Difficulties in managing patients with hepatolenticular degeneration
In this paper, presented some typical problems of managing patients with hepatolenticular degeneration (HLD), a severe hereditary disorder of cupper metabolism requiring permanent cuppereliminating therapy. Special attention should be paid the following questions: choice of combined treatment with Dpenicillamine (DPAM) and zinc vs. monotherapy with either of these medications; the possibility of temporary worsening of neurologic symptoms induced by massive increase in free cupper level in the blood following therapy with DPAM; the need for special psychological support of patients and their relatives; strict adherence to a regimen prohibiting physical exercises and the need for following a special diet. Two clinical cases have been described: a fatal case of manyyear compensated HLD after a physical load during a travel to the seaside, and a case of an infected foot wound in a HLD patient with the development of septicopyaemia (lower leg phlegmon and osteomyelitis of the lumbar vertebrae) following a physical activity and violation of the dietary pattern.