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Archives of Industrial Hygiene and Toxicology

The Journal of Institute for Medical Research and Occupational Health

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Volume 62, Issue 2 (Jun 2011)

Issues

Work Ability Assessment in a Patient with Wilson's Disease

Martin Popević / Gordana Kisić / Milenko Đukić / Petar Bulat
Published Online: 2011-06-24 | DOI: https://doi.org/10.2478/10004-1254-62-2011-2102

Work Ability Assessment in a Patient with Wilson's Disease

Wilson's disease (WD) is a rare, progressive autosomal recessive disorder characterised by impaired transport and excessive accumulation of copper in the liver, brain, and other tissues. The disease is diagnosed based on clinical manifestations and screening tests results. Work ability assessment of patients with WD is based on the analysis of liver, kidney, neurological, and cognitive impairments, and takes into account patient's level of education.

This article presents a case with a 48-year-old male patient, who was admitted for work ability assessment due to polymorphic symptoms. The patient had been working as a salesman for 28 years. A detailed interview and examination by occupational health and other medical specialists revealed that the patient had been suffering from Wilson's disease from the age of 13, and had now developed hepatic manifestations (compensated liver cirrhosis with portal hypertension), neurological manifestations (dystonia, dysarthria, muscle weakness, vertigo), and psychiatric manifestations (depression, insomnia, cognitive impairment) of the disease, including problems partially caused by long-lasting treatment with copper chelating agents (neurological and haematological manifestations). There were no ocular manifestations of Wilson's disease (Kayser-Fleischer rings or sunflower cataract).

The patient was assessed as having drastically diminished general work ability, dominantly due to neurological and psychiatric impairments caused by Wilson's disease.

Ocjena radne sposobnosti pacijenta s wilsonovom bolesti - prikaz bolesnika

Wilsonova je bolest rijetka, progresivna autosomno recesivna bolest karakterizirana poremećajem transporta bakra i posljedičnim prekomjernim nakupljanjem bakra u jetri, mozgu i drugim tkivima i organima. Dijagnoza bolesti postavlja se na osnovi kliničkih manifestacija bolesti i nalaza laboratorijskih ispitivanja. Ocjena radne sposobnosti pacijenata s Wilsonovom bolesti zasniva se na analizi postojanja oštećenja i stupnja oštećenja hepatičkih, neuroloških, bubrežnih i kognitivnih funkcija, kao i na analizi stupnja obrazovanja pacijenata.

Prikazan je slučaj D. M., 48-godišnjeg pacijenta, koji je primljen zbog polimorfnih tegoba na bolničko ispitivanje radi ocjene radne sposobnosti. Pacijent je radio kao prodavač posljednjih 28 godina. Nakon detaljne anamneze i pregleda koje su obavili specijalisti medicine rada i drugi specijalisti utvrđeno je da pacijent boluje od Wilsonove bolesti od 13. godine života i da u ovom trenutku ima izražene hepatične manifestacije (kompenzirana ciroza jetre s portalnom hipertenzijom), neurološke manifestacije (distonija, dizartrija, mišićna slabost, vrtoglavica) i psihijatrijske manifestacije (depresija, nesanica, kognitivno oštećenje) Wilsonove bolesti, kao i da su prisutne tegobe djelomično uzrokovane dugotrajnom upotrebom kelatne terapije (neurološki i hematološki poremećaji). Nisu uočene karakteristične očne promjene Wilsonove bolesti (Kayser-Fleischerov prsten, katarakta u obliku suncokreta).

Ocjenom radne sposobnosti utvrđeno je da pacijent ima drastično smanjenu radnu sposobnost pretežno zbog neuroloških i psihičkih poremećaja u sklopu Wilsonove bolesti.

Keywords: chelation therapy; cognitive impairment; copper; depression; disability evaluation; Dpenicillamine; dysarthria; dystonia; insomnia; Kayser-Fleischer rings; liver cirrhosis; muscle weakness; portal hypertension; psychiatric disorders; vertigo

Keywords: bakar; ciroza jetre; depresija; distonija; dizartrija; D-penicilamin; Kayser-Fleischerov prsten; kelatna terapija; kognitivno oštećenje; mišićna slabost; nesanica; portalna hipertenzija; vrtoglavica

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About the article


Published Online: 2011-06-24

Published in Print: 2011-06-01


Citation Information: Archives of Industrial Hygiene and Toxicology, ISSN (Print) 0004-1254, DOI: https://doi.org/10.2478/10004-1254-62-2011-2102.

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