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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
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
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.
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
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
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
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
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
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
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
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
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 (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).
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
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