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Porphyria

1. Georges III of England

1.1 Introduction

For many, George III was simply mad and there is nothing else to say not to write about it. For other, he suffered from late stage of syphilis. The truth is far from there. His illness has been recently diagnosed as porphyry.

Contrary to general believe, Georges was not affected by his illness all his life. He was born in 1738 and became king of England and Ireland in 1760. At the time of his nervous break down in 1765, Great Britain has entered a turbulent period: wars against France, independence of USA, etc. His mental state was so affected in 1788-9 that, at this point, political discussion took place for the Regency. Georges III recovered the following year. However, contrary to what might be expected, he was loved by his contemporary people. He was also keen on knowledge. He had a large library that he donated to the country.

His illness resulted to various side effects. The most notorious one was hallucination: primarily sexual one. In one occasion, he attacked a pregnant Queen-lady-in-waiting. He also thought that he was poisonednote 1. After his relapsed in 1810, He was declared permanently insane and he did not recognise his own family. He died in 1820 without knowing or realising that his wife has died note 1. Another consequence of his illness was his urine colour that was described as pink.

His life is well documented. See note 1 for further readings as it is not the purpose of this page.

From his doctor's diary note 2, it is widely accepted that he inherited porphyria or porphyry. Usually, this type of illness is much minor than on George's case.

1.2 Porphyria definitions

From the internet, there are multiple references. I took one from the medicine libray. Porphyria is a group of at least eight diseases caused by abnormalities in the chemical steps that lead to heme production. Found mostly in the blood, bone marrow, and liver, heme is a vital molecule for all of the body's organs. Heme is a component of hemoglobin, the molecule that carries oxygen in the blood.
Genetic changes are related to the following types of porphyria. »acute intermittent porphyria
»ALAD deficiency porphyria
»congenital erythropoietic porphyria
»erythropoietic protoporphyria
»hepatoerythropoietic porphyria
»hereditary coproporphyria
»porphyria cutanea tarda
»variegate porphyria
The signs and symptoms of porphyria vary among types. Some types of porphyria (called cutaneous porphyrias) cause the skin to become overly sensitive to sunlight. Areas that are exposed to the sun develop redness, blistering, and often scarring. The clinical features of other types of porphyria (called acute porphyrias) affect the nervous system. Appearing quickly and lasting from days to weeks, these signs and symptoms include chest and abdominal pain, emotional and mental disorders, seizures, and muscle weakness. Some porphyrias have a combination of acute symptoms and symptoms that affect the skin.

Environmental factors can strongly influence the occurrence and severity of signs and symptoms in some types of porphyria. Alcohol, smoking, certain drugs, hormones, exposure to sunlight, stress, and dieting or fasting can all trigger the signs and symptoms of the disorder.

There are plenty of references regarding this illness see notes 3 and 4 for futher informations.

1.3 Illness' origin

Inheritance patterns depend on the type of porphyria. Some forms of the condition are inherited in an autosomal dominant pattern, which means one copy of the altered gene is sufficient to cause the disorder. Other porphyrias are inherited in an autosomal recessive pattern, which means two copies of the gene must be altered for a person to be affected by the condition.

It is general that Georges suffered from the acute form. However, the form is suffered was more acute than it has been reported regarding for example alucination. His illness origin is believed to come from mariage between first degree cousins.