hepatolenticular degeneration/albumin

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Albumin dialysis: effective removal of copper in a patient with fulminant Wilson disease and successful bridging to liver transplantation: a new possibility for the elimination of protein-bound toxins.

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BACKGROUND Acute liver failure may be the first manifestation of Wilson disease. If copper elimination fails, liver transplantation is the only remaining therapeutic option. Albumin dialysis, a new method for the removal of protein-bound toxins, was performed in a patient with fulminant Wilson

Reference intervals of spot urine copper excretion in preschool children and potential application in pre-symptomatic screening of Wilson disease.

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The objectives were to determine the reference intervals of spot urine copper excretion indexes in pre-school children and to evaluate their utility in screening for Wilson disease (WD). With spot urine collected from a control sample of preschool children (aged 3-7 years, n=153), the reference

WTX101 - an investigational drug for the treatment of Wilson disease.

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BACKGROUND Wilson disease (WD) is a genetic disorder in which excess toxic copper accumulates in the liver, brain, and other tissues leading to severe and life-threatening symptoms. Copper overload can be assessed as non-ceruloplasmin-bound copper non-ceruloplasmin-bound copper (NCC) in blood.

Structural changes and metal binding by proalbumins and other amino-terminal genetic variants of human serum albumin.

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Proalbumins are rare genetic variants of human serum albumin containing a basic propeptide that is not removed during post-transcriptional processing because of a mutation in the site of excision, an Arg-Arg sequence. We have identified the amino acid substitutions in three different types of

Serum 'free' copper in Wilson disease.

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BACKGROUND The relationship between serum 'free' copper and urine copper in patients with Wilson disease has not been explored. OBJECTIVE The object of this study is to ascertain if there is a direct relationship between these two parameters. METHODS The case notes of 320 patients with Wilson

[Study on improvement of liver cirrhosis and liver function in hepatolenticular degeneration patients treated with integrated Traditional and Western medicine].

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OBJECTIVE To observe the effects of sodium dimercaptosulphonate (DMPS) plus Gandou tablet, DMPS and calcium disodium ethylene diaminotetraacetate (EDTA) on improving liver cirrhosis and liver function of hepatolenticular degeneration (HLD) patients. METHODS One hundred and forty-six HLD patients

Iatrogenic copper deficiency associated with long-term copper chelation for treatment of copper storage disease in a Bedlington Terrier.

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A 9-year-old Bedlington Terrier was evaluated because of weight loss, inappetence, and hematemesis. Copper storage disease had been diagnosed previously on the basis of high hepatic copper concentration. Treatment had included dietary copper restriction and administration of trientine for chelation

Metabolic disposition of WTX101 (bis-choline tetrathiomolybdate) in a rat model of Wilson disease.

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1. WTX101 (bis-choline tetrathiomolybdate) is an investigational copper (Cu)-protein-binding agent developed for the treatment of Wilson disease (WD), a rare genetic disorder caused by mutations in the ATP7B Cu-transporter and resulting in toxic Cu accumulation. 2. Mass balance of a single

Molybdenum and copper kinetics after tetrathiomolybdate injection in LEC rats: specific role of serum albumin.

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Chelation therapy with tetrathiomolybdate (TTM) was applied to Long-Evans rats with a cinnamon coat-color (LEC rats), an animal model for Wilson disease, to remove copper (Cu) accumulated in the liver in a form bound to metallothionein (MT). Changes in molybdenum (Mo) and Cu concentrations and their

[Efficacy of microencapsulated HepG2 cells transplantation in rats with hepatolenticular degeneration].

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OBJECTIVE To evaluate the efficacy of intraperitoneal transplantation of microencapsulated HepG2 cells in rats with hepatolenticular degeneration (HLD). METHODS HLD was induced by copper-overloaded diet with forage containing 1 g/kg copper sulfate and water with 0.185% copper sulfate for 12 weeks in

Diminution of toxic copper accumulation in toxic milk mice modeling Wilson disease by embryonic hepatocyte intrasplenic transplantation.

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OBJECTIVE To observe the therapeutic effect of intrasplenic transplantation with embryonic hepatocytes on amelioration of hereditary copper accumulation in toxic milk (TX) mouse modeling Wilson disease. METHODS Donor hepatocytes were harvested from 14-d fetal liver of a pregnant homogeneous DL

[Evaluating the efficacy of diet therapy with protein component modification at Wilson disease]

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Wilson disease (WD) is a rare hereditary disorder of copper metabolism, based on of the ATP7B gene mutation, resulting in defect of cooper excretion, which leads to accumulation of cooper in tissues and internal organs (especially in the liver and brain). The basic principle of diet therapy for

Disordered copper metabolism in LEC rats, an animal model of Wilson disease: roles of metallothionein.

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Disordered copper (Cu) metabolism in LEC rats, an animal model of Wilson disease, was characterized by specifying Cu in the liver, bloodstream and kidneys during the accumulation process and at the onset of jaundice; Cu accumulates in the liver with age in a form bound to metallothionein (MT).

Effect of molecular adsorbents recirculating system treatment in children with acute liver failure caused by Wilson disease.

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OBJECTIVE Because fulminant Wilson disease (WD) has an extremely poor prognosis, the use of liver support that can bridge patients to liver transplantation is lifesaving. We report the experience of albumin dialysis in acute liver failure (ALF) caused by WD in children. METHODS Chart review of

Urinary abnormalities in children and adolescents with Wilson disease before and during treatment with d-penicillamine.

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Renal abnormalities can occur at any time point during the course of Wilson disease (WD). We aimed to fill a literature gap in this respect by studying urinary abnormalities in children and adolescents with WD.This study included 60 children with WD

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