Summary
Post-mortem atomic absorption spectrophotometry of the myocardium of a 14-year-old boy revealed a hundred-fold increase in copper. Further electrolyte analysis of the myocardium showed changes corresponding to metabolic heart muscle damage. Ultrastructural examination showed all the feature of a cardiomyopathy at the cellular level. Laser-Microprobe-Mass-Analysis demonstrated an inhomogeneous distribution of copper. An essential factor in the mechanism of death is heart damage.
Zusammenfassung
Im Myokard eines 14jährigen Jungen mit Morbus Wilson ergab die postmortale Gewebsmineralanalyse eine ca. 100 fache Erhöhung des Kupfergehaltes. Das Gesamtgewebsionogramm der Herzmuskulatur entsprach in seiner Konstellation einer Myokardose. Die ultrastrukturellen morphologischen Veränderungen waren für eine Kardiomyopathie charakteristisch. Im Laser-Mikrosonden-Massenanalysator LAMMA 500 fand sich eine inhomogene Kupferverteilung. Der Herzmuskelschaden hat offensichtlich eine wesentliche Beteiligung am Todesmechanismus.
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Kaduk, B., Metze, K., Schmidt, P.F. et al. Secondary athrocytotic cardiomyopathy — Heart damage due to Wilson's disease. Virchows Arch. A Path. Anat. and Histol. 387, 67–80 (1980). https://doi.org/10.1007/BF00428430
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DOI: https://doi.org/10.1007/BF00428430