What are the different types of porphyria?
What are the different types of porphyria?
The specific names of the eight types of porphyrias are:
- Delta-aminolevulinate-dehydratase deficiency porphyria.
- Acute intermittent porphyria.
- Hereditary coproporphyria.
- Variegate porphyria.
- Congenital erythropoietic porphyria.
- Porphyria cutanea tarda.
- Hepatoerythropoitic porphyria.
- Erythropoietic protoporphyria.
What are the two types of porphyria?
There are two general categories of porphyria: acute, which mainly affects the nervous system, and cutaneous, which mainly affects the skin. Some types of porphyria have both nervous system symptoms and skin symptoms.
Can you have more than one type of porphyria?
Can I have more than one type of porphyria? Because all porphyrias are uncommon, it is very unlikely that more than one type of porphyria will occur in the same family, or that a person with one type of porphyria will go on to develop another type.
How many cases of porphyria are there?
The exact prevalence of porphyria is unknown, but it probably ranges from 1 in 500 to 1 in 50,000 people worldwide. Overall, porphyria cutanea tarda is the most common type of porphyria.
When should you suspect porphyria?
Therefore, it is currently recommended that patients undergo screening by liver imaging for early detection at least yearly after age 50, especially if porphobilinogen (PBG) remains elevated.
What triggers porphyria?
Porphyria can be triggered by drugs (barbiturates, tranquilizers, birth control pills, sedatives), chemicals, fasting, smoking, drinking alcohol, infections, emotional and physical stress, menstrual hormones, and exposure to the sun. Attacks of porphyria can develop over hours or days and last for days or weeks.
Is porphyria a mental illness?
Porphyria is important in psychiatry as it may present with only psychiatric symptoms; it may masquerade as a psychosis and the patient may be treated as a schizophrenic person for years; the only manifestation may be histrionic personality disorder which may not receive much attention.
How is porphyria detected?
To diagnose porphyrias, laboratories measure porphyrins and their precursors in urine, blood, and/or stool. Testing may include measurement of one or more of the following: Porphobilinogen (PBG), a porphyrin precursor, in urine. Delta-aminolevulinic acid (ALA), another porphyrin precursor, in urine.
Does porphyria make you crave blood?
The type of porphyria that can cause serious gum and skin disfigurement, congenital erythropoetic porphyria, is extremely rare, with only some 200 cases ever having been diagnosed. Victims do not crave blood, and in any case, ingested blood would be of no help for treatment of the disease.
How do you test for porphyria?
How is porphyria treated today?
Acute porphyrias Treatment may include: Injections of hemin, a medication that is a form of heme, to limit the body’s production of porphyrins. Intravenous sugar (glucose), or sugar taken by mouth, if able, to maintain an adequate intake of carbohydrates.
Can porphyria make you go crazy?
Acute intermittent porphyria mimics a variety of commonly occurring disorders and thus poses a diagnostic quagmire. Psychiatric manifestations include hysteria, anxiety, depression, phobias, psychosis, organic disorders, agitation, delirium, and altered consciousness ranging from somnolence to coma.
What is the prognosis of porphyria?
About prognosis: The ‘prognosis’ of Acute intermittent porphyria usually refers to the likely outcome of Acute intermittent porphyria. The prognosis of Acute intermittent porphyria may include the duration of Acute intermittent porphyria, chances of complications of Acute intermittent porphyria, probable outcomes, prospects for recovery,…
What is acute intermittent porphyria and variegate porphyria?
Acute porphyrias. The acute porphyrias are acute intermittent porphyria (AIP), variegate porphyria (VP) , aminolevulinic acid dehydratase deficiency porphyria (ALAD) and hereditary coproporphyria (HCP). These diseases primarily affect the nervous system, resulting in episodic crises known as acute attacks.
What is the cause of porphyria?
Porphyria is a rare disease caused by excessive porphyrins secretion, which deposits in the body. Porphyria is caused by genetic mutation resulting in malfunction of enzyme, which metabolizes the porphyrin and heme.
What is the history of porphyria?
History of Porphyria A Little Bit of History. 1841 The term ‘porphyrin’ comes from the Greek word, porphyus, meaning reddish-purple. It was first thought that the reddish color of blood was from iron. One early scientist performed an experiment to prove that this was not the case. He washed dried blood with concentrated sulfuric acid to free the iron.