that overlap those of tumor necrosis factor, has not been studied in depth. The present study demonstrates that IL-1, at doses similar to those that cause fever and anorexia and that stimulate adrenocorticotropic hormone secretion, rapidly increases serum triglyceride levels; this elevation persists for at least 17 hours. Serum cholesterol levels are not altered by IL-1. Neither is the clearance of triglyceride-rich lipoproteins affected by IL-1. However, hepatic triglyceride secretion, measured by the Triton WR-1339 technique, is increased in IL-1-treated animals. Accompanying this stimulation in hepatic lipid secretion is an increase in de novo fatty acid synthesis in the liver. IL-1 does not increase serum free fatty acid and glycerol levels, suggesting that IL-1 does not stimulate lipolysis in vivo. Additionally, inhibition of lipolysis does not prevent the increase in serum triglyceride levels, providing further evidence that lipolysis does not play a crucial role in the increased hepatic lipid synthesis and secretion induced by IL-1. In contrast, tumor necrosis factor increases lipolysis, which contributes to the increase in serum triglycerides. That multiple cytokines rapidly elevate plasma triglyceride levels suggest that these changes in lipid metabolism may play an important role in the organism's response to infection and inflammation.

Animals↗

Lipid infusion with different triglyceride cores (long-chain vs medium-chain/long-chain triglycerides): effect on plasma lipids and bilirubin binding in premature infants.

The possible beneficial effects of infusing a lipid emulsion containing 50% by weight of medium-chain triglycerides (MCT) compared with a standard long-chain triglyceride (LCT) emulsion were studied in 18 premature neonates (gestational age less than 34 weeks) requiring parenteral nutrition. The infants were assigned in a double-blind manner to receive one of the two lipid emulsions over 17 hours a day as a supplemental regimen for total parenteral nutrition. A lipid load of 1 g/kg per day was initiated on the third day of life and was increased at the rate of 1 g/kg per day until a maximal dose of 3 g/kg per day was obtained on the fifth day of life and maintained thereafter. Both bound and unbound bilirubin decreased with both infusion regimens during the study period. Despite a marked increase in plasma free fatty acid levels (260% in the MCT/LCT group compared with 210% in the LCT group), the fraction of unbound (free) bilirubin was significantly lower in the MCT/LCT group (34% vs 13%). Free fatty acid levels, corrected to albumin, were positively correlated to the percentage of free bilirubin only for the LCT lipid infusion. The finding of a significant elevation of plasma cholesterol levels only in the MCT/LCT group is now under investigation. Use of the MCT-containing emulsion was not associated with a higher frequency of adverse effects than the commonly used LCT-containing emulsion.(ABSTRACT TRUNCATED AT 250 WORDS)

Bilirubin↗

The peroxisome deficient PEX2 Zellweger mouse: pathologic and biochemical correlates of lipid dysfunction.

Zellweger syndrome is the prototypic human peroxisomal biogenesis disorder that results in abnormal neuronal migration in the central nervous system and severe neurologic dysfunction. A murine model for this disorder was previously developed by targeted deletion of the PEX2 peroxisomal gene. By labeling neuronal precursor cells in vivo with a mitotic marker, we can demonstrate a delay in neuronal migration in the cerebral cortex of homozygous PEX2 mutant mice. Postnatal PEX2 Zellweger mice develop severe cerebellar defects with abnormal Purkinje cell development and an altered folial pattern. When the PEX2 mutation is placed on an inbred murine genetic background, there is significant embryonic lethality and widespread neuronal lipidosis throughout the brain. Biochemical analysis of PEX2 mutant mice shows the characteristic accumulation of very long chain fatty acids and deficient plasmalogens in a wide variety of tissues. Docosahexaenoic acid levels (DHA; 22:6n-3) were found to be reduced in the brain of mutant mice but were normal in visceral organs at birth. All tissues examined in postnatal mutant mice had reduced DHA. The combined use of morphologic and biochemical analyses in these mice will be essential to elucidate the pathogenesis of this complex peroxisomal disease.

Animals↗

[Bilateral pheochromocytoma associated with duodeno-jejunal GIST in patient with von Recklinghausen disease: report of a clinical case].

The authors present the case of a 60-year-old male patient suffering from von Recklinghausen's disease (neurofibromatosis type I, NF1) with bilateral pheochromocytoma and occasional intraoperative reports of duodenojejunal GIST (GastroIntestinal Stromal Tumour). Through a review of the literature the authors analyze the frequency and the features of bilateral pheochromocytoma and its rare histological variant, the so-called composite pheochromocytoma, characterized by the combination of pheochromocytoma and ganglioneuroma or ganglioneuro-blastoma. Bilaterality of pheochromocytoma is more frequent in patients with familiarity for pheochromocytoma without NF1. Composite pheochromocytoma accounts for about 3% of total pheochromocytomas. In addition, the authors summarize the present knowledge about gastrointestinal stromal tumours and investigate the possible association between them and NF1 or pheochromocytoma, concluding that any such association is purely casual, while confirming the well known, genetically determined association between NF1 and pheochromocytoma.

Adrenal Gland Neoplasms↗