Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_45_библиотеки_им_акад_М_И_Перельмана

ranges between 40 and 70% (Bergstrom 1981). Many studies have shown that penicillamine reduces copper accumulation and provides clinical beneit in WD (Walshe 1973, Grand 1975, Sternlieb 1980). Signs of liver disease oten regress during the initial 6 months of treatment. Non-compliance has been shown to cause progression of liver disease, liver failure, death and LTX (Scheinberg 1987). However, neurological symptoms may deteriorate at the start of penicillamine treatment; it remains controversial how oten this neurological deterioration occurs and whether it is reversible; the rate of neurological worsening ranges from 10–50% in diferent cohorts (Brewer 1987, Walshe 1993). Some authors even recommend not using penicillamine in WD patients with neurological disease (Brewer 2006). Penicillamine is associated with many side efects that lead to its discontinuation in up to 30% of patients (for literature see Roberts 2003). An early sensitivity reaction may occur during the irst three weeks including fever, cutaneous exanthema, lymphadenopathy, neutropenia, thrombocytopenia and proteinuria. In such early sensitivity, penicillamine should be replaced by trientine immediately. Nephrotoxicity is another frequent side efect of penicillamine, which occurs later and includes proteinuria and signs of tubular damage. In this case penicillamine should be immediately discontinued. Penicillamine may also cause a lupus-like syndrome with hematuria, proteinuria, positive antinuclear antibody and Goodpasture’s Syndrome. More rarely the drug can damage the bone marrow leading to thrombocytopenia or total aplasia. Dermatologic side efects include elastosis perforans serpiginosa, pemphigoid lesions, lichen planus and aphthous stomatitis. There have also been reports of myasthenia gravis, polymyositis, loss of taste, reduction of IgA and serous retinitis due to administration of penicillamine.