Hereditary diseases among the nobility

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Hereditary diseases among the nobility are among the phenomena that are documented in some of the nobility's family trees . They are also the subject of individual scientific studies. The play endogamy among the nobility and cousin marriage associated with inbreeding in humans a certain role.

Inbreeding and Hereditary Diseases

Inbreeding among aristocrats and non-aristocrats can occur “as an effect of the small size or high isolation of a population - for example on a small island, in a mountain valley or even in a social ghetto”. The risk of recessive hereditary diseases breaking out is greatly increased with extreme ancestral decline .

The actor and writer Gregor von Rezzori (1914–1998) wrote in a book published in 1962 a. a .:

“The nobility undoubtedly shows more signs of degeneration than the paradoxical example of the high nobility. But princes traveled more. Sometimes they took their wives from another country. The majority of the high nobility is also Catholic, so they were saved from the worst by the canonical marriage laws which forbid marriages between close blood relatives. The old local nobility, however, was relegated to the neighborhood with which they had been related by blood for centuries. But beyond prognathy and strange skull shapes, flat feet, which are very common, character heads from early youth and similar harmless deformities, the effects of great inbreeding do not go beyond the small nobility. Certainly, it can be presumed that cousins ​​who have the same fool as their grandfather, if they copulate with one another, will more likely give birth to the same fool than a genius. However, calculation errors can creep in there too. And the percentage of idiots in the nobility is very high, but not higher than in the common people. "

The historian David Sabean pointed out in 2010 that marriages with first or second cousins ​​in Europe between 1740 and the beginning of the 20th century in both Catholic and Protestant societies in the wealthy groups, i.e. from peasants to commoners to the nobility, were very common.

The genealogist Hans Peter Stamp examined 12,531 European, above all noble, full sibling groups from 17 centuries and came to the conclusion in 1999 that even with this subgroup of the nobility, more inbreeding was “the exception rather than the rule”. "Of the 12531 groups, 10587 had an inbreeding coefficient of less than 1%, a majority of them even 0%."

A study led by the geneticist Francisco Ceballo from 2013 deals with the theory that in the years 1450 to 1800, despite inbreeding, there was a reduction in inbreeding depression (" purging ") because many of those affected were already in childhood and Infancy died and thus never reached a fertile age. According to Ceballo, the findings of the study show that purging could have eliminated the negative effects of inbreeding on some fitness components .

High nobility

The European dynastic nobility had restrictive marriage rules. The exaggerated requirement of equality and a foreign policy based on family relationships should help to maintain political influence and economic potency within the family. In addition, for a long time marriages could only be concluded within one's own religious community , so that between the 16th and 19th centuries there were more or less closed Catholic, Lutheran and Reformed “marriage circles”. Ideologically, these restrictive marriage rules were exaggerated by the belief in a “divine power” of “good blood”, which, it was believed, was reinforced by marriage and procreation with owners of equal or equal “noble” blood. This ideology, which is also believed by the people, goes back to the expressions still used today such as “of noble blood” or “blue blood”.

The canon law of the Catholic Church forbade marriages between close relatives; However, she almost always made use of her prerogative of an exemption (papal dispensation ) for members of the high nobility and lifted the obstacle to marriage .

The best-known hereditary diseases widespread among the European nobility are hemophilia (blood disease) and intellectual disability . The high number of marriages in close and closest relatives is assumed to be the cause of the extinction of some large European dynasties (especially the Spanish branch of the House of Habsburg ).

House of Habsburg

A study led by the geneticist Gonzalo Álvarez, Santiago de Compostela, from 2008 dealt with the historical hypothesis that inbreeding was the reason for the extinction of the Spanish Habsburgs (1516-1700) and came after the investigation of more than 3000 individuals from 16 generations to the result that the inbreeding coefficient of the Spanish Habsburgs increased from 0.025 for King Philip I , the founder of the dynasty, to 0.254 for Charles II and that towards the end of the dynasty several members had an inbreeding coefficient of over 0 , 20 had.

Álvarez came to the conclusion that this high inbreeding coefficient was not only responsible for the outward appearance of the representatives of this line of the Habsburgs (including " Habsburg Lippe "), but also for infertility and increased mortality . Ultimately responsible for the final extinction of the Spanish Habsburgs, according to the study, was the "coincidence" of two hereditary diseases in Charles II. Charles died without children; the War of the Spanish Succession followed .

Hemophilia in the British royal family

Inheritance of hemophilia among Queen Victoria's descendants

Leopold (1853-1884), son of Queen Victoria of Great Britain and Prince Albert of Saxe-Coburg and Gotha , was the first member of the British royal family to suffer from hemophilia , here hemophilia B. Hemophilia B is an X-linked hereditary disease.

There were also cases in the House of Hesse . The Tsar's son Alexej (1904–1918) was hemophiliacs. Alfons (1907–1938) and Gonzalo (1914–1934), sons of the Spanish King Alfonso XIII. , also suffered from hemophilia. The Prussian Prince Waldemar (1889–1945) and his brother Heinrich Viktor Ludwig Friedrich (1900–1904) were affected . The reason is a spontaneous mutation, which probably goes back to Queen Victoria.

Porphyria

Macalpine and Hunter suspected in 1966 that George III. suffered from the hereditary disease porphyria , which affected the houses of Stuart , Hanover and Prussia . At least in his descendant Wilhelm von Gloucester , this disease was detected in the form of Porphyria variegata .

literature

  • Hans-Joachim Neumann : Hereditary diseases in European royal houses: Habsburg, Hohenzollern, Romanow, Welfen, Wettiner, Bourbon. Bechter coin 2002.

Individual evidence

  1. ↑ Antje Schmelcher: You don't talk about (and research) about it. In: FAZ.net . June 6, 2011, accessed January 9, 2015 .
  2. ^ Gregor von Rezzori: Idiot leader through the German society. Volume 1: high nobility; Volume 2: Nobility. Rowohlt Verlag (1962)
  3. ^ Welcome to UCLA's History Department: David Warren Sabean. In: history.ucla.edu. Retrieved January 9, 2015 .
  4. Jump up ↑ David Warren Sabean: On the Walk of Incest . Online at: Science.orf.at, Kulturanthropologie, May 17, 2010, accessed on March 9, 2013.
  5. ^ Hans Peter Stamp: Inbreeding in the European nobility, 12531 full sibling groups in the test. Retrieved May 26, 2011.
  6. ^ FC Ceballos, G. Alvarez: Royal dynasties as human inbreeding laboratories: the Habsburgs. In: Heredity. Volume 111, number 2, August 2013, ISSN  1365-2540 , pp. 114-121, doi : 10.1038 / hdy.2013.25 . PMID 23572123 . PMC 3716267 (free full text).
  7. ↑ DNA Identification Of Czar's Children Available . ScienceDaily. February 27, 2009. Retrieved June 21, 2013.
  8. ↑ a b c G. Alvarez, FC Ceballos, C. Quinteiro: The role of inbreeding in the extinction of a European royal dynasty. In: PloS one. Volume 4, number 4, 2009, ISSN  1932-6203 , p. E5174, doi : 10.1371 / journal.pone.0005174 . PMID 19367331 . PMC 2664480 (free full text).
  9. ↑ Victorian Suffering . In: Der Spiegel . No. 25 , 1961 ( online ).
  10. ↑ a b I. Macalpine, R. Hunter: The "insanity" of King George 3d: a classic case of porphyria. In: British medical journal. Volume 1, Number 5479, January 1966, ISSN  0007-1447 , pp. 65-71, PMID 5323262 . PMC 1843211 (free full text).
  11. ↑ Macalpine I, Hunter R, Rimington C: Porphyria in the Royal Houses of Stuart, Hanover, and Prussia. A follow-up study of George 3d's illness . In: Br Med J . 1, No. 5583, January 1968, pp. 7-18. doi : 10.1136 / bmj.1.5583.7 . PMID 4866084 . PMC 1984936 (free full text).
  12. ↑ Martin Warren: Tetrapyrroles. Springer Science & Business Media, 2009, ISBN 978-0-387-78518-9 , p. 21 ( limited preview in Google book search).