![]() ![]() Rushton has not taken on board these serious doubts and has based his book on a diagnosis of variegate porphyria in the King. Only recently has a detailed re-evaluation of the King's medical records shown that the claims of Macalpine and Hunter and their adherents are seriously flawed and they were guilty of being ‘economical with the truth’. In spite of authoritative and well argued doubts expressed at the time by experts in the field, this dubious claim has entered the canon of historical fact and is a widely-accepted interpretation that features in academic and popular history books, television documentaries and notably Alan Bennett's award-winning In a second paper they altered the diagnosis, again categorically, to the milder condition variegate porphyria on the history of skin lesions in the King (adolescent acne, an instance of sunburn and a possible episode of urticaria). Rushton bases his diagnosis of porphyria on the work of the psychiatrists and amateur historians, mother and son duo, Ida Macalpine and Richard Hunter, who categorically stated that King George III was not ‘mad’ at all but suffered from the undiagnosed medical condition acute intermittent porphyria. These steps are not shown and thus the reader is confused on the nature of the defect in acute intermittent porphyria and its key relationship to the symptomatology of variegate porphyria. In 1980 Battersby and colleagues clarified the steps between porphobilinogen and the uroporphyrins. ![]() Thus he attempts to outline the pathway of haem biosynthesis, vital to the understanding of the nature, severity and cause of the acute porphyrias. There are serious errors both of omission and comission. The major part of Rushton's study (84 pages) is, however, devoted to variegate porphyria. More discussion on paternal age effects, mechanism of X chromosome inactivation and the molecular genetics of haemophilia would increase the value of this aspect of the book. A new mutation in Queen Victoria's elderly father Edward, Duke of Kent (aged 50 when she was conceived) is postulated quite reasonably as the source of the problem. Nevertheless it would be useful, for the medical and non-medical readership, to have more detail about the process of the blood clotting and, in particular, the nature of the clotting-defect in the families (haemophilia A or B). He examines the effects of haemophilia on the royal houses in Russia and Spain with devastating political consequences for both. On haemophilia (16 pages) the author is on reasonably firm ground. ![]() This book discusses two familial diseases affecting the royal houses of Europe: haemophilia and variegate porphyria. ![]()
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