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[Charles II: From spell to genitourinary pathology].

Angel García-Escudero López1, A Arruza Echevarría, J Padilla Nieva

  • 1Servicio de Urología, Hospital Txagorritxu, Vitoria, Alava, España. agareslo@telefonica.net

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|June 23, 2009
PubMed
Summary

The Hapsburg dynasty ended with Charles II due to his complex genetic and genitourinary disorders, likely including hypospadias and a XX male syndrome, leading to infertility and death. Further genetic studies of his remains could clarify his condition.

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Area of Science:

  • Medical history
  • Genetics
  • Endocrinology

Background:

  • Charles II of Spain, the last Hapsburg monarch, suffered from a debilitating genitourinary disorder that prevented him from producing an heir.
  • His condition was exacerbated by a history of consanguineous marriages within the Hapsburg dynasty, leading to widespread speculation and the belief he was 'bewitched'.

Discussion:

  • Analysis suggests Charles II may have had posterior hypospadias, monorchism, and an atrophic testicle, contributing to an intersex state with ambiguous genitalia.
  • His phenotype aligns more with true hermaphroditism or XX male syndrome, possibly with Fragile X syndrome, rather than Klinefelter's syndrome.
  • Congenital monorenal condition and chronic kidney failure due to nephropathy and recurrent urinary tract infections likely caused his death.

Key Insights:

  • The Hapsburg dynasty's end in Spain was precipitated by Charles II's complex pluripathological condition.
  • Endogamic matrimonial policies significantly contributed to the Habsburg dynasty's extinction.
  • Genetic and chromosomal analysis of Charles II's remains is crucial for definitive diagnosis.

Outlook:

  • Further research into Charles II's genetic makeup could provide definitive answers regarding his intersex condition.
  • Understanding the genetic impact of royal inbreeding offers insights into historical health outcomes.
  • The case of Charles II highlights the long-term consequences of consanguinity on dynastic survival.