Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The observation in 14 dialysis patients of an encephalopathy associating myoclonia, dysarthria, generalised seizures in some cases, worsening over a few months, led to an aetiological inquiry based upon comparative study of patients with or without encephalopathy treated in the same centre or at home, and controls. Higher levels of aluminium were found in the frontal cortex grey matter of encephalopathy patients as compared to the control group. The same applies to manganese in the white matter. Copper, zinc and iron contents were not different. Aluminium levels in blood, dialysis bath and tap water supply were higher in center dialysis than in home dialysis. Blood aluminium levels at the end of hemodialysis were correlated with bath aluminium levels. The ingestion of alumine gels was not greater in the encephalopathy patients than in other hemodialysis patients; its estimation, in each case, was not related to the blood aluminium levels at the begining of hemodialysis. These finding indicate the need of a routine measure of metal content - mainly aluminium and manganese - in tap water used for dialysis, in order to treat this water if necessary.
 ...
PMID:[Progressive myoclonic encephalopathy in dialysis patients. The role of the water used for haemodialysis (author's transl)]. 65 14

Serum and 24 hours' urinary copper levels were studied in 71 patients with acute viral hepatitis including 35 with encephalopathy. Thirty age and sex matched healthy controls were also studied. Copper estimation was done by atomic absorption spectrophotometry. Serum and 24 hours' urinary copper levels (164.85 +/- 29.31 micrograms/dl and 123.54 +/- 7.87 micrograms/24 h respectively) were significantly (P less than 0.001) increased in acute viral hepatitis patients. There was no significant difference in levels between patients with and without encephalopathy.
 ...
PMID:Serum and urinary copper in acute hepatic encephalopathy. 229 50

The neuronal ceroid-lipofuscinoses (NCLs) are a group of recessively inherited neurodegenerative lysosomal storage diseases, the pathogenesis of which is unknown. In the present study, we have measured iron and cooper in cerebrospinal fluids (CSF) using methods that detect these metals in a "loosely bound" form, complexable to the chelators bleomycin and 1,10-phenanthroline. We studied 25 children with NCL, 21 children with encephalopathy of some other type, and 5 control children without neurological complications. The CSF concentrations of loosely bound iron at neutral pH values and of loosely bound copper did not correlate with the clinical diagnosis of the patients, nor did they parallel degenerative symptoms in NCL, such as mental impairment, visual loss, motor handicap, and epilepsy. However, the concentrations of loosely bound iron and copper increased significantly with the age of the patient; this is a novel finding and may represent increasing tissue destruction with age. Our present findings do not support a major role for primary iron toxicity in the development of neuronal degeneration. To investigate any secondary pathological role for malplaced transition metals, further research is required.
 ...
PMID:Bleomycin-detectable iron and phenanthroline-detectable copper in the cerebrospinal fluid of patients with neuronal ceroid-lipofuscinoses. 246 55

The successful management of hepatic diseases of dogs and cats requires an understanding of hepatic metabolism and nutritional processes. General aspects of dietary therapy for hepatic diseases are described, along with specific recommendations for the promotion of tissue regeneration. Special considerations, including the role of diet in encephalopathy, hepatic lipidosis, and copper-associated hepatic toxicosis, are also discussed.
 ...
PMID:Nutritional management of hepatic disease. 265 88

The authors describe two new cases of Menkes' syndrome, both of which belong to the same family. Clinically, one of them began with a convulsive encephalopathy and the most significant features observed in the other were psychomotor delay and changes in the hair. In both cases, the quantity of copper and ceruloplasmin was very low. A description is given of the clinical, electroencephalographic and radiological findings and a microscopic study of the hair. One of them died at the age of 16 months and the other is at present 2 5/12 years old and demonstrates a serious psychomotor delay. Given the inefficiency of the treatment, genetic counsel and prenatal diagnosis are the only useful alternatives for the control of this phenomenon.
 ...
PMID:[Menkes syndrome: study of 2 new cases]. 269 65

Zinc and copper levels in the blood and urine of 44 liver cirrhosis patients and 10 healthy volunteers are reported. The clinical severity of the liver disease (according to the Child classification system) was correlated with the levels of the two metals. An attempt was also made to break down the parameters on which the Child System in based in order to contribute to the pathogenic interpretation of changing zinc levels in liver disease patients. The results show a significant reduction in zinc in the blood but an increase in the urine in the various degrees of liver damage. A connection was also noted between low blood zinc and encephalopathy.
 ...
PMID:[Determination of zinc and copper in patients with liver cirrhosis of diverse clinical severity]. 318 51

In 2 patients with a progressive dialysis encephalopathy and increased serum aluminium concentrations a Desferal-treatment with 500 mg per week was performed and in these cases the kinetics of Al, Fe, Cu was investigated. After the application of Desferal an increase of the serum levels, the ultrafiltrable proportion, the dialysance and the eliminated quantity of aluminium developed. In the course of the treatment in the two patients the aluminium level could be reduced to approximately normal values. Nevertheless one patient died of a severe course of a progressive dialysis encephalopathy. An increase of the copper concentration independent of Desferal-treatment after the dialysator passage remained without influence on the course of concentration of the serum copper in the examination period. A remarkable effect of the Desferal-therapy on the iron metabolism could not be established. Since in the dosage used there did not appear any side effects the Desferal-application is to characterized as a distinct and effective technique for the decrease of the serum-aluminium-concentration in patients undergoing dialysis.
 ...
PMID:[Desferal treatment of aluminum poisoning; effect on serum concentrations of aluminum (Al), iron (Fe) and copper (Cu)]. 381 45

Seven patients with rheumatoid disease were given the iron-chelating drug desferrioxamine (DFX) to evaluate its possible anti-inflammatory effects. Two of these patients, who also received the anti-emetic prochlorperazine, lost consciousness for 48-72 h and then fully recovered. Electroencephalography showed abnormalities of the type associated with metabolic disturbance. One of these patients showed pyramidal features and subsequently developed an optic neuropathy and pigmentary retinopathy. Analysis of his cerebrospinal fluid showed a decrease in loosely-bound (catalytic) iron and increase in loosely-bound (catalytic) copper, total iron and products of lipid peroxidation, with values approaching normal as the symptoms resolved. Subsequent in vivo/vitro studies clearly demonstrated that the neurological effects were due to a synergistic action of desferrioxamine and prochlorperazine, probably resulting in exceptional fluxes of intra/extra cellular iron/copper disturbing noradrenergic and serotonergic systems. Two other patients who did not receive prochlorperazine, developed retinal problems which later improved, one after only 15 g of desferrioxamine. Our observations suggest a new model for metabolic encephalopathy studies and provide insight into the mechanisms of pigmentary retinopathy.
 ...
PMID:Cerebral and ocular toxicity induced by desferrioxamine. 409 47

Bone tissue from a patient with chronic renal failure and a dialysis encephalopathy syndrome has been studied by histological and histochemical means, by flame emission spectroscopy and by electron probe X-ray microanalysis. There was significant renal osteodystrophy manifest as an osteomalacia. Emission spectroscopy showed the presence of iron (Fe), aluminium (Al), silicon (Si), zinc (Zn), strontium (Sr), lead (Pb) and copper (Cu) in the concentration range 100-1000 parts per million (ppm). Electron probe X-ray microanalysis showed focal concentrations of Fe and Si in the marrow tissue only, whereas Al was localized to the calcification front zones at the junction of osteoid and mineralized tissue of both trabecular and cortical bone. It is concluded that the presence of Al at these sites could interfere with the mineralization process and significantly contribute to the pathogenesis of haemodialysis-related osteomalacia and that it is unlikely that the other elements detected are significant in this regard.
 ...
PMID:The localization of aluminium and other elements in bone tissue of a case of renal osteodystrophy with an associated dialysis encephalopathy syndrome. 671 13

Authors report the cases of a boy with a Menkes' disease which began by a convulsive encephalopathy at the age of two months with growth failure and metaphysical abnormalities. An oldest brother died at 2 years of age in an analogous scene. The diagnosis proved to be founded by the low serum copper and coeruleo-plasmia levels and by the increased uptake of copper in the cultured skin-fibroblasts. The hair was thin and kinky; microscopical study showed pili torti and trichorrhexis nodosa. For the following pregnancy of the mother, a prenatal diagnosis by precocious amniocentesis (amniotic fluid punction) allowed to say that the boy she was expecting for was clear of the disease because of the normal uptake of copper 64 Cu in cultured amniotic fluid cells of the foetus. Authors recall that Menkes' disease is a X linked recessive disorder beginning by epileptic seizure in the early months of life. The basic biochemical lesion of copper metabolism is unknown; there are abnormalities in copper and his binding protein (metallothionein) distribution with increased level is the kidney and decreased level in brain and liver. The copper therapy does not lead to clinical improvement. There is now a prenatal diagnosis: the study of copper uptake in cultured amniotic fluid cells of male fetus. It is abnormally increased in Menkes' disease.
 ...
PMID:[A new case of Menkes syndrome. Prenatal exclusion diagnosis in a subsequent pregnancy]. 673 27


1 2 3 4 5 Next >>