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Wilson-Konovalov disease: prognosis

 
, medical expert
Last reviewed: 19.10.2021
 
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Without treatment, Wilson's disease progresses and leads to the death of patients. The most dangerous situation is when the disease remains unrecognized and the patient dies without receiving treatment.

In acute neurological form, the prognosis is unfavorable, since cystic changes in basal nuclei are irreversible. In chronic course, the prognosis depends on the early diagnosis, preferably before the onset of symptoms. Significant prognostic value has the results of a 6-month continuous therapy with penicillamine. In one study, treatment in 16 patients with asymptomatic disease allowed not only to save their lives, but also to eliminate clinical manifestations of the disease. In addition, in 16 out of 22 patients with clinical manifestations of Wilson's disease, more than two years of treatment resulted in the disappearance of symptoms. At a dystonia the forecast less favorable, as treatment by chelators is ineffective. There have been described cases of normal pregnancy wear during the successful treatment of Wilson's disease, and the negative effect of penicillamine on the fetus has not been noted.

In chronic hepatitis, treatment may be ineffective. So, in one of the studies 9 of 17 patients died. Disease with fulminant current often ends with the death of the patient, despite the treatment with chelators. Adverse symptoms are jaundice, ascites, high levels of bilirubin and high activity of ASAT in the serum and an increase in prothrombin time. Liver transplantation can save life for such patients.

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