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Medical Quiz #6 – Answers

by Suzanne Daniluk, RN on April 20th, 2011

Answers to last week’s Royal Diseases quiz:

1.  This British King suffered from a disease that was unknown to physicians and caused him to lose his throne.

a.  Name the unfortunate king.

King George III of Great Britain and Ireland (1738 – 1820).

b.  What was the disease?

“Mad” King George, whose tyrannical acts against the American colonies are enumerated in the Declaration of Independence, had porphyria. His form of the disease can be hereditary or acquired, and is due to ­­­­­­­­­excess porphyrins – one of which is heme, the red pigment in blood. Accumulation of porphyrins can destroy tissues and turn urine to a port-wine color. The disease was not discovered until 1871, too late for the unfortunate King. He not only lost the colonies, he lost his mind. He had episodes of dementia, and became blind and deaf. By 1810, King George could no longer rule, and his eldest son assumed the throne in his father’s place. Despite his incurable illness and imposed confinement to Windsor Castle in strait jackets, King George lived to age 81.

2.  This Queen carried a gene that passed an illness to the royal houses of Great Britain, Europe, and Russia.

a.  Who is the monarch?

Queen Victoria of the United Kingdom of Great Britain and Ireland, (1819 -1901), granddaughter of King George III.

b.  What is the hereditary illness?

Queen Victoria carried the gene for hemophilia B, also known as Christmas disease, a genetic disorder of blood clotting due to Factor IX deficiency that manifests only in males. Queen Victoria passed the gene to 3 of her 9 children. One son, Leopold, was a hemophiliac. Two of her daughters were carriers who in turn passed the gene to royalty in Europe and Russia; most famously to the family of Czar Nicholas II and Empress Alexandra, a granddaughter of Queen Victoria. Their only son Alexis had hemophilia B, but did not die from the disease. Forensic evidence has shown that Alexis, along with his entire family, was assassinated in 1918. Today, the hemophilia gene is believed to no longer exist among European royalty.

3.  This Prince became a King who had to postpone his own coronation, due to a surgical condition common today, but with a high mortality rate at the time.

a.  Who is the Prince that became King?

Albert Edward, Prince of Wales (1841-1910), eldest son of Queen Victoria, great-grandfather of Queen Elizabeth, became King Edward VII upon his mother’s death.

b.  What was the condition?

Edward, age 61, complained of acute abdominal pain about two weeks before his coronation date set for June 26, 1902. He had appendicitis, rarely treated with surgery in the early 20th century due to the high risk. The king at first refused the procedure, but eventually consented upon insistence by his surgeon, Sir Frederick Treves of Elephant Man fame (portrayed by Anthony Hopkins in the film). The successful operation took place at Buckingham Palace, and the patient was crowned King on August 9, 1902. His dauntless doctor was knighted.

 

References:

  1. Lane, Nick. December 16, 2002. Born to the Purple: The Story of Porphyria. http://www.scientificamerican.com/article.cfm?id=born-to-the-purple-the-st.
  2. Lowenfels, Albert B., MD. Historical Perspectives in Surgery; Famous Patients, Famous Operations, 2002 – The Case of a Royal Pain in the Abdomen. http://medscape.com/viewarticle/430259_print.
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