Wilson's disease (also called hepatolenticular degeneration) is usually a genetic problem characterized by the excess build-up of copper in your body. Indications are generally related to the Mind and liver.
Les professionnels de la santé doivent effectuer des analyses notamment pour connaître le taux de cuivre que ce soit dans le foie ou les urines. Pour le premier organe, l’intervention est un peu plus complexe puisque le médecin doit effectuer une biopsie à l’aide d’une aiguille.
Il faut un intervalle d’au moins deux heures entre la prise éventuelle de zinc et celle de pénicillamine ou de trientine, parce que le zinc peut se lier à ces médicaments et les rendre inopérants. Les personnes contactées par la maladie de Wilson doivent prendre pendant leur vie entière de la pénicillamine, de la trientine, du zinc ou une combinaison de ces agents.
A brown ring on the edge on the iris (Kayser–Fleischer ring) is common in Wilson's disease, specially when neurological indications are existing. Professional medical disorders are joined with copper accumulation in Wilson's disease:
Les premiers symptômes de la maladie débutent rarement avant l’âge de 3 ans et varient selon les organes have an effect onés :
On assessment, indications of Continual liver disease for instance spider angiomata (smaller distended blood vessels, typically around the chest) might be noticed. Serious active hepatitis has by now caused cirrhosis of the liver in most patients by the point they create symptoms. While most individuals with cirrhosis have an elevated hazard of hepatocellular carcinoma (liver read more cancer), this danger is fairly lower in Wilson's disease.[five]
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Atteinte des yeux : la maladie est responsable chez 75% des malades de la formation d’un anneau orangé au bord externe de l’iris : l’anneau de Kayser-Fleischer.
En cas d’intoxication au cuivre chez le chien, la gestion de la problem doit se faire à deux niveaux : individuellement et au niveau de la race. Pour gérer individuellement un chien atteint de la maladie de Wilson, il est critical :
The normal gene will wind up managing the effects in the irregular gene and besides, one particular gene is sufficient to Regulate the speed at which copper is absorbed by your body.
There are several hereditary diseases that bring about copper overload from the liver; Wilson's disease is the commonest of them. All can result in cirrhosis at a youthful age. The other copper overload diseases are Indian childhood cirrhosis (ICC), endemic Tyrolean infantile cirrhosis, and idiopathic copper toxicosis.
The first set of indicators to be skilled are linked to the liver. It is because the harmful impact on the copper will First off strike the liver and then start to cause liver issues.
When the quantity of copper within the liver overwhelms the proteins that normally bind it, it results in oxidative damage to the liver by way of a method referred to as Fenton chemistry; this destruction finally results in Serious active hepatitis, fibrosis (deposition of connective tissue), and cirrhosis. The liver also releases copper in the bloodstream that's not bound to ceruloplasmin. This cost-free copper precipitates throughout the entire body, but significantly inside the kidneys, eyes, and brain.