Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bentonite clay has been used for the adsorption of Fe(II) from aqueous solutions over a concentration range of 80-200 mg/l, shaking time of 1-60 min, adsorbent dosage from 0.02 to 2 g and pH of 3. The process of uptake follows both the Langmuir and Freundlich isotherm models and also the first-order kinetics. The maximum removal (>98%) was observed at pH of 3 with initial concentration of 100 mg/l and 0.5 g of bentonite. The efficiency of Fe(II) removal was also tested using wastewater from a galvanized pipe manufacturing industry. More than 90% of Fe(II) can be effectively removed from the wastewater by using 2.0 g of the bentonite. The effect of cations (i.e. zinc, manganese, lead, cadmium, nickel, cobalt, chromium and copper) on the removal of Fe(II) was studied in the concentration range of 10-500 mg/l. All the added cations reduced the adsorption of Fe(II) at high concentrations except Zn. Column studies have also been carried out using a certain concentration of wastewater. More than 99% recovery has been achieved by using 5 g of the bentonite with 3M nitric acid solution.
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PMID:Removal of Fe(II) from the wastewater of a galvanized pipe manufacturing industry by adsorption onto bentonite clay. 1553 87

Wilson's disease is a rare autosomal recessive disease of copper accumulation and copper toxicity, due to mutations in the ATP7B gene, which leads to a failure of copper excretion in the bile. It presents clinically primarily as liver disease, psychiatric disease, neurological disease, or a combination of these. The neurological disease is a movement disorder, with abnormalities of speech, tremor, incoordination and dystonia being common features. Diagnosis of neurologically presenting patients is usually straightforward, with Kayser-Fleischer rings and a urine copper over 100 microg/day almost invariably present. In the treatment of neurologically presenting patients, penicillamine should always be avoided, because of the high risk of permanent, drug-induced, additional neurological deterioration. A new drug we have developed, tetrathiomolybdate, given for 8-16 weeks, in combination with zinc, is our first choice for treating these patients. In the absence of availability of tetrathiomolybdate, zinc or trientine are the next best choices.
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PMID:Neurologically presenting Wilson's disease: epidemiology, pathophysiology and treatment. 1574 Jan 74

Wilson's disease and Menkes disease are inherited genetic disorders of copper metabolism. Each disease results from the absence or dysfunction of homologous copper-transporting ATPases present in the trans-Golgi network of cells. The Wilson ATPase transports copper into the hepatocyte secretory pathway for incorporation into ceruloplasmin and excretion into the bile. Thus, patients with Wilson's disease of the autosomal recessive trait present with signs and symptoms arising from impaired biliary copper excretion. The Menkes ATPase transports copper across the placenta, gastrointestinal tract, and blood-brain barrier, and the clinical features of this X-linked disease arise from copper deficiency. Despite striking differences in the clinical presentation of these two diseases, the respective ATPases function in precisely the same fashion within the cell. The different clinical features of each disease are the results of the tissue specific expression of these ATPases. In Wilson's disease, impaired biliary copper excretion leads to accumulation of this metal in the liver. When the capacity for hepatic storage is exceeded, cell death ensues, with copper release into the plasma resulting in hemolysis and deposition of copper in extrahepatic tissues. Affected patients usually present in the first or second decade of life with chronic hepatitis and cirrhosis or acute liver failure. Copper accumulation in the cornea results in Kayser-Fleischer rings. Neuropsychiatric symptoms are more common in adults and include dystonia, tremor, personality changes, and cognitive impairment as a results of copper accumulation in the basal ganglia and other brain regions. The diagnosis of Wilson's disease is confirmed by decreased serum ceruloplasmin, increased urinary copper, and elevated hepatic copper concentration. A large number of different mutations occur in the genes of patients with Wilson disease. Copper chelation drugs and zinc are effective in most cases. New treatment guidelines now advise physicians to start patients on zinc.
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PMID:[Genetic disorders of copper transport--diagnosis and new treatment for the patients of Wilson's disease]. 1577 21

Wilson disease is an autosomal recessive inherited disorder of human copper metabolism that leads to neurological symptoms and hepatic damage of variable degree. The affected gene ATP7B encodes a hepatic copper transport protein, which plays a key role in human copper metabolism. Clinical symptoms are complex with neurologic symptoms such as tremor, dysarthria, psychiatric disorders etc., predominant hepatic disease or mixed forms. Copper deposition in the liver results in acute liver failure, chronic hepatitis or liver cirrhosis. Early recognition by means of clinical, biochemical or genetic examination and early initiation of therapy with chelators or zinc-salts are essential for outcome and prognosis. Liver transplantation is an alternative in cases with acute and chronic liver failure and cures the hepatic disease. Frequent monitoring of drug therapy, adverse effects, and compliance is critical for the prognosis of the disease.
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PMID:[Wilson disease]. 1591 61

A sensitive spectrophotometric method for the determination of freely dissolved copper concentrations in aqueous samples after preconcentration with hollow fiber membrane extraction has been developed. The method is based on the equilibrium sampling through a selective membrane into an acceptor solution containing 4-(pyridyl-2-azo)resorcinol (PAR), which serves as stripping agent and metal indicator. Negligible extraction of interferences and equilibrium enrichment of copper allowed for selective spectrophotometric determination of the Cu-PAR complex. Some important extraction parameters such as acceptor composition, shaking, equilibrium time, and sample volume were studied. The optimized methodology showed good linearity in the range of 5-100 microg/L, an enrichment factor of 93, good repeatability and reproducibility (RSDs < 6%, n = 6), and a detection limit of 4 microg/L. The cationic metals Ni2+, C(2+, Cd2+, Fe3+, Pb2+, Zn2+, and Mn2+ were shown not to interfere with the measurement of Cu2+. Measurements on samples containing mixtures of various ligands and cations were in good agreement with theoretically calculated concentrations, and the method was also applied to environmental samples. The developed technique requires less labor and less sophisticated equipment than conventional methods typically based on atomic absorption spectrometry or ICP.
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PMID:Equilibrium sampling through membranes of freely dissolved copper concentrations with selective hollow fiber membranes and the spectrophotometric detection of a metal stripping agent. 1631 67

A high-performance liquid chromatography (HPLC) method developed for analysis of zinc dithiocarbamates was validated and used to perform a survey of disposable medical gloves used in southern Sweden. The gloves were extracted with acetone at room temperature for 10 min by shaking. The extracts were injected into a polyether ether ketone lined HPLC column, and peaks were analysed by a diode-array detector. The survey shows that of 19 gloves analysed, 10 contained zinc diethyldithiocarbamate (0.070-3.5 mg/g), 3 contained zinc pentamethylenedithiocarbamate (1.0-4.3 mg/g), 4 contained zinc dibutyldithiocarbamate (0.9-1.1 mg/g), and 2 contained 2-mercaptobenzothiazole (0.005-0.008 mg/g). None of them contained thiurams.
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PMID:High-performance liquid chromatography analysis of rubber allergens in protective gloves used in health care. 1695 18

Infants and children may undergo severe oxidative stress due to disease state, pre-existing nutritional status, frequent use of oxygen, and lower levels of antioxidant defenses. Antioxidant defenses, made up of intracellular and extra-cellular components, work synergistically to prevent oxidative damage. Total antioxidant activity (TAA) was analyzed by method of ferric reducing antioxidant power assay (FRAP). Patients admitted in Pediatric Dept, RNT Medical College, Udaipur, India were selected for these studies. TAA level in neonates with hypoxic-ischemic-encephalopathy (HIE) stage III and in poor outcome cases was significantly low. Erythrocyte SOD activity level was low in pre-term neonates. TAA level in severely malnourished children at the time of hospital admission was low. This low antioxidant level in severely malnourished children could be multi-factorial viz. low zinc, selenium, vitamin A & C deficiency, recurrent infections, elevated free iron and chronic starvation stage. Delayed recovery of oxidant injury may lead to delayed incomplete recovery at cellular level. In a study of 29 tuberculosis patients TAA level was found to be low in tubercular patients compared with control. TAA level decreased more in CNS tuberculosis compared with other system tuberculosis. In a study of nutritional tremor syndrome TAA, ascorbic acid and alpha-tocopherol levels were low during pre-tremor phase compared with tremor phase (ATS). Pre-term neonates have incompletely developed antioxidant defenses and are deficient in vitamin E, which is normally derived from maternal circulation at the end of 3rd trimester. Therefore, decreased TAA level in HIE with poor outcome indicates addition of antioxidants in therapeutic strategy. Since rise in TAA in antioxidant supplemented group of severely malnutrition children was higher with good outcome compared with nonsupplemented group it would be prudent to supplement antioxidant during nutritional management. These studies have shown that health benefits can be obtained by children with a reduced risk of disease from supplements of antioxidant nutrients. The amounts of optimal supplements in these disorders, whether pharmacologic or large, are to be determined. Further work is needed to show whether modest increases in nutrient intakes in children with these disorders will delay or prevent the complications and improve the outcome. Therefore, available evidence regarding health benefits to be achieved by supplementing antioxidant nutrients is encouraging. Free radical injury and antioxidant deficiency is more common than what we think. Severely malnourished children and children suffering from chronic infections and diseases are at several fold increased risk of antioxidant deficiency and likely to suffer from free radical injury. Appropriate interventions are required in reducing the risk associated with these observations.
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PMID:Free radicals: emerging challenge in environmental health research in childhood and neonatal disorders. 1696 76

A stable chelating sorbent was synthesized by covalently linking 4-hydroxytoluene or 4-hydroxyacetophenone with the polyurethane foam (PUF) through -N=N- group. The synthesized chelating sorbents were characterized by IR and UV/vis measurements. The modified foams show excellent stability towards various solvents. Factors influencing the extraction process of Zn(II), Pb(II), Cd(II) and Hg(II) were studied and evaluated as a function of pH of metal ion solution and equilibration shaking time. The values of sorption capacity of metal ions (microg g(-1)) were determined with the two types of bonded foams. The two phenolic bonded foams were studied comparatively. The potential applications of the two newly synthesized foams for the removal and separation of the examined metal ions from two natural water samples (drinking tap water and Qaroun lake water at Fayoum City, Egypt) were investigated. Precision (assessed as a relative standard deviation, R.S.D.) was also evaluated and found to be < or =7.3% (N=5) with a detection limit under 0.46 microg L(-1).
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PMID:Separation and determination of trace amounts of zinc, lead, cadmium and mercury in tap and Qaroun lake water using polyurethane foam functionalized with 4-hydroxytoluene and 4-hydroxyacetophenone. 1772 43

The rat granular retrosplenial cortex (GRS) is a simplified cortex, with distinct stratification and, in the uppermost layers, distinct modularity. Thalamic and cortical inputs are segregated by layers and in layer 1 colocalize, respectively, with apical dendritic bundles originating from neurons in layers 2 or 5. To further investigate this organization, we turned to reelin-deficient reeler mouse and Shaking rat Kawasaki. We found that the disrupted lamination, evident in Nissl stains in these rodents, is in fact a patch-matrix mosaic of segregated afferents and dendrites. Patches consist of thalamocortical connections, visualized by vesicular glutamate transporter 2 (VGluT2) or AChE. The surrounding matrix consists of corticocortical terminations, visualized by VGluT1 or zinc. Dendrites concentrate in the matrix or patches, depending on whether they are OCAM positive (matrix) or negative (patches). In wild-type rodents and, presumably, mutants, OCAM(+) structures originate from layer 5 neurons. By double labeling for dendrites (filled by Lucifer yellow in fixed slice) and OCAM immunofluorescence, we ascertained 2 populations in reeler: dendritic branches either preferred (putative layer 5 neurons) or avoided (putative supragranular neurons) the OCAM(+) matrix. We conclude that input-target relationships are largely preserved in the mutant GRS and that dendrite-dendrite interactions involving OCAM influence the formation of the mosaic configuration.
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PMID:Unusual patch-matrix organization in the retrosplenial cortex of the reeler mouse and Shaking rat Kawasaki. 1772 62

Simple field-screening methods are presented for detecting 2,4,6-TNT, 2,4-DNT and RDX in soil. A 20-g portion of soil is extracted by manually shaking with 100 ml of acetone for three minutes. After the soil settles, the supernatant is filtered and divided into three aliquots. Two aliquots are reacted with potassium hydroxide and sodium sulfite to form the red-colored Janowsky complex when 2,4,6-TNT is present or the blue-purple complex when 2,4-DNT is present. The third aliquot of the extract is passed through a strong anion exchange resin to remove nitrate and nitrite. Then the extract is acidified and RDX is reduced with zinc to nitrous acid, which is reacted with a Griess reagent to produce a highly colored azo dye. Concentrations of TNT, 2,4-DNT and RDX are estimated from their absorbances at 540, 570 and 507 nm, respectively. Detection limits are about 1 microg/g for 2,4,6-TNT and RDX and about 2 microg/g for 2,4-DNT. Concentration estimates from field analyses correlate well with laboratory analyses.
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PMID:Development of field screening methods for TNT, 2,4-DNT and RDX in soil. 1896 97


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