Porphyria in the context of "The Madness of King George"

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⭐ Core Definition: Porphyria

Porphyria (/pɔːrˈfɪriə/ or /pɔːrˈfriə/) is a group of disorders in which substances called porphyrins build up in the body, adversely affecting the skin or nervous system. The types that affect the nervous system are also known as acute porphyria, as symptoms are rapid in onset and short in duration. Symptoms of an attack include abdominal pain, chest pain, vomiting, confusion, constipation, fever, high blood pressure, and high heart rate. The attacks usually last for days to weeks. Complications may include paralysis, low blood sodium levels, and seizures. Attacks may be triggered by alcohol, smoking, hormonal changes, fasting, stress, or certain medications. If the skin is affected, blisters or itching may occur with sunlight exposure.

Most types of porphyria are inherited from one or both of a person's parents and are due to a mutation in one of the genes that make heme. They may be inherited in an autosomal dominant, autosomal recessive, or X-linked dominant manner. One type, porphyria cutanea tarda, may also be due to hemochromatosis (increased iron in the liver), hepatitis C, alcohol, or HIV/AIDS. The underlying mechanism results in a decrease in the amount of heme produced and a build-up of substances involved in making heme. Porphyrias may also be classified by whether the liver or bone marrow is affected. Diagnosis is typically made by blood, urine, and stool tests. Genetic testing may be done to determine the specific mutation. Hepatic porphyrias are those in which the enzyme deficiency occurs in the liver. Hepatic porphyrias include acute intermittent porphyria (AIP), variegate porphyria (VP), aminolevulinic acid dehydratase deficiency porphyria (ALAD), hereditary coproporphyria (HCP), and porphyria cutanea tarda.

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👉 Porphyria in the context of The Madness of King George

The Madness of King George is a 1994 British biographical comedy drama film directed by Nicholas Hytner and adapted by Alan Bennett from his own 1991 play The Madness of George III. It tells the true story of George III of Great Britain's deteriorating mental health, and his equally declining relationship with his eldest son, the Prince of Wales, particularly focusing on the period around the Regency Crisis of 1788–89. Two text panels at the end of the film note that the colour of the King's urine suggests that he was suffering from porphyria, adding that the disease is "periodic, unpredictable and hereditary."

The Madness of King George won the BAFTA Awards in 1995 for Outstanding British Film and Best Actor in a Leading Role for Nigel Hawthorne, who was also nominated for the Academy Award for Best Actor. The film won the Oscar for Best Art Direction and was also nominated for Oscars for Best Supporting Actress for Mirren and Best Adapted Screenplay. Helen Mirren also won the Cannes Film Festival Award for Best Actress and Hytner was nominated for the Palme d'Or.

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Porphyria in the context of Porphyria cutanea tarda

Porphyria cutanea tarda (PCT) is a form of long-term porphyria characterised by fragile skin and sore blisters in areas of skin that receive higher levels of exposure to sunlight, such as the face and backs of the hands. These blisters burst easily resulting in erosions, crusts, and superficial ulcers. There is often associated darkened skin color and extra facial hair growth. Healing is typically slow, leading to scarring and milia, while changes such as hair loss, and alterations in nails may also occur. A slightly purplish tint may be seen around the eyes. Scleroderma-like thick skin may develop over fingers, scalp, behind the ears, at the back of the neck, or in the front of the chest. The urine may appear dark. Unlike other porphyrias, PCT does not cause severe illness.

The disorder results from a deficiency of uroporphyrinogen III decarboxylase, used in the production of heme, a vital component of hemoglobin. It is generally divided into three types; familial, non-familial, and acquired. The condition can be related to liver disease and a history of excessive alcohol consumption. It is a recognised complication of hepatitis C. Other associated conditions include type 2 diabetes, metabolic syndrome, HIV, SLE, and haemochromatosis. Smoking, and the use of estrogen in males with prostate cancer and females on combined contraceptive pill may also trigger PCT. Renal dialysis may cause retention of porphyrins and cause PCT.

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Porphyria in the context of Halothane

Halothane (Bromochlorotrifluoroethane), sold under the brand name Fluothane among others, is a halocarbon with the chemical formula CF3CHBrCl. It is used as a general anaesthetic given by inhalation. It can be used to induce or maintain anaesthesia. Its use in developed countries has been mostly replaced by newer anesthetic agents such as sevoflurane. One of its benefits is that it does not increase the production of saliva, which can be particularly useful in those who are difficult to intubate.

Side effects include an irregular heartbeat, respiratory depression, and hepatotoxicity. Like all volatile anesthetics, it should not be used in people with a personal or family history of malignant hyperthermia. It appears to be safe in porphyria. It is unclear whether its usage during pregnancy is harmful to the fetus, and its use during a C-section is generally discouraged. Halothane is a chiral molecule that is used as a racemic mixture.

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