Cox Timothy M, Jack Nicola, Lofthouse Simon, Watling John, Haines Janice, Warren Martin J
Department of Medicine, University of Cambridge Addenbrooke's Hospital, Cambridge, UK.
Lancet. 2005;366(9482):332-5. doi: 10.1016/S0140-6736(05)66991-7.
In 1969 it was proposed that the episodic madness suffered by King George III (1738-1820) resulted from an acute hereditary porphyria, variegate porphyria, caused by deficiency of protoporphyrinogen oxidase. The diagnosis was based on the historical archive and a contentious claim that living members of the House of Hanover were affected with the condition. A re-examination of the medical evidence and the appearance of new historical material have suggested that porphyria did indeed exist in the Royal Houses of Europe. We report the analysis of hair obtained from George III. Although no genomic DNA could be obtained, metal analysis revealed high concentrations of arsenic. Since arsenic interferes with haem metabolism, it might have contributed to the King's unusually severe and prolonged bouts of illness. We have identified sources of arsenic in the context of the medication George III received from physicians.
1969年有人提出，乔治三世（1738 - 1820）所患的间歇性精神错乱是由一种急性遗传性卟啉病——杂合性卟啉病引起的，该病是由于原卟啉原氧化酶缺乏所致。该诊断基于历史档案以及一项有争议的说法，即汉诺威家族的在世成员也患有此病。对医学证据的重新审视以及新历史资料的出现表明，卟啉病确实在欧洲王室中存在。我们报告了对从乔治三世处获取的头发的分析结果。尽管未能获得基因组DNA，但金属分析显示砷含量很高。由于砷会干扰血红素代谢，它可能是导致国王异常严重且持久疾病发作的原因之一。我们已在乔治三世从医生那里接受的药物背景下确定了砷的来源。
