hepatolenticular degeneration/carbohydrate

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Lipid and energy metabolism in Wilson disease

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Copper accumulation and deficiency are reciprocally connected to lipid metabolism. In Wilson disease (WD), which is caused by a genetic loss of function of the copper-transporting P-type ATPase beta, copper accumulates mainly in the liver and lipid metabolism is dysregulated. The underlying

[The new aspects of clinical nutrition at Wilson disease: actuality and perspectives].

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Wilson disease is hereditary disorder of copper metabolism, based on defect of cooper excretion, which leads to accumulation of cooper in the liver and brain. This disease is one of the most difficult to diagnose. Without treatment disease brings to early disability and lethal outcome. In the

Copper absorption and bioavailability.

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The human gastrointestinal system can absorb 30-40% of ingested copper from the typical diets consumed in industrialized countries. Experimental data support the existence of a carrier-mediated transport mechanism with an affinity constant in the micromolar range. Aging probably decreases the

Interrelation between structure and protective action of normal and pathological ceruloplasmins during copper-induced lysis of red blood cells.

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The origin of the difference between the protective action of ceruloplasmin (CP) from healthy donors blood and of ceruloplasmin-like protein (p-CP) from blood of patients with Wilson disease which they exert during copper-induced lysis of red blood cells (RBC) was elucidated. The difference is due

Diagnostic utility of laboratory tests in alcoholic liver disease.

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Alcoholic liver disease is an important health problem in the US. A public health approach is proposed, using laboratory tests to identify patients with early fibrosis of the liver. A variety of serological markers of liver fibrosis based on collagen-related products (e.g., amino-terminal

The use Prometheus FPSA system in the treatment of acute liver failure: preliminary results.

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The preliminary outcomes of patients with acute liver failure treated with the Prometheus Fractionated Plasma Separation and Absorption (FPSA) system are presented herein. METHODS The procedures were performed in 13 patients (4, intoxication by Amanita phalloides; 4, unknown reason; 3, acetaminophen

Metal-induced hepatotoxicity.

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Figure 3 summarizes several proposed mechanisms of iron- or copper-induced hepatotoxicity. It has long been suspected that free radicals may play a role in iron- and copper-induced cell toxicity because of the powerful prooxidant action of iron and copper salts in vitro. In the presence of available

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