Porphyria - Knowledge (XXG)

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the folkloric vampire, when unearthed, was always described as looking quite healthy ("as they were in life"), while due to disfiguring aspects of the disease, sufferers would not have passed the exhumation test. Individuals with congenital erythropoietic porphyria do not crave blood. The enzyme (hematin) necessary to alleviate symptoms is not absorbed intact on oral ingestion, and drinking blood would have no beneficial effect on the sufferer. Finally, and most important, the fact that vampire reports were literally rampant in the 18th century, and that congenital erythropoietic porphyria is an extremely rare manifestation of a rare disease, makes it an unlikely explanation of the folkloric vampire.

620:(vesicles) and open sores (erosions) noted in patients—is the pattern seen in CEP, PCT, VP, and HCP. The changes are noted only in sun-exposed areas such as the face and back of the hands. Milder skin disease, such as that seen in VP and HCP, consists of increased skin fragility in exposed areas with a tendency to form blisters and erosions, particularly after minor knocks or scrapes. These heal slowly, often leaving small scars that may be lighter or darker than normal skin. More severe skin disease is sometimes seen in PCT, with prominent lesions, darkening of exposed skin such as the face, and 2318: 1701:(trade name NormoSang) are the drugs of choice in acute porphyria, in the United States and the United Kingdom, respectively. These drugs need to be given very early in an attack to be effective; effectiveness varies amongst individuals. They are not curative drugs but can shorten attacks and reduce the intensity of an attack. Side effects are rare but can be serious. These heme-like substances theoretically inhibit ALA synthase and hence the accumulation of toxic precursors. In the United Kingdom, supplies of NormoSang are kept at two national centers; emergency supply is available from 52: 446: 1344: 4019: 3975: 1582:(PBG) is the first step if acute porphyria is suspected. As a result of feedback, the decreased production of heme leads to increased production of precursors, PBG being one of the first substances in the porphyrin synthesis pathway. In nearly all cases of acute porphyria syndromes, urinary PBG is markedly elevated except for the very rare 1605:, general hospital labs typically do not have the expertise, technology, or staff time to perform porphyria testing. In general, testing involves sending samples of blood, stool, and urine to a reference laboratory. All samples to detect porphyrins must be handled properly. Samples should be taken during an acute attack; otherwise a 499: 2337: 2183:. The closing credits of the film include the comment that the King's symptoms suggest that he had porphyria, and note that the disease is "periodic, unpredictable, and hereditary". The traditional argument that George III did not have porphyria, but rather bipolar disorder, is thoroughly defended by 646:

Diagnosis of congenital porphyrias involves clinical evaluation, biochemical testing, and genetic analysis. Treatment aims to manage symptoms and prevent acute attacks by avoiding triggers, such as sunlight exposure, certain medications, and alcohol. Additionally, treatments may include phlebotomy to

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may have caused his porphyria. This study found high levels of arsenic in King George's hair. In 2010, one analysis of historical records argued that the porphyria claim was based on spurious and selective interpretation of contemporary medical and historical sources. The mental illness of George

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These prevalence estimates may vary across different regions and populations, and the actual prevalence of congenital porphyrias may be underreported due to challenges in diagnosis and awareness. Additionally, advances in genetic testing and increased awareness of porphyria may lead to more accurate

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that transform the various porphyrins into others, leading to abnormally high levels of one or more of these substances. Porphyrias are classified in two ways, by symptoms and by pathophysiology. Physiologically, porphyrias are classified as liver or erythropoietic based on the sites of accumulation

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Congenital erythropoietic porphyria (CEP), also known as Günther's disease, results from a deficiency in uroporphyrinogen III synthase. This leads to the accumulation of uroporphyrin I and coproporphyrin I in the bone marrow, blood, and urine. Symptoms of CEP include severe photosensitivity, anemia,

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in adult patients with EPP. In a 2023 industry-funded phase 2 trial, dersimelagon, an orally administered, selective melanocortin 1 receptor agonist that increases levels of skin eumelanin, was reported to have increased the duration of symptom-free sunlight exposure and quality of life compared to

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Skin disease is encountered where excess porphyrins accumulate in the skin. Porphyrins are photoactive molecules, and exposure to light results in promotion of electrons to higher energy levels. When these return to the resting energy level or ground state, energy is released. This accounts for the

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team, were written as though the case for porphyria had been proven, but the response demonstrated that many experts, including those more intimately familiar with the manifestations of porphyria, were unconvinced. Many psychiatrists disagreed with the diagnosis, suggesting bipolar disorder as far

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as a more likely diagnosis. The first suggestion that a physical illness was the cause of King George's mental derangement came in 1966, in a paper called "The Insanity of King George III: A Classic Case of Porphyria", with a follow-up in 1968, "Porphyria in the Royal Houses of Stuart, Hanover and

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As it was believed that the folkloric vampire could move about freely in daylight hours, as opposed to the 20th century variant, congenital erythropoietic porphyria cannot readily explain the folkloric vampire but may be an explanation of the vampire as we know it in the 20th century. In addition,

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Congenital porphyrias are genetic disorders caused by mutations in enzymes involved in the heme biosynthesis pathway. There are several types of congenital porphyrias, including erythropoietic protoporphyria (EPP), congenital erythropoietic porphyria (CEP), and porphyria cutanea tarda (PCT). Each

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Porphyrias have been detected in all races and in multiple ethnic groups on every continent. There are high incidence reports of AIP in areas of India and Scandinavia. More than 200 genetic variants of AIP are known, some of which are specific to families, although some strains have proven to be

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PCT is the most common form of porphyria, with an estimated prevalence of 1 to 2 cases per 10,000 individuals in the general population. It predominantly affects adults, with a higher prevalence in men than in women. PCT can be sporadic or familial and is often associated with underlying liver

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CEP is extremely rare, with fewer than 200 cases reported worldwide. It is inherited in an autosomal recessive manner, meaning both parents must carry a mutated gene for a child to develop the condition. CEP occurs with higher frequency in certain populations, including individuals of Northern

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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

598:(EPP). None of these are associated with acute attacks; their primary manifestation is with skin disease. For this reason, these four porphyrias—along with two acute porphyrias, VP and HCP, that may also involve skin manifestations—are sometimes called cutaneous porphyrias. 638:

Erythropoietic protoporphyria (EPP) is caused by a deficiency in ferrochelatase, leading to the accumulation of protoporphyrin IX in red blood cells, plasma, and tissues. Patients with EPP experience severe photosensitivity, with exposure to sunlight causing painful skin

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or other identification at all times. This is in case they develop severe symptoms, or in case of accidents where there is a potential for drug exposure, and as a result they are unable to explain their condition to healthcare professionals. Some drugs are absolutely

628:(HEP); symptoms include severe shortening of digits, loss of skin appendages such as hair and nails, and severe scarring of the skin with progressive disappearance of ears, lips, and nose. Patients may also show deformed, discolored teeth or gum and eye abnormalities. 407:

The precise prevalence of porphyria is unclear, but it is estimated to affect between 1 and 100 per 50,000 people. Rates are different around the world. Porphyria cutanea tarda is believed to be the most common type. The disease was described as early as 370 BC by

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reduce iron levels in PCT, administration of heme preparations to alleviate symptoms, and liver transplantation in severe cases. Early diagnosis and appropriate management are crucial in improving the quality of life for individuals with congenital porphyrias.

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are safe and, when used in conjunction with newer anti-seizure medications such as gabapentin, offer a possible regimen for seizure control. Gabapentin has the additional feature of aiding in the treatment of some kinds of neuropathic pain.

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The links between porphyrias and mental illness have been noted for decades. In the early 1950s, patients with porphyrias (occasionally referred to as "porphyric hemophilia") and severe symptoms of depression or catatonia were treated with

1865:. Some psychotropic drugs are porphyrinogenic, limiting the therapeutic scope. Other psychiatric symptoms such as anxiety, restlessness, insomnia, depression, mania, hallucinations, delusions, confusion, catatonia, and psychosis may occur. 2238:, the same physician who treated George III, was even summoned by the Portuguese court but returned to England after the court limited the treatments he could oversee. Contemporary sources, such as Secretary of State for Foreign Affairs 1553:, which is characterized by a deficiency in coproporphyrinogen oxidase, coded for by the CPOX gene, may also present with both acute neurologic attacks and cutaneous lesions. All other porphyrias are either skin- or nerve-predominant. 3912:

Murray ED, Buttner N, Price BH. (2012) Depression and Psychosis in Neurological Practice. In: Neurology in Clinical Practice, 6th Edition. Bradley WG, Daroff RB, Fenichel GM, Jankovic J (eds.) Butterworth Heinemann. 12 April 2012.

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The theory has been rejected by a few folklorists and researchers as not accurately describing the characteristics of the original werewolf and vampire legends or the disease, and as potentially stigmatizing people with porphyria.

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Treatment depends on the type of porphyria and the person's symptoms. Treatment of porphyria of the skin generally involves the avoidance of sunlight, while treatment for acute porphyria may involve giving intravenous heme or a

2294: 2234:. Maria I—known as "Maria the Pious" or "Maria the Mad" because of both her religious fervor and her acute mental illness, which made her incapable of handling state affairs after 1792—is also thought to have had porphyria. 573:

Given the many presentations and the relatively low occurrence of porphyria, patients may initially be suspected to have other, unrelated conditions. For instance, the polyneuropathy of acute porphyria may be mistaken for

578:, and porphyria testing is commonly recommended in those situations. Elevation of aminolevulinic acid from lead-induced disruption of heme synthesis results in lead poisoning having symptoms similar to acute porphyria. 1982:

Rates of all types of porphyria taken together have been estimated to be approximately one in 25,000 in the United States. The worldwide prevalence has been estimated to be between one in 500 and one in 50,000 people.

613:—typically about 30 minutes—patients complain of severe pain, burning, and discomfort in exposed areas. Typically, the effects are not visible, though occasionally there may be some redness and swelling of the skin. 549:

The most severe episodes may involve neurological complications: typically motor neuropathy (severe dysfunction of the peripheral nerves that innervate muscle), which leads to muscle weakness and potentially to

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in children have been associated with porphyrias. This is thought to be due to axonal nerve deterioration in affected areas of the nervous system and vagal nerve dysfunction. Pain treatment with long-acting

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Di Pierro, Elena; De Canio, Michele; Mercadante, Rosa; Savino, Maria; Granata, Francesca; Tavazzi, Dario; Nicolli, Anna Maria; Trevisan, Andrea; Marchini, Stefano; Fustinoni, Silvia (26 July 2021).

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Often, empirical treatment is required if the diagnostic suspicion of a porphyria is high since acute attacks can be fatal. A high-carbohydrate diet is typically recommended; in severe attacks, a

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Balwani, Manisha; Bonkovsky, Herbert L.; Levy, Cynthia; Anderson, Karl E.; Bissell, D. Montgomery; Parker, Charles; Takahashi, Fumihiro; Desnick, Robert J.; Belongie, Kirstine (13 April 2023).

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EPP is relatively rare, with an estimated prevalence of 1 to 9 cases per 100,000 individuals worldwide. It affects both males and females, typically presenting in childhood or early adulthood.

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Clinical Evaluation: A thorough medical history and physical examination focusing on symptoms related to photosensitivity, skin lesions, abdominal pain, and neurological manifestations.

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Acute porphyria can be triggered by a number of drugs, most of which are believed to trigger it by interacting with enzymes in the liver which are made with heme. Such drugs include:

3960: 1723:, as these are signs of impending syndrome of inappropriate antidiuretic hormone (SIADH) or peripheral nervous system involvement that may be localized or severe, progressing to 4336: 4968: 519: 4916: 2224:'s ancestor, also had acute intermittent porphyria, although this is subject to much debate. It is assumed she inherited the disorder, if indeed she had it, from her father, 2093: 3413:

Lourenço, Charles Marquez; Lee, Chul; Anderson, Karl E. (2012). "Disorders of Haem Biosynthesis". In Saudubray, Jean-Marie; van den Berghe, Georges; Walter, John H. (eds.).

3713: 1598:-induced porphyria, other changes in porphyrin profiles appear, most notably elevations of uroporphyrins I & III, coproporphyrins I & III, and pre-coproporphyrin. 956:

In the autosomal recessive types, if a person inherits a single gene they may become a carrier. Generally they do not have symptoms, but may pass the gene onto offspring.

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Anderson KE, Bloomer JR, Bonkovsky HL, Kushner JP, Pierach CA, Pimstone NR, Desnick RJ (2005). "Recommendations for the diagnosis and treatment of the acute porphyrias".

1950:. The signs may present from birth and include severe photosensitivity, brown teeth that fluoresce in ultraviolet light due to deposition of Type 1 porphyrins, and later 1464:

precursors, which are toxic to tissue in high concentrations. The chemical properties of these intermediates determine the location of accumulation, whether they induce

2393:. Some of the narrator's symptoms resemble those of porphyria, and one passage of the story states that the name of the narrator's disease "had some Ps and Rs in it." 2160:

which documents the ultimately unsuccessful search for genetic evidence of porphyria in the remains of royals suspected to have had it. In 2005, it was suggested that

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Singal, AK; Anderson, KE; Pagon, RA; Adam, MP; Ardinger, HH; Wallace, SE; Amemiya, A; Bean, LJH; Bird, TD; Dolan, CR; Fong, CT; Smith, RJH; Stephens, K (2013).

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may be used in its treatment. A bone marrow transplant has also been successful in curing CEP in a few cases, although long-term results are not yet available.

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to shut down menstrual cycles. However, oral contraceptives have also triggered photosensitivity and withdrawal of oral contraceptives has triggered attacks.

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Other Tests: Liver function tests, iron studies, and imaging studies such as ultrasound or MRI may be conducted to evaluate liver and spleen involvement.

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Indeed, lead poisoning, like all porphyrin diseases, is accompanied by obstinate constipation, nervous lesions, hyperpigmentation and abdominal attacks.

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Dracula, "The Impaler." Vlad III was also said to have had acute porphyria, which may have started the notion that vampires were allergic to sunlight.

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Left figure is urine on the first day while the right figure is urine after three days of sun exposure showing the classic change in color to purple.

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The pain, burning, swelling, and itching that occur in erythropoietic porphyrias (EP) generally require avoidance of bright sunlight. Most kinds of

2096:, "Porphyria, Vampires, and Werewolves: The Aetiology of European Metamorphosis Legends," gained widespread media coverage, popularizing the idea. 4909: 4547:

Cox TM, Jack N, Lofthouse S, Watling J, Haines J, Warren MJ (2005). "King George III and porphyria: an elemental hypothesis and investigation".

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drugs but is sometimes intractable. Hot baths and showers may lessen nausea temporarily, though caution should be used to avoid burns or falls.

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Stated or implied references to porphyria are included in some literature, particularly gothic literature. These include the following:

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to have had porphyria (based on analysis of their extensive and detailed medical correspondence) were his great-great-granddaughter

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Wang, B.; Bissell, D. M.; Adam, M. P.; Ardinger, H. H.; Pagon, R. A.; Wallace, S. E.; Bean LJH; Stephens, K.; Amemiya, A. (2018).

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synthesis, which in turn reduces the rate of porphyrin accumulation. However, this can worsen cases of low blood sodium levels (

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Heme arginate (NormoSang) is used during crises but also in preventive treatment to avoid crises, one treatment every 10 days.

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Woods, J.S. (1995). "Porphyrin metabolism as indicator of metal exposure and toxicity". In Goyer, R.A.; Cherian, M.G. (eds.).

575: 5002: 2228:. Both father and daughter endured well-documented attacks that could fall within the constellation of symptoms of porphyria. 855: 826: 625: 467: 3780: 4753: 3176: 1861:

often accompanies the disease and is best dealt with by treating the offending symptoms and if needed the judicious use of

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The epidemiology of congenital porphyrias varies depending on the specific type of porphyria. Here's a general overview:

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produced and a build-up of substances involved in making heme. Porphyrias may also be classified by whether the liver or

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Peters TJ, Wilkinson D (2010). "King George III and porphyria: a clinical re-examination of the historical evidence".

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Genetic Testing: Molecular genetic testing to identify specific gene mutations associated with congenital porphyrias.

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The condition is heavily implied to be the cause of the symptoms suffered by the narrator in the gothic short story "

4636: 1900:(primary liver cancer) and may require monitoring. Other typical risk factors for liver cancer need not be present. 1738:

has also been reported to be effective for acute porphyric crisis and possibly effective for long-term prophylaxis.

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Hormonal fluctuations that contribute to cyclical attacks in women have been treated with oral contraceptives and

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Tsao YC, Niu DM, Chen JT, Lin CY, Lin YY, Liao KK (2010). "Gabapentin reduces neurovisceral pain of porphyria".

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at The Porphyrias Consortium (a part of NIH Rare Diseases Clinical Research Network (RDCRN)) Retrieved June 2011

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often accompany this disease. Most seizure medications exacerbate this condition. Treatment can be problematic:

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Authors: Vikramjit S Kanwar, Thomas G DeLoughery, Richard E Frye and Darius J Adams. Updated: 27 July 2010.

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type is characterized by specific enzyme deficiencies leading to the accumulation of different porphyrins.

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It is recommended that patients with a history of acute porphyria, and even genetic carriers, wear an

2375: 2323: 2242:, noted that the queen had ever-worsening stomach pains and abdominal spasms: hallmarks of porphyria. 2217: 1947: 1873:

Some liver diseases may cause porphyria even in the absence of genetic predisposition. These include

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Faraci M, Morreale G, Boeri E, Lanino E, Dallorso S, Dini G, Scuderi F, Cohen A, Cappelli B (2008).

2254:. Other commentators have suggested that Vincent van Gogh may have had acute intermittent porphyria. 5256: 5167: 5032: 2343: 2231: 2225: 1966: 1909: 1893: 1858: 1529: 920: 876: 863: 810: 769: 710: 515: 354: 51: 4312:

Denver, Joness. "An Encyclopaedia of Obscure Medicine". Published by University Books, Inc., 1959.

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Martin A Crook.2006. Clinical chemistry and Metabolic Medicine. seventh edition. Hodder Arnold.

3355: 3349: 3422: 3022: 2757: 2646: 2521: 530:, resulting in episodic crises known as acute attacks. The major symptom of an acute attack is 5207: 4939: 4852: 4825: 4798: 4729: 4657: 4609: 4566: 4514: 4487: 4438: 4387: 4343: 4295: 4160: 4152: 4075: 4036: 3914: 3895: 3844: 3814: 3762: 3692: 3631: 3613: 3557: 3522: 3455: 3426: 3386: 3359: 3207: 3168: 3139: 3082: 2981: 2896: 2869: 2818: 2763: 2710: 2652: 2616: 2581: 2527: 2257: 1940: 1836: 1746:

Pain is severe, frequently out of proportion to physical signs, and often requires the use of

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Most types of porphyria are inherited from one or both of a person's parents and are due to a

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Descendants of George III. Among other descendants of George III theorized by the authors of

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result may occur. Samples must be protected from light and either refrigerated or preserved.

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Immediate photosensitivity. This is typical of XLDPP and EPP. Following a variable period of

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Medscape - Diseases of Tetrapyrrole Metabolism - Refsum Disease and the Hepatic Porphyrias

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are not effective, but SPF-rated long-sleeve shirts, hats, bandanas, and gloves can help.

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The Drug Database for Acute Porphyria - comprehensive database on drug porphyrinogenicity

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to reduce it to tolerable levels. Pain should be treated as early as medically possible.

1046:. (Topical use of these agents is thought to be safe due to minimal systemic absorption.) 2432: 5111: 4793: 4768: 4482: 4457: 4433: 4406: 4382: 4357: 3890: 3863: 3626: 3591: 3077: 3052: 2864: 2837: 2813: 2788: 2386: 2354: 2278: 1951: 1862: 1831: 1606: 1524: 1516: 1512: 1327: 1279: 1103: 1070: 911: 621: 531: 527: 421: 323: 283: 275: 239: 170: 5172: 4562: 330:, hormonal changes, fasting, stress, or certain medications. If the skin is affected, 282:, as symptoms are rapid in onset and short in duration. Symptoms of an attack include 5245: 4846: 4821: 4172: 4070: 4053: 4023: 3979: 3415: 2804: 2283: 2089: 1970: 1962: 1955: 1848: 1755: 1719:) or weakness should be treated with the addition of hematin, heme arginate, or even 1698: 1683: 1637: 1542: 1352: 1138: 1074: 1016: 990: 976: 868:

Jaundice, anemia, enlarged liver and spleen, often neonatal. Photosensitivity later.

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Stokvis BJ (December 1989). "Over twee zeldzame kleurstoffen in urine van zieken".

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Akshatha LN, Rukmini MS, Mamatha TS, Sadashiva Rao P, Prashanth B (December 2014).

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argued for a connection between porphyria and the vampire belief in her 1973 book,

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These are associated with accumulation of porphyrins in erythrocytes and are rare.

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property of fluorescence typical of the porphyrins. This causes local skin damage.

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Birgisdottir BT, Asgeirsson H, Arnardottir S, Jonsson JJ, Vidarsson B (2010). "".

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Severe photosensitivity with erythema, swelling and blistering. Hemolytic anemia,

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The non-acute porphyrias are X-linked dominant protoporphyria (XLDPP), congenital

412:. The underlying mechanism was first described by German physiologist and chemist 5183: 4054:"Unrelated HSCT in an adolescent affected by congenital erythropoietic porphyria" 3032:

Author: Norman C Reynolds. Chief Editor: Stephen A Berman. Updated: 23 March 2009

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legends, based upon certain perceived similarities between the condition and the

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biosynthesis. The principal problem in these deficiencies is the accumulation of

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Mustajoki P (1980). "Variegate porphyria. Twelve years' experience in Finland".

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was approved in the United States for the treatment of acute hepatic porphyria.

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and bromides have also been used in porphyria seizures; however, development of

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biosynthetic pathway, four of which—the first one and the last three—are in the

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The rarest is congenital erythropoietic porphyria (CEP), otherwise known as

1932: 1917: 1816:, is often indicated, and, in cases where seizure or neuropathy is present, 1616:. A careful medication review often will find the cause of pseudoporphyria. 1508: 1504: 1457: 1376: 1364: 1348: 1343: 1297: 1293: 1273: 1255: 1251: 1235: 1231: 1227: 1223: 1203: 1199: 1169: 1154: 1146: 1118: 1110: 1066: 1020: 1002: 311: 271: 157: 31: 4613: 4570: 4164: 4079: 3899: 3848: 3818: 3766: 3635: 3526: 3459: 3172: 3086: 2900: 2873: 2854: 2822: 2714: 2648:

Pathobiology of Human Disease: A Dynamic Encyclopedia of Disease Mechanisms

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is affected. Diagnosis is typically made by blood, urine, and stool tests.

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present with skin problems, usually a light-sensitive blistering rash and

5224: 5218: 4473: 4411: 3226:"OMIM Entry - # 300752 - PROTOPORPHYRIA, ERYTHROPOIETIC, X-LINKED; XLEPP" 2980:. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. 2705: 2261: 2165: 2063: 2059: 1913: 1823: 1813: 1706: 1675: 1380: 1187: 942: 535: 376: 338: 291: 214: 178: 27: 2030:

The underlying mechanism was first described by the German physiologist

310:. The attacks usually last for days to weeks. Complications may include 2161: 2055: 2018:

disease, alcohol abuse, hepatitis C infection, or certain medications.

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Photosensitivity with skin lesions. Gallstones, mild liver dysfunction

617: 559: 470: in this section. Unsourced material may be challenged and removed. 397: 331: 327: 319: 194: 190: 5136: 2759:

Clinical Hepatology: Principles and Practice of Hepatobiliary Diseases

2696: 2576: 2559: 2523:

Clinical Hepatology: Principles and Practice of Hepatobiliary Diseases

616:

Vesiculo-erosive skin disease. This—a reference to the characteristic

5148: 4879: 2912:

the symptoms in lead poisoning closely mimic those of acute porphyria

2374:

The condition is the name of the title character in the gothic poem "

1809: 1751: 1624:

Further diagnostic tests of affected organs may be required, such as

1480: 1399: 674: 429: 2972:

Marks, Dawn B.; Swanson, Todd; Sandra I Kim; Marc Glucksman (2007).

2034:

in 1871, and acute porphyrias were described by the Dutch physician

1678:

10% infusion is commenced, which may aid in recovery by suppressing

4274:

Stokvis BJ. "Over twee zeldzame kleurstoffen in urine van zieken".

4884: 2140: 1796: 1575: 1571: 1567: 1495:. Defects in any of these can lead to some form of porphyria. The 1473: 1469: 1408: 1360: 1342: 1080: 946: 840: 497: 379:. The underlying mechanism results in a decrease in the amount of 299: 186: 4818:

One Man's Medicine: An Autobiography of Professor Archie Cochrane

4637:"The Last King of America: The Misunderstood Reign of George III" 4022:

This article incorporates text from this source, which is in the

3978:

This article incorporates text from this source, which is in the

3326: 2054:

Porphyria has been suggested as an explanation for the origin of

1843:

in porphyria may not respond to magnesium alone. The addition of

1686:) and should be done with extreme caution as it can prove fatal. 19:

This article is about the medical condition. For other uses, see

4925: 3225: 3100: 2500:

National Institute of Diabetes and Digestive and Kidney Diseases

1679: 1636:

of the liver. Basic biochemical tests may assist in identifying

1484: 1461: 1453: 1439: 1431: 1427: 1404: 1395: 1368: 563: 498: 380: 346: 342: 4898: 4407:"The 'insanity' of King George 3d: a classic case of porphyria" 2607:

Kothadia, Jiten P.; LaFreniere, Kilian; Shah, Jamil M. (2024),

87: 4507:

Purple Secret: Genes, "Madness" and the Royal Houses of Europe

2164:(which is known to be porphyrogenic) given to George III with 2069:

In January 1964, L. Illis's 1963 paper, "On Porphyria and the

439: 3579:. Vol. 115. Berlin: Springer. pp. 19–52, Chapter 2. 1916:

and fertility hormones have also triggered attacks. In 2019,

1783:

Patients who experience frequent attacks can develop chronic

1612:

If all the porphyrin studies are negative, one must consider

1426:

of the porphyrin pathway leads to insufficient production of

605:

Two distinct patterns of skin disease are seen in porphyria:

278:. The types that affect the nervous system are also known as 664:

Subtypes of porphyrias depend on which enzyme is deficient.

110: 78: 16:

Metabolic disorders in which porphyrins build up in the body

4769:"Vincent van Gogh's illness: acute intermittent porphyria?" 895:

Photosensitivity, neurologic symptoms, developmental delay

656:

The porphyrias are generally considered genetic in nature.

128: 125: 119: 93: 90: 4750:"BBC News - UK - Charles visits 'relative' Dracula's home" 4334:"Did vampires suffer from the disease porphyria — or not?" 3446:

Ogun, Aminat S.; Joy, Neena V.; Valentine, Menogh (2022),

1954:. Hemolytic anemia usually develops. Pharmaceutical-grade 4505:

Warren, Martin; Rh̲l, John C. G.; Hunt, David C. (1998).

3949:"FDA approves first treatment for inherited rare disease" 3354:(65 ed.). London, UK: Pharmaceutical Press. p. 1943:

is occasionally used to suppress innate heme production.

1939:

may be used to increase porphyrin secretion in some EPs.

1545:, manifests itself with skin lesions similar to those of 4533:

The authors demonstrated a single point mutation in the

4102:"Community register of medicinal products for human use" 1974:

placebo in patients with erythropoietic protoporphyria.

3101:"OMIM Entry - 176090 - PORPHYRIA CUTANEA TARDA, TYPE I" 2762:. Springer Science & Business Media. p. 1088. 2526:. Springer Science & Business Media. p. 1088. 1566:

Porphyria is diagnosed through biochemical analysis of

1438:. This is not the main problem in the porphyrias; most 3134:

Arceci, Robert.; Hann, Ian M.; Smith, Owen P. (2006).

1347:

Heme synthesis. Note that some reactions occur in the

643:

splenomegaly, and often disfiguring cutaneous lesions.

274:

build up in the body, adversely affecting the skin or

4358:"Porphyria and vampirism: another myth in the making" 116: 84: 5126: 3993:"FDA approves givosiran for acute hepatic porphyria" 3830: 3828: 2742:. Hilger & Watts, Hilger Division. p. 126. 2739:

Porphyrins: Their Biological and Chemical Importance

2558:

Stein, PE; Badminton, MN; Rees, DC (February 2017).

2131:. The mental illness exhibited by George III in the 520:

aminolevulinic acid dehydratase deficiency porphyria

122: 107: 75: 5272:

Skin conditions resulting from errors in metabolism

5193: 5130: 5095: 5065: 5050: 5011: 4994:

Gunther disease/congenital erythropoietic porphyria

4982: 4957: 4933: 4456:Macalpine I, Hunter R, Rimington C (January 1968). 4324:, Norine Dresser, W. W. Norton & Company, 1989. 3781:"Porphyria - Diagnosis and treatment - Mayo Clinic" 2094:

American Association for the Advancement of Science

1787:pain in extremities as well as chronic pain in the 1709:manufactures and supplies Panhematin for infusion. 1468:, and whether the intermediate is excreted (in the 871:Extremely rare; fewer than 10 cases ever reported. 718:Extremely rare; fewer than 10 cases ever reported. 392:coproporphyria (HCP), and porphyria cutanea tarda. 270:is a group of disorders in which substances called 257: 249: 233: 220: 209: 201: 163: 143: 113: 81: 63: 44: 4537:but not one that has been associated with disease. 3417:Inborn Metabolic Diseases: Diagnosis and Treatment 3244:"Orphanet: X linked erythropoietic protoporphyria" 2973: 4650:Smith, Martin; Smith, Alison (21 December 2009). 1499:are characterized by acute neurological attacks ( 732:(HMBS) formerly porphobilinogen deaminase (PBGD) 4133:"Dersimelagon in Erythropoietic Protoporphyrias" 2966: 2964: 2962: 2214:was reliably diagnosed with variegate porphyria. 696:Aminolevulinate dehydratase deficiency porphyria 4322:American Vampires: Fans, Victims, Practitioners 3482:. American Porphyria Foundation. Archived from 3454:, Treasure Island (FL): StatPearls Publishing, 3294:. American Porphyria Foundation. Archived from 2960: 2958: 2956: 2954: 2952: 2950: 2948: 2946: 2944: 2942: 2615:, Treasure Island (FL): StatPearls Publishing, 1881:. Treatment of iron overload may be required. 1541:), which results from a partial deficiency in 4910: 4695:Bulletin: The University of Sussex Newsletter 3741:"Cimetidine and acute intermittent porphyria" 3681:Royal Pharmaceutical Society of Great Britain 3421:(5th ed.). New York: Springer. pp. 2427: 334:or itching may occur with sunlight exposure. 8: 4245:"Born to the purple: the story of porphyria" 3325:. European Porphyria Network. Archived from 2425: 2423: 2421: 2419: 2417: 2415: 2413: 2411: 2409: 2407: 2220:. It is believed that Mary, Queen of Scots, 2076:Proceedings of the Royal Society of Medicine 1896:) are at increased risk over their life for 1452:—have enough residual activity to assist in 3129: 3127: 3125: 3123: 3121: 3018: 3016: 2793:Journal of Clinical and Diagnostic Research 2789:"Lead poisoning mimicking acute porphyria!" 2553: 2551: 2549: 2547: 526:(HCP). These diseases primarily affect the 5252:Pages with image sizes containing extra px 5127: 5062: 4954: 4917: 4903: 4895: 4726:The Remarkable Life of Maria I of Portugal 3167:. Seattle, USA: University of Washington. 3046: 3044: 3042: 3040: 3038: 2175:, a 1994 British film based upon the 1991 2007:Congenital Erythropoietic Porphyria (CEP): 1434:function plays a central role in cellular 50: 41: 4792: 4481: 4432: 4381: 4069: 3889: 3879: 3756: 3683:(March 2009). "9.8.2: Acute porphyrias". 3625: 3607: 3577:Toxicology of metals, biochemical aspects 3548:Berkó G, Durkó I (19 December 1971). "". 3076: 2926:"What Tests Can Help Diagnose Porphyria?" 2863: 2853: 2812: 2704: 2640: 2638: 2636: 2575: 1775:for patients with any form of porphyria. 486:Learn how and when to remove this message 205:Recurrent attacks that last days to weeks 2490: 2488: 2486: 2484: 2482: 2480: 2478: 2476: 2156:more probable. The theory is treated in 1549:combined with acute neurologic attacks. 1394:The body requires porphyrins to produce 666: 4816:Ranpura, Dhruv; Dhyey, Khavdu (2009) . 3931:"Hormonal Contraceptives and Porphyria" 3758:10.7326/0003-4819-143-9-200511010-00023 3739:Cherem JH, Malagon J, Nellen H (2005). 3519:10.7326/0003-4819-142-6-200503150-00010 3053:"Diagnosis and management of porphyria" 2560:"Update review of the acute porphyrias" 2474: 2472: 2470: 2468: 2466: 2464: 2462: 2460: 2458: 2456: 2403: 2290: 2277:. The daughter of the Chilean novelist 2264:suggests to some that he had porphyria. 839:Photosensitivity, neurologic symptoms, 4405:Macalpine I, Hunter R (January 1966). 4224:Hoppe-Seyler F (1871). "Das Hämatin". 4205:Genetics Home Reference > Porphyria 3712:American Porphyria Foundation (2010). 3051:Thadani H, Deacon A, Peters T (2000). 2776:from the original on 8 September 2017. 2665:from the original on 8 September 2017. 2540:from the original on 8 September 2017. 2151:Prussia". The papers, by a mother/son 502:A skin rash in a person with porphyria 4112:from the original on 24 December 2014 4007:from the original on 21 November 2019 3963:from the original on 21 November 2019 3323:The Drug Database for Acute Porphyria 1969:as a treatment for the prevention of 792:uroporphyrinogen decarboxylase (UROD) 747:, psychiatric disorders, tachycardia 279: 7: 4890:Orphanet's disease page on Porphyria 4756:from the original on 24 August 2013. 4691:"The Royal Family's Toxic Time-Bomb" 4653:Tetrapyrroles: Birth, Life and Death 3179:from the original on 18 January 2017 2836:Tsai MT, Huang SY, Cheng SY (2017). 2143:. M. Guttmacher, in 1941, suggested 2022:epidemiological data in the future. 2000:Erythropoietic Protoporphyria (EPP): 1884:Patients with the acute porphyrias ( 1779:Neurologic and psychiatric disorders 1586:or in patients with symptoms due to 468:adding citations to reliable sources 4635:Roberts, Andrew (9 November 2021). 4186:eMedicine > Porphyria, Cutaneous 4003:(Press release). 20 November 2019. 3959:(Press release). 20 November 2019. 3685:British National Formulary (BNF 57) 3651:"Porphyrias - immediate management" 3592:"Laboratory Diagnosis of Porphyria" 2206:'s eldest sister) and her daughter 756:Congenital erythropoietic porphyria 3348:Joint Formulary Committee (2013). 3288:"About Porphyria: Safety database" 2976:Biochemistry and molecular biology 2842:Case Reports in Emergency Medicine 2260:. The description of this king in 2208:Princess Feodora of Saxe-Meiningen 1578:. In general, urine estimation of 1491:, while the other four are in the 554:(paralysis of all four limbs) and 546:(an abnormally rapid heart rate). 14: 3691:and RPS Publishing. p. 549. 3262:"Porphyria - Symptoms and causes" 3200:The Quarterly Journal of Medicine 2924:Murphy, Brian (3 November 2020). 2135:has inspired several attempts at 2073:of Werewolves," was published in 1830:especially must be avoided. Some 1754:can be severe; it may respond to 937:protoporphyria is a rare form of 883:protoporphyrinogen oxidase (PPOX) 856:coproporphyrinogen oxidase (CPOX) 827:coproporphyrinogen oxidase (CPOX) 4670:from the original on 4 July 2014 4071:10.1111/j.1399-3046.2007.00842.x 4017: 3973: 3714:"Panhematin for Acute Porphyria" 3379:Kumari, Asha (12 October 2017). 3351:British National Formulary (BNF) 3138:. Malden, Mass.: Blackwell Pub. 2336: 2316: 2293: 2169:III is the basis of the plot in 1965:received authorization from the 1705:, London. In the United States, 1306:Miscellaneous others including: 799:Approximately 80% sporadic, 20% 762:uroporphyrinogen synthase (UROS) 444: 103: 71: 4137:New England Journal of Medicine 3480:"Tests for Porphyria diagnosis" 542:(elevated blood pressure), and 455:needs additional citations for 371:(increased iron in the liver), 226:Blood, urine, and stool tests, 5003:Hepatoerythropoietic porphyria 4243:Nick Lane (16 December 2002). 3448:"Biochemistry, Heme Synthesis" 3292:Porphyria Drug Safety Database 2564:British Journal of Haematology 2258:King Nebuchadnezzar of Babylon 2015:Porphyria Cutanea Tarda (PCT): 1715:Any sign of low blood sodium ( 1371:, an essential constituent of 1296:used for skin conditions like 626:hepatoerythropoietic porphyria 349:. They may be inherited in an 322:. Attacks may be triggered by 1: 5052:Hereditary hyperbilirubinemia 5038:Erythropoietic protoporphyria 4767:Loftus LS, Arnold WN (1991). 4563:10.1016/S0140-6736(05)66991-7 3862:Cherian A, Thomas SV (2009). 2347: 2326: 2200:Princess Charlotte of Prussia 1820:is known to improve outcome. 1793:Intestinal pseudo-obstruction 1588:hereditary tyrosinemia type I 981:trimethoprim/sulfamethoxazole 941:caused by a gain-of-function 939:erythropoietic protoporphyria 906:Erythropoietic protoporphyria 702:5-aminolevulinate dehydratase 596:erythropoietic protoporphyria 4974:Acute intermittent porphyria 4639:. Viking – via Amazon. 4362:Postgraduate Medical Journal 3999:Food and Drug Administration 3955:Food and Drug Administration 2805:10.7860/JCDR/2014/10597.5315 2240:Luís Pinto de Sousa Coutinho 2212:Prince William of Gloucester 2106:Postgraduate Medical Journal 1697:(trade name Panhematin) and 1644:, and other organ problems. 1182:Some cancer treatments like 730:Hydroxymethylbilane synthase 724:Acute intermittent porphyria 512:Acute intermittent porphyria 253:Depends on type and symptoms 4851:. New York: HarperCollins. 4689:Röhl, John (25 June 1999). 4226:Tubinger Med-Chem Untersuch 3677:British Medical Association 3609:10.3390/diagnostics11081343 2889:"Hereditary Coproporphyria" 2676:Albers JW, Fink JK (2004). 1727:and respiratory paralysis. 1670:Carbohydrate administration 1367:are the main precursors of 715:Abdominal pain, neuropathy 5290: 4722:The Madness of Queen Maria 3001:Overview of the Porphyrias 2651:. Elsevier. p. 1488. 2520:Dancygier, Henryk (2009). 2172:The Madness of King George 1584:ALA dehydratase deficiency 1407:precursors, either in the 25: 18: 5023:Hereditary coproporphyria 4785:10.1136/bmj.303.6817.1589 4720:Roberts, Jenifer (2009). 4374:10.1136/pgmj.71.841.643-a 3069:10.1136/bmj.320.7250.1647 2609:"Acute Hepatic Porphyria" 2303:in his coronation robes. 2181:The Madness of George III 1551:Hereditary coproporphyria 821:Hereditary coproporphyria 743:Periodic abdominal pain, 524:hereditary coproporphyria 261:1 to 100 in 50,000 people 58: 49: 21:Porphyry (disambiguation) 5267:Red blood cell disorders 4845:Allende, Isabel (1995). 4728:]. Templeton Press. 4606:10.1177/0957154X09102616 4276:Nederl Tijdschr Geneeskd 2189:The Last King of America 2103:A 1995 article from the 1924:Erythropoietic porphyria 1898:hepatocellular carcinoma 1869:Underlying liver disease 1803:, hypoganglionosis, and 1642:hepatocellular carcinoma 1626:nerve conduction studies 1026:Systemic treatment with 949:characterized by severe 898:1 in 300 in South Africa 780:1 in 1,000,000 or less. 588:erythropoietic porphyria 26:Not to be confused with 5087:Lucey–Driscoll syndrome 5082:Crigler–Najjar syndrome 4999:Porphyria cutanea tarda 4339:5 February 2013 at the 4191:5 December 2010 at the 4108:. European Commission. 3881:10.4103/0972-2327.56312 2645:McManus, Linda (2014). 2353:portrait attributed to 2137:retrospective diagnosis 2050:Vampires and werewolves 1601:As most porphyrias are 1547:porphyria cutanea tarda 900:1 in 75,000 in Finland 786:Porphyria cutanea tarda 592:porphyria cutanea tarda 576:Guillain–Barré syndrome 534:, often accompanied by 364:porphyria cutanea tarda 316:low blood sodium levels 244:alcoholic liver disease 5107:Dubin–Johnson syndrome 3868:Ann Indian Acad Neurol 2678:"Porphyric neuropathy" 2133:regency crisis of 1788 2120: 2088:. In 1985, biochemist 1356: 1083:derivatives including 1005:is thought to be safe. 805:Photosensitivity with 631:Congenital Porphyerias 556:central nervous system 503: 235:Differential diagnosis 4594:History of Psychiatry 4425:10.1136/bmj.1.5479.65 4288:Ned Tijdschr Geneeskd 4210:5 August 2010 at the 4149:10.1056/NEJMoa2208754 3161:"Variegate Porphyria" 2187:in his new biography 2115: 1851:has been used during 1525:increased hair growth 1346: 1332:sodium aurothiomalate 745:peripheral neuropathy 501: 367:, may also be due to 169:Depending on subtype— 4960:early mitochondrial: 4928:metabolism disorders 4701:on 15 September 2013 4474:10.1136/bmj.1.5583.7 4356:Cox, Ann M. (1995). 3864:"Status epilepticus" 3385:. Elsevier Science. 3006:22 July 2011 at the 2855:10.1155/2017/9050713 2756:Dancygier H (2009). 2324:Mary, Queen of Scots 2218:Mary, Queen of Scots 1987:repeated mutations. 1910:luteinizing hormones 1762:Early identification 1703:St Thomas's Hospital 1521:erythropoietic forms 1448:—even dysfunctional 928:1 in 75,000–200,000 464:improve this article 404:may be carried out. 400:solution. Rarely, a 5033:Variegate porphyria 5014:late mitochondrial: 4779:(6817): 1589–1591. 4249:Scientific American 3136:Pediatric hematolog 3063:(7250): 1647–1651. 3028:4 June 2011 at the 2736:Vannotti A (1954). 2496:"Porphyria | NIDDK" 2344:Maria I of Portugal 2287:, dedicated to her. 2275:Paula Frías Allende 2232:Maria I of Portugal 2226:James V of Scotland 1967:European Commission 1535:porphyria variegata 1530:Variegate porphyria 921:Autosomal recessive 877:Variegate porphyria 864:Autosomal recessive 770:Autosomal recessive 750:1 in 10,000–20,000 711:Autosomal recessive 516:variegate porphyria 355:autosomal recessive 304:high blood pressure 5194:External resources 5077:Gilbert's syndrome 4255:on 11 October 2007 4214:Reviewed July 2009 4058:Pediatr Transplant 3837:Acta Neurol Taiwan 3785:www.mayoclinic.org 3687:. United Kingdom: 3657:on 20 October 2016 3382:Sweet Biochemistry 2366:Uses in literature 2204:Emperor William II 2032:Felix Hoppe-Seyler 2010:European descent. 1961:In December 2014, 1853:status epilepticus 1841:status epilepticus 1497:hepatic porphyrias 1422:Deficiency in the 1357: 1166:dextropropoxyphene 891:Autosomal dominant 835:Autosomal dominant 801:Autosomal dominant 739:Autosomal dominant 679:Type of porphyria 582:Chronic porphyrias 504: 436:Signs and symptoms 416:in 1871. The name 414:Felix Hoppe-Seyler 361:manner. One type, 351:autosomal dominant 5262:Diseases of liver 5239: 5238: 5124: 5123: 5120: 5119: 5046: 5045: 4858:978-0-06-017253-4 4831:978-0-9540884-3-9 4600:(81 Pt 1): 3–19. 4557:(9482): 332–335. 4520:978-0-593-04148-2 4344:The Straight Dope 4143:(15): 1376–1385. 3937:. September 2020. 3720:on 25 August 2010 3698:978-0-85369-845-6 3432:978-3-642-15719-6 3365:978-0-85711-084-8 3329:on 23 August 2017 3145:978-1-4051-3400-2 2987:978-0-7817-8624-9 2697:10.1002/mus.20137 2577:10.1111/bjh.14459 2376:Porphyria's Lover 2092:'s paper for the 1991:Other Information 1948:Gunther's disease 1941:Blood transfusion 1904:Hormone treatment 1837:Magnesium sulfate 1721:tin mesoporphyrin 1647:•Other Diagnosis 1596:arsenic poisoning 1562:Porphyrin studies 1387:, and P450 liver 1085:dihydroergotamine 935:X-linked dominant 932: 931: 558:symptoms such as 496: 495: 488: 359:X-linked dominant 265: 264: 222:Diagnostic method 39:Medical condition 5279: 5128: 5100: 5070: 5063: 5028:Harderoporphyria 5016: 4987: 4962: 4955: 4919: 4912: 4905: 4896: 4863: 4862: 4842: 4836: 4835: 4813: 4807: 4806: 4796: 4764: 4758: 4757: 4746: 4740: 4739: 4717: 4711: 4710: 4708: 4706: 4697:. Archived from 4686: 4680: 4679: 4677: 4675: 4647: 4641: 4640: 4632: 4626: 4625: 4589: 4583: 4582: 4544: 4538: 4531: 4525: 4524: 4502: 4496: 4495: 4485: 4453: 4447: 4446: 4436: 4402: 4396: 4395: 4385: 4368:(841): 643–644. 4353: 4347: 4331: 4325: 4319: 4313: 4310: 4304: 4303: 4283: 4271: 4265: 4264: 4262: 4260: 4251:. Archived from 4240: 4234: 4233: 4221: 4215: 4202: 4196: 4183: 4177: 4176: 4128: 4122: 4121: 4119: 4117: 4098: 4092: 4091: 4073: 4049: 4043: 4033: 4027: 4021: 4020: 4016: 4014: 4012: 3989: 3983: 3977: 3976: 3972: 3970: 3968: 3945: 3939: 3938: 3927: 3921: 3910: 3904: 3903: 3893: 3883: 3859: 3853: 3852: 3832: 3823: 3822: 3809:(in Icelandic). 3802: 3796: 3795: 3793: 3791: 3777: 3771: 3770: 3760: 3745:Ann. Intern. Med 3736: 3730: 3729: 3727: 3725: 3716:. Archived from 3709: 3703: 3702: 3673: 3667: 3666: 3664: 3662: 3653:. Archived from 3646: 3640: 3639: 3629: 3611: 3587: 3581: 3580: 3572: 3566: 3565: 3552:(in Hungarian). 3545: 3539: 3538: 3507:Ann. Intern. Med 3502: 3496: 3495: 3493: 3491: 3486:on 20 March 2014 3476: 3470: 3469: 3468: 3466: 3443: 3437: 3436: 3420: 3410: 3404: 3403: 3401: 3399: 3376: 3370: 3369: 3345: 3339: 3338: 3336: 3334: 3314: 3308: 3307: 3305: 3303: 3283: 3277: 3276: 3274: 3272: 3258: 3252: 3251: 3240: 3234: 3233: 3222: 3216: 3215: 3206:(194): 191–203. 3195: 3189: 3188: 3186: 3184: 3156: 3150: 3149: 3131: 3116: 3115: 3113: 3111: 3097: 3091: 3090: 3080: 3048: 3033: 3020: 3011: 2998: 2992: 2991: 2979: 2968: 2937: 2936: 2934: 2932: 2921: 2915: 2914: 2909: 2907: 2884: 2878: 2877: 2867: 2857: 2833: 2827: 2826: 2816: 2784: 2778: 2777: 2753: 2747: 2746: 2733: 2727: 2726: 2708: 2682: 2673: 2667: 2666: 2642: 2631: 2630: 2629: 2627: 2604: 2598: 2597: 2579: 2555: 2542: 2541: 2517: 2511: 2510: 2508: 2506: 2492: 2451: 2450: 2448: 2446: 2429: 2352: 2349: 2340: 2331: 2328: 2320: 2297: 2252:Vincent van Gogh 2145:manic-depressive 1620:Additional tests 1479:There are eight 1466:photosensitivity 1351:and some in the 951:photosensitivity 851:Harderoporphyria 667: 507:Acute porphyrias 491: 484: 480: 477: 471: 448: 440: 402:liver transplant 135: 134: 131: 130: 127: 124: 121: 118: 115: 112: 109: 100: 99: 96: 95: 92: 89: 86: 83: 80: 77: 54: 42: 5289: 5288: 5282: 5281: 5280: 5278: 5277: 5276: 5242: 5241: 5240: 5235: 5234: 5189: 5188: 5139: 5125: 5116: 5096: 5091: 5066: 5054: 5042: 5012: 5007: 4983: 4978: 4958: 4946: 4938: 4929: 4923: 4872: 4867: 4866: 4859: 4844: 4843: 4839: 4832: 4815: 4814: 4810: 4766: 4765: 4761: 4748: 4747: 4743: 4736: 4719: 4718: 4714: 4704: 4702: 4688: 4687: 4683: 4673: 4671: 4664: 4649: 4648: 4644: 4634: 4633: 4629: 4591: 4590: 4586: 4546: 4545: 4541: 4532: 4528: 4521: 4504: 4503: 4499: 4455: 4454: 4450: 4419:(5479): 65–71. 4404: 4403: 4399: 4355: 4354: 4350: 4341:Wayback Machine 4332: 4328: 4320: 4316: 4311: 4307: 4294:(51): 2562–70. 4285: 4273: 4272: 4268: 4258: 4256: 4242: 4241: 4237: 4223: 4222: 4218: 4212:Wayback Machine 4203: 4199: 4193:Wayback Machine 4184: 4180: 4130: 4129: 4125: 4115: 4113: 4100: 4099: 4095: 4051: 4050: 4046: 4034: 4030: 4018: 4010: 4008: 3991: 3990: 3986: 3974: 3966: 3964: 3947: 3946: 3942: 3929: 3928: 3924: 3911: 3907: 3861: 3860: 3856: 3834: 3833: 3826: 3804: 3803: 3799: 3789: 3787: 3779: 3778: 3774: 3738: 3737: 3733: 3723: 3721: 3711: 3710: 3706: 3699: 3675: 3674: 3670: 3660: 3658: 3649:patient.co.uk. 3648: 3647: 3643: 3589: 3588: 3584: 3574: 3573: 3569: 3547: 3546: 3542: 3504: 3503: 3499: 3489: 3487: 3478: 3477: 3473: 3464: 3462: 3445: 3444: 3440: 3433: 3412: 3411: 3407: 3397: 3395: 3393: 3378: 3377: 3373: 3366: 3347: 3346: 3342: 3332: 3330: 3316: 3315: 3311: 3301: 3299: 3298:on 12 July 2017 3285: 3284: 3280: 3270: 3268: 3260: 3259: 3255: 3242: 3241: 3237: 3224: 3223: 3219: 3197: 3196: 3192: 3182: 3180: 3158: 3157: 3153: 3146: 3133: 3132: 3119: 3109: 3107: 3099: 3098: 3094: 3050: 3049: 3036: 3030:Wayback Machine 3021: 3014: 3008:Wayback Machine 2999: 2995: 2988: 2971: 2970:Table 18-1 in: 2969: 2940: 2930: 2928: 2923: 2922: 2918: 2905: 2903: 2886: 2885: 2881: 2835: 2834: 2830: 2786: 2785: 2781: 2770: 2755: 2754: 2750: 2735: 2734: 2730: 2680: 2675: 2674: 2670: 2659: 2644: 2643: 2634: 2625: 2623: 2606: 2605: 2601: 2557: 2556: 2545: 2534: 2519: 2518: 2514: 2504: 2502: 2494: 2493: 2454: 2444: 2442: 2431: 2430: 2405: 2400: 2391:Margaret Atwood 2380:Robert Browning 2368: 2361: 2350: 2341: 2332: 2329: 2321: 2312: 2298: 2269:Archie Cochrane 2222:King George III 2125: 2052: 2028: 1993: 1980: 1926: 1906: 1875:hemochromatosis 1871: 1863:antidepressants 1832:benzodiazepines 1801:intussusception 1781: 1773:contraindicated 1764: 1744: 1742:Symptom control 1733: 1692: 1672: 1667: 1665:Acute porphyria 1662: 1622: 1614:pseudoporphyria 1603:rare conditions 1580:porphobilinogen 1564: 1559: 1539:mixed porphyria 1515:, and an acute 1417:red blood cells 1341: 1262:antidepressants 1135:anticonvulsants 1073:and, possibly, 962: 917:Erythropoietic 899: 860:Erythropoietic 766:Erythropoietic 670:Porphyria type 662: 654: 584: 509: 492: 481: 475: 472: 461: 449: 438: 389:Genetic testing 369:hemochromatosis 308:high heart rate 280:acute porphyria 228:genetic testing 139: 106: 102: 74: 70: 40: 35: 24: 17: 12: 11: 5: 5287: 5286: 5283: 5275: 5274: 5269: 5264: 5259: 5254: 5244: 5243: 5237: 5236: 5233: 5232: 5221: 5210: 5198: 5197: 5195: 5191: 5190: 5187: 5186: 5175: 5160: 5140: 5135: 5134: 5132: 5131:Classification 5122: 5121: 5118: 5117: 5115: 5114: 5112:Rotor syndrome 5109: 5103: 5101: 5093: 5092: 5090: 5089: 5084: 5079: 5073: 5071: 5060: 5048: 5047: 5044: 5043: 5041: 5040: 5035: 5030: 5025: 5019: 5017: 5009: 5008: 5006: 5005: 4996: 4990: 4988: 4980: 4979: 4977: 4976: 4971: 4969:ALAD porphyria 4965: 4963: 4952: 4944:erythropoietic 4931: 4930: 4924: 4922: 4921: 4914: 4907: 4899: 4893: 4892: 4887: 4882: 4871: 4870:External links 4868: 4865: 4864: 4857: 4837: 4830: 4808: 4759: 4741: 4735:978-0954558918 4734: 4712: 4681: 4662: 4642: 4627: 4584: 4539: 4526: 4519: 4497: 4468:(5583): 7–18. 4448: 4397: 4348: 4326: 4314: 4305: 4266: 4235: 4216: 4197: 4178: 4123: 4093: 4064:(1): 117–120. 4044: 4028: 3984: 3940: 3935:Porphyria news 3922: 3919:978-1437704341 3905: 3854: 3824: 3797: 3772: 3731: 3704: 3697: 3668: 3641: 3582: 3567: 3556:(51): 3085–6. 3550:Orvosi Hetilap 3540: 3497: 3471: 3438: 3431: 3405: 3391: 3371: 3364: 3340: 3309: 3278: 3253: 3235: 3217: 3190: 3151: 3144: 3117: 3092: 3034: 3012: 2993: 2986: 2938: 2916: 2879: 2828: 2799:(12): CD01-2. 2779: 2768: 2748: 2728: 2691:(4): 410–422. 2668: 2657: 2632: 2599: 2570:(4): 527–538. 2543: 2532: 2512: 2452: 2402: 2401: 2399: 2396: 2395: 2394: 2383: 2367: 2364: 2363: 2362: 2355:Giuseppe Troni 2342: 2335: 2333: 2322: 2315: 2313: 2299: 2292: 2289: 2288: 2279:Isabel Allende 2272: 2265: 2255: 2249: 2243: 2236:Francis Willis 2229: 2215: 2192: 2185:Andrew Roberts 2158:Purple Secret, 2124: 2121: 2051: 2048: 2036:Barend Stokvis 2027: 2024: 1992: 1989: 1979: 1976: 1952:hypertrichosis 1925: 1922: 1905: 1902: 1870: 1867: 1780: 1777: 1768:alert bracelet 1763: 1760: 1743: 1740: 1732: 1729: 1725:bulbar paresis 1691: 1688: 1671: 1668: 1666: 1663: 1661: 1658: 1621: 1618: 1607:false negative 1590:. In cases of 1563: 1560: 1558: 1555: 1517:polyneuropathy 1513:abdominal pain 1340: 1337: 1336: 1335: 1328:pentoxifylline 1304: 1290: 1280:antipsychotics 1276: 1258: 1180: 1158: 1131: 1126: 1104:antiretroviral 1100: 1078: 1071:nitrofurantoin 1047: 1024: 1006: 984: 961: 958: 930: 929: 926: 923: 918: 915: 912:ferrochelatase 909: 902: 901: 896: 893: 888: 885: 880: 873: 872: 869: 866: 861: 858: 853: 847: 846: 843: 837: 832: 829: 824: 817: 816: 813: 803: 797: 794: 789: 782: 781: 778: 772: 767: 764: 759: 752: 751: 748: 741: 736: 733: 727: 720: 719: 716: 713: 708: 705: 699: 692: 691: 686: 683: 680: 677: 671: 661: 658: 653: 650: 649: 648: 644: 640: 636: 632: 629: 622:hypertrichosis 614: 583: 580: 568:hallucinations 532:abdominal pain 528:nervous system 508: 505: 494: 493: 452: 450: 443: 437: 434: 375:, alcohol, or 341:in one of the 284:abdominal pain 276:nervous system 263: 262: 259: 255: 254: 251: 247: 246: 240:Lead poisoning 237: 231: 230: 224: 218: 217: 211: 207: 206: 203: 199: 198: 171:abdominal pain 167: 161: 160: 147: 141: 140: 138: 137: 67: 65: 61: 60: 56: 55: 47: 46: 38: 15: 13: 10: 9: 6: 4: 3: 2: 5285: 5284: 5273: 5270: 5268: 5265: 5263: 5260: 5258: 5255: 5253: 5250: 5249: 5247: 5231: 5227: 5226: 5222: 5220: 5216: 5215: 5211: 5209: 5205: 5204: 5200: 5199: 5196: 5192: 5185: 5181: 5180: 5176: 5174: 5170: 5169: 5165: 5161: 5159: 5155: 5151: 5150: 5146: 5142: 5141: 5138: 5133: 5129: 5113: 5110: 5108: 5105: 5104: 5102: 5099: 5094: 5088: 5085: 5083: 5080: 5078: 5075: 5074: 5072: 5069: 5068:unconjugated: 5064: 5061: 5058: 5053: 5049: 5039: 5036: 5034: 5031: 5029: 5026: 5024: 5021: 5020: 5018: 5015: 5010: 5004: 5000: 4997: 4995: 4992: 4991: 4989: 4986: 4981: 4975: 4972: 4970: 4967: 4966: 4964: 4961: 4956: 4953: 4950: 4945: 4941: 4936: 4932: 4927: 4920: 4915: 4913: 4908: 4906: 4901: 4900: 4897: 4891: 4888: 4886: 4883: 4881: 4877: 4874: 4873: 4869: 4860: 4854: 4850: 4849: 4841: 4838: 4833: 4827: 4823: 4822:HarperCollins 4819: 4812: 4809: 4804: 4800: 4795: 4790: 4786: 4782: 4778: 4774: 4770: 4763: 4760: 4755: 4751: 4745: 4742: 4737: 4731: 4727: 4723: 4716: 4713: 4700: 4696: 4692: 4685: 4682: 4669: 4665: 4663:9780387785189 4659: 4655: 4654: 4646: 4643: 4638: 4631: 4628: 4623: 4619: 4615: 4611: 4607: 4603: 4599: 4595: 4588: 4585: 4580: 4576: 4572: 4568: 4564: 4560: 4556: 4552: 4551: 4543: 4540: 4536: 4530: 4527: 4522: 4516: 4512: 4508: 4501: 4498: 4493: 4489: 4484: 4479: 4475: 4471: 4467: 4463: 4459: 4452: 4449: 4444: 4440: 4435: 4430: 4426: 4422: 4418: 4414: 4413: 4408: 4401: 4398: 4393: 4389: 4384: 4379: 4375: 4371: 4367: 4363: 4359: 4352: 4349: 4346:, 7 May 1999. 4345: 4342: 4338: 4335: 4330: 4327: 4323: 4318: 4315: 4309: 4306: 4301: 4297: 4293: 4289: 4284:Reprinted in 4281: 4277: 4270: 4267: 4254: 4250: 4246: 4239: 4236: 4231: 4227: 4220: 4217: 4213: 4209: 4206: 4201: 4198: 4194: 4190: 4187: 4182: 4179: 4174: 4170: 4166: 4162: 4158: 4154: 4150: 4146: 4142: 4138: 4134: 4127: 4124: 4111: 4107: 4103: 4097: 4094: 4089: 4085: 4081: 4077: 4072: 4067: 4063: 4059: 4055: 4048: 4045: 4042: 4041:0-340-90616-2 4038: 4032: 4029: 4025: 4024:public domain 4006: 4002: 4000: 3994: 3988: 3985: 3981: 3980:public domain 3962: 3958: 3956: 3950: 3944: 3941: 3936: 3932: 3926: 3923: 3920: 3916: 3909: 3906: 3901: 3897: 3892: 3887: 3882: 3877: 3874:(3): 140–53. 3873: 3869: 3865: 3858: 3855: 3850: 3846: 3842: 3838: 3831: 3829: 3825: 3820: 3816: 3813:(6): 413–18. 3812: 3808: 3801: 3798: 3786: 3782: 3776: 3773: 3768: 3764: 3759: 3754: 3750: 3746: 3742: 3735: 3732: 3719: 3715: 3708: 3705: 3700: 3694: 3690: 3686: 3682: 3678: 3672: 3669: 3656: 3652: 3645: 3642: 3637: 3633: 3628: 3623: 3619: 3615: 3610: 3605: 3601: 3597: 3593: 3586: 3583: 3578: 3571: 3568: 3563: 3559: 3555: 3551: 3544: 3541: 3536: 3532: 3528: 3524: 3520: 3516: 3513:(6): 439–50. 3512: 3508: 3501: 3498: 3485: 3481: 3475: 3472: 3461: 3457: 3453: 3449: 3442: 3439: 3434: 3428: 3424: 3419: 3418: 3409: 3406: 3394: 3392:9780128144534 3388: 3384: 3383: 3375: 3372: 3367: 3361: 3357: 3353: 3352: 3344: 3341: 3328: 3324: 3320: 3313: 3310: 3297: 3293: 3289: 3286:Tishler, PV. 3282: 3279: 3267: 3263: 3257: 3254: 3249: 3248:www.orpha.net 3245: 3239: 3236: 3231: 3227: 3221: 3218: 3213: 3209: 3205: 3201: 3194: 3191: 3178: 3174: 3170: 3166: 3162: 3155: 3152: 3147: 3141: 3137: 3130: 3128: 3126: 3124: 3122: 3118: 3106: 3102: 3096: 3093: 3088: 3084: 3079: 3074: 3070: 3066: 3062: 3058: 3054: 3047: 3045: 3043: 3041: 3039: 3035: 3031: 3027: 3024: 3019: 3017: 3013: 3009: 3005: 3002: 2997: 2994: 2989: 2983: 2978: 2977: 2967: 2965: 2963: 2961: 2959: 2957: 2955: 2953: 2951: 2949: 2947: 2945: 2943: 2939: 2927: 2920: 2917: 2913: 2902: 2898: 2894: 2890: 2883: 2880: 2875: 2871: 2866: 2861: 2856: 2851: 2847: 2843: 2839: 2832: 2829: 2824: 2820: 2815: 2810: 2806: 2802: 2798: 2794: 2790: 2783: 2780: 2775: 2771: 2769:9783642045196 2765: 2761: 2760: 2752: 2749: 2745: 2741: 2740: 2732: 2729: 2724: 2720: 2716: 2712: 2707: 2706:2027.42/34640 2702: 2698: 2694: 2690: 2686: 2679: 2672: 2669: 2664: 2660: 2658:9780123864574 2654: 2650: 2649: 2641: 2639: 2637: 2633: 2622: 2618: 2614: 2610: 2603: 2600: 2595: 2591: 2587: 2583: 2578: 2573: 2569: 2565: 2561: 2554: 2552: 2550: 2548: 2544: 2539: 2535: 2533:9783642045196 2529: 2525: 2524: 2516: 2513: 2501: 2497: 2491: 2489: 2487: 2485: 2483: 2481: 2479: 2477: 2475: 2473: 2471: 2469: 2467: 2465: 2463: 2461: 2459: 2457: 2453: 2440: 2439: 2434: 2428: 2426: 2424: 2422: 2420: 2418: 2416: 2414: 2412: 2410: 2408: 2404: 2397: 2392: 2388: 2387:Lusus Naturae 2384: 2381: 2377: 2373: 2372: 2371: 2365: 2360: 2359:Thomas Hickey 2356: 2345: 2339: 2334: 2325: 2319: 2314: 2310: 2306: 2302: 2296: 2291: 2286: 2285: 2280: 2276: 2273: 2270: 2266: 2263: 2259: 2256: 2253: 2250: 2247: 2244: 2241: 2237: 2233: 2230: 2227: 2223: 2219: 2216: 2213: 2209: 2205: 2201: 2197: 2196:Purple Secret 2193: 2190: 2186: 2182: 2178: 2174: 2173: 2167: 2163: 2159: 2154: 2149: 2146: 2142: 2138: 2134: 2130: 2127: 2126: 2123:Notable cases 2122: 2119: 2114: 2112: 2108: 2107: 2101: 2097: 2095: 2091: 2090:David Dolphin 2087: 2083: 2079: 2077: 2072: 2067: 2065: 2061: 2057: 2049: 2047: 2045: 2039: 2037: 2033: 2025: 2023: 2019: 2016: 2011: 2008: 2003: 2001: 1996: 1990: 1988: 1984: 1977: 1975: 1972: 1971:phototoxicity 1968: 1964: 1963:afamelanotide 1959: 1957: 1956:beta carotene 1953: 1949: 1944: 1942: 1938: 1934: 1929: 1923: 1921: 1919: 1915: 1911: 1903: 1901: 1899: 1895: 1891: 1887: 1882: 1880: 1876: 1868: 1866: 1864: 1860: 1856: 1854: 1850: 1849:heme arginate 1846: 1842: 1838: 1833: 1829: 1825: 1821: 1819: 1815: 1811: 1806: 1802: 1798: 1794: 1790: 1786: 1778: 1776: 1774: 1769: 1761: 1759: 1757: 1756:phenothiazine 1753: 1749: 1741: 1739: 1737: 1730: 1728: 1726: 1722: 1718: 1713: 1710: 1708: 1704: 1700: 1699:heme arginate 1696: 1689: 1687: 1685: 1684:hyponatraemia 1681: 1677: 1669: 1664: 1659: 1657: 1654: 1651: 1648: 1645: 1643: 1639: 1638:liver disease 1635: 1631: 1627: 1619: 1617: 1615: 1610: 1608: 1604: 1599: 1597: 1593: 1589: 1585: 1581: 1577: 1573: 1569: 1561: 1556: 1554: 1552: 1548: 1544: 1543:PROTO oxidase 1540: 1536: 1532: 1531: 1526: 1522: 1519:), while the 1518: 1514: 1510: 1506: 1502: 1498: 1494: 1490: 1486: 1482: 1477: 1475: 1471: 1467: 1463: 1459: 1455: 1451: 1447: 1444: 1441: 1437: 1433: 1429: 1425: 1420: 1418: 1414: 1410: 1406: 1401: 1397: 1392: 1390: 1386: 1382: 1378: 1374: 1370: 1366: 1362: 1354: 1353:mitochondrion 1350: 1345: 1338: 1333: 1329: 1325: 1321: 1317: 1313: 1309: 1305: 1303: 1299: 1295: 1291: 1289: 1285: 1281: 1277: 1275: 1271: 1267: 1263: 1259: 1257: 1253: 1249: 1245: 1241: 1237: 1233: 1229: 1225: 1221: 1217: 1213: 1209: 1205: 1201: 1197: 1193: 1189: 1185: 1181: 1179: 1175: 1171: 1167: 1163: 1159: 1156: 1152: 1148: 1144: 1140: 1139:carbamazepine 1136: 1132: 1130: 1127: 1124: 1120: 1116: 1112: 1109: 1106:medications ( 1105: 1101: 1098: 1094: 1090: 1086: 1082: 1079: 1076: 1075:metronidazole 1072: 1068: 1064: 1060: 1056: 1052: 1048: 1045: 1041: 1037: 1033: 1029: 1025: 1022: 1018: 1017:phenobarbital 1014: 1010: 1007: 1004: 1000: 996: 992: 991:glibenclamide 988: 987:Sulfonylureas 985: 982: 978: 977:sulfasalazine 974: 970: 967: 966: 965: 959: 957: 954: 952: 948: 944: 940: 936: 927: 924: 922: 919: 916: 913: 910: 907: 904: 903: 897: 894: 892: 889: 886: 884: 881: 878: 875: 874: 870: 867: 865: 862: 859: 857: 854: 852: 849: 848: 845:1 in 500,000 844: 842: 838: 836: 833: 830: 828: 825: 822: 819: 818: 814: 812: 808: 804: 802: 798: 795: 793: 790: 787: 784: 783: 779: 777: 773: 771: 768: 765: 763: 760: 757: 754: 753: 749: 746: 742: 740: 737: 734: 731: 728: 725: 722: 721: 717: 714: 712: 709: 706: 703: 700: 697: 694: 693: 690: 687: 684: 681: 678: 676: 672: 669: 668: 665: 659: 657: 651: 645: 641: 637: 633: 630: 627: 623: 619: 615: 612: 608: 607: 606: 603: 599: 597: 593: 589: 581: 579: 577: 571: 569: 565: 561: 557: 553: 547: 545: 541: 537: 533: 529: 525: 521: 517: 513: 506: 500: 490: 487: 479: 469: 465: 459: 458: 453:This section 451: 447: 442: 441: 435: 433: 431: 427: 423: 419: 415: 411: 405: 403: 399: 393: 390: 386: 382: 378: 374: 370: 366: 365: 360: 356: 352: 348: 344: 340: 335: 333: 329: 325: 321: 317: 313: 309: 305: 301: 297: 294:, confusion, 293: 289: 285: 281: 277: 273: 269: 260: 256: 252: 248: 245: 241: 238: 236: 232: 229: 225: 223: 219: 216: 212: 208: 204: 200: 197:with sunlight 196: 192: 188: 184: 181:, confusion, 180: 176: 172: 168: 166: 162: 159: 155: 151: 148: 146: 142: 133: 98: 69: 68: 66: 64:Pronunciation 62: 57: 53: 48: 43: 37: 33: 29: 22: 5223: 5212: 5201: 5177: 5162: 5143: 5097: 5067: 5013: 4985:cytoplasmic: 4984: 4959: 4934: 4847: 4840: 4817: 4811: 4776: 4772: 4762: 4744: 4725: 4721: 4715: 4703:. Retrieved 4699:the original 4694: 4684: 4672:. Retrieved 4656:. Springer. 4652: 4645: 4630: 4597: 4593: 4587: 4554: 4548: 4542: 4529: 4511:Bantam Books 4506: 4500: 4465: 4461: 4451: 4416: 4410: 4400: 4365: 4361: 4351: 4329: 4321: 4317: 4308: 4291: 4290:(in Dutch). 4287: 4279: 4278:(in Dutch). 4275: 4269: 4257:. Retrieved 4253:the original 4248: 4238: 4229: 4225: 4219: 4200: 4181: 4140: 4136: 4126: 4114:. Retrieved 4106:ec.europa.eu 4105: 4096: 4061: 4057: 4047: 4031: 4009:. Retrieved 3996: 3987: 3965:. Retrieved 3952: 3943: 3934: 3925: 3908: 3871: 3867: 3857: 3843:(2): 112–5. 3840: 3836: 3810: 3807:Laeknabladid 3806: 3800: 3788:. Retrieved 3784: 3775: 3751:(9): 694–5. 3748: 3744: 3734: 3722:. Retrieved 3718:the original 3707: 3684: 3671: 3659:. Retrieved 3655:the original 3644: 3599: 3595: 3585: 3576: 3570: 3553: 3549: 3543: 3510: 3506: 3500: 3488:. Retrieved 3484:the original 3474: 3463:, retrieved 3451: 3441: 3416: 3408: 3396:. Retrieved 3381: 3374: 3350: 3343: 3331:. 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