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Mandibular prognathism
« on: May 11, 2015, 11:39:09 AM »
Pathologic mandibular prognathism is a potentially disfiguring genetic disorder where the lower jaw outgrows the upper, resulting in an extended chin. It is sometimes a result of acromegaly.

This condition is colloquially known as Habsburg jaw, Habsburg lip or Austrian Lip (see House of Habsburg) due to its prevalence in that bloodline. The trait is easily traceable in portraits of Habsburg family members.[5] This has provided tools for people interested in studying genetics and pedigree analysis. Most instances are considered polygenetic.

It is alleged to have been derived through a female from the princely Polish family of Piasts, its Masovian branch. The deformation of lips is clearly visible on tomb sculptures of Mazovian Piasts in the St. John's Cathedral in Warsaw. However this may be, there exists evidence that the trait is longstanding. It is perhaps first observed in Maximilian I (1459–1519).

Traits such as these that were common to royal families are believed to have been passed on and exaggerated over time through royal intermarriage which caused acute inbreeding. Due to the large amount of politically motivated intermarriage among Habsburgs, the dynasty was virtually unparalleled in the degree of its inbreeding. Charles II of Spain is said to have had the most pronounced case of the Habsburg jaw on record. His jaw was so deformed that he was unable to chew.

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Re: Mandibular prognathism
« Reply #1 on: May 11, 2015, 11:41:54 AM »
tumblr_inline_mpogh4jOR21qz4rgp - Mandibular prognathism - Science and Research


Also called the Habsburg Lip and the Austrian Lip, the Habsburg Jaw is a physical condition known by the modern term mandibular prognathism. It is characterized by a jutting lower jaw, often accompanied by an abnormally thick lower lip and sometimes including a tongue that is abnormally large.

The House of Habsburg is associated with the Habsburg Jaw because so many of its members had it. It really became pronounced in the line of Spanish Habsburgs, and specifically because of inbreeding. -- http://yveinthesky.tumblr.com/

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Re: Mandibular prognathism
« Reply #2 on: May 11, 2015, 12:38:46 PM »

charles-ii-of-spain_mainstory1 - Mandibular prognathism - Science and Research
Charles II (1661-1700), the last Habsburg King of Spain had the most extreme case of Habsburg Jaw recorded.  He was also mentally disabled. An analysis of the previous marriages that led to his birth reveals multiple uncle-niece marriages and first cousin marriages.
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Re: Mandibular prognathism
« Reply #3 on: May 11, 2015, 12:50:29 PM »

How keeping it in the family spelled the end of the line for the Hapsburg royal dynasty

By DAILY MAIL REPORTER
15 April 2009


article-1170143-04761CE1000005DC-77_233x423 - Mandibular prognathism - Science and Research
Charles II who is believed to have suffered from two inherited disorders which prevented him from fathering an heir. This resulted in the end of the Hapsburg dynasty

The Hapsburg dynasty, one of the most influential and celebrated in Europe was driven to extinction because of inbreeding, say researchers.

The kings who ruled Spain and its empire from 1516 for almost 200 years during the most glorious period in its history frequently married close relatives such as nieces and first cousins. This in turn led to ill health and a high rate of infant and child mortality.

The first scientific evidence for the significance of inter-marriage in this major European family shows that down the generations successors to the throne were much more likely to receive copies of two identical 'homozygous' genes, one from each parent.

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Re: Mandibular prognathism
« Reply #4 on: May 11, 2015, 12:53:52 PM »

Mothers and fathers who share their ancestry make their offspring more vulnerable to birth defects and harmful DNA mutations.

By the time King Charles II died in 1700 without any children from his two marriages, the male line of the Spanish branch of the family, which produced rulers in Austria, Hungary and the Netherlands, died out.

His failure to produce an heir, despite having married twice, sparked the War of the Spanish Succession between 1701 and 1714.

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Re: Mandibular prognathism
« Reply #5 on: May 11, 2015, 12:56:28 PM »

During that period several European powers, including Great Britain, combined to stop a possible unification of the Kingdoms of Spain and France under a single Bourbon monarch, upsetting the European balance of power.
Monarchs tried to consolidate their power by intermarrying and Charles II was the son of Philip IV by his second marriage with Maria, daughter of the emperor Ferdinand III, his niece.

The study shows that he was the offspring of a marriage almost as genetically inbred as a relationship between a parent and child or brother and sister.

Nicknamed El Hechizado ('The Hexed') because people at the time thought Charles II's disabilities were down to witchcraft, it is believed he suffered from at least two inherited disorders.

One was a hormone deficiency and the other a kidney malfunction which could explain his impotence and infertility which led to the extinction of the dynasty.

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Re: Mandibular prognathism
« Reply #6 on: May 11, 2015, 01:01:42 PM »

He was born on the 11th of November 1661, and was the only surviving son of his father's two marriages - a child of old age and disease, in whom the constant intermarriages of the Habsburgs had developed the family type to deformity.

Study leader Professor Gonzalo Alvarez, of the University of Santiago de Compostela in Galicia, Spain, said: 'He was unable to speak until the age of four, and could not walk until the age of eight.

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Re: Mandibular prognathism
« Reply #7 on: May 11, 2015, 01:03:04 PM »

article-1170143-047659AB000005DC-415_224x423 - Mandibular prognathism - Science and Research article-1170143-047666C8000005DC-431_224x423 - Mandibular prognathism - Science and Research
Charles II's father King Philip IV, left, with the 'Hapsburg jaw' and, right, his mother Maria Anna. King Philip was her uncle

'He was short, weak and quite lean and thin. He first marries at 18 and later at 29, leaving no descendency.

'His first wife talks of his premature ejaculation, while his second spouse complains about his impotency. He looked like an old person when he was only 30 years old, suffering from edemas on his feet, legs, abdomen and face.

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Re: Mandibular prognathism
« Reply #8 on: May 11, 2015, 01:13:04 PM »

'During the last years of his life he barely can stand up, and suffers from hallucinations and convulsive episodes. His health worsens until his premature death when he was 39, after an episode of fever, abdominal pain, hard breathing and coma.'

The cosanguineous marriages also contributed to the development of the 'Hapsburg jaw' which featured in paintings by Titian and Velazaquez. This disfiguring condition is where the lower jaw grows faster than upper jaw.
As well as having this trait, Charles II's tongue was so big he had difficulty speaking and drooled.

article-1170143-04777E72000005DC-22_468x325 - Mandibular prognathism - Science and Research

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Re: Mandibular prognathism
« Reply #9 on: May 11, 2015, 01:14:26 PM »

The research, published in the journal PLoS ONE, examined the genealogical information of more than 3,000 members of the Spanish Habsburg family over 16 generations.

They found nine of the 11 marriages within the Habsburg dynasty over 200 years were between biological relatives including two uncle-niece marriages.

Furthermore, despite their power and wealth the study found there was also a high rate of infant and child mortality in the Hapsburg families.

Only half of the children born in the dynasty during the years examined survived to age one, compared to about 80 per cent in Spanish villages of the time.

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