[Controlling the binding of porphyrins to ligands using antimalarial agents].
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Biomedical subjects
Publications and source records attributed to Z Marecek.
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This paper reports on a study of the heterozygous children of patients with Wilson's disease. A total of 16 children of 10 patients with the disease were followed up. Detailed biochemical, clinical and EEG tests were done. Nearly all the children were found to have reduced serum copper and caeruloplasmin levels and high rates of urine copper excretion following exposure to penicillamine. These findings were different from the results obtained in adult heterozygous carriers. Thirty per cent of the children had pathological neurological findings, and EEG abnormalities were found in 75%.
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In patients with chronic renal failure the administration of a diet providing 20 g protein per day for five weeks caused a significant rise in the serum ceruloplasmin level. A low dietary copper content (0.8 mg/day) may have been the limiting factor for any further rise of the ceruloplasmin level. The rise of ceruloplasmin was associated with a continuous decline of total iron binding capacity. The temporarily raised serum iron levels and the continuous decline of unsaturated iron binding capacity suggested enhanced iron mobilization caused by the raised ceruloplasmin level. The rise of ceruloplasmin correlated with the decline of UIBC.
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