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Porphyria is a disorder in which the body produces too much of the chemical porphyrin. Porphyrin is used to make heme, the part of blood that carries oxygen. Heme also gives blood its color. Any circulating porphyrin the body doesn't use is excreted in urine and stool. When the body produces and excretes too much porphyrin, as happens with porphyria, not enough heme remains to keep a person healthy. Porphyria affects either the nervous system or the skin. When porphyria affects the nervous system, it can cause chest pain, abdominal pain, muscle cramps, weakness, hallucinations, seizures, purple-red-colored urine, or mental disorders like depression, anxiety, and paranoia. When porphyria affects the skin, blisters, itching, swelling, and sensitivity to the sun can result. Porphyria is an inherited condition. Attacks of the disease can be triggered by drugs (barbiturates, tranquilizers, birth control pills, sedatives), chemicals, certain foods, and exposure to the sun. Porphyria is diagnosed through tests on blood, urine, and stool. It can be treated with medicines to relieve symptoms, a drug called hemin (which is like heme), or a high-carbohydrate diet.
 
 
 
Definition
The term porphyria refers to a group of at least eight disorders that differ clinically from each other, but all share the same problem of accumulation of "porphyrins" or "porphyrin precursors" in the body. Porphyrin is a building block for heme. And heme is the iron-containing part of the hemoglobin in red blood cells that carries oxygen to all of the organs in our body.
The build up of the prophyrins in the body cause symptoms in two major organ systems: the nervous system and the skin.
Effects on the nervous system occur in the acute porphyrias. The diagnosis is often delayed because the symptoms are nonspecific. Some patients have attacks in which the nerves of the body do not function properly. Abdominal pain and weakness can be a symptom.
Skin manifestations can include burning, blistering and scarring of sun-exposed areas.
The terms "porphyrin" and "porphyria" are derived from the Greek word "porphyrus" meaning purple. Urine from some porphyria patients may be reddish in color due to the presence of excess porphyrins and related substances, and the urine may darken after standing in the light. Excess prophyrins are also excreted in the stool.
Some types of porphyria have their onset in early childhood, some at puberty, and others during adulthood. The onset most frequently occurs between the ages of 20 and 40. The disease affects men less often than women, in whom attacks are related to the menstrual cycle. Long periods may separate attacks, which can be precipitated by drugs, infections, alcohol consumption and dieting.
Causes
Most types of porphyrias are inherited. One subtype, porphyria cutanea tarda, is acquired. The porphyrias may be inherited as either autosomal dominant (caused by inheriting one abnormal copy of the gene), or autosomal recessive (requires inheritance of two abnormal genes) traits depending on which type of porphyria is involved.
Risk Factors
A risk factor is something that increases your chances of getting a disease or condition. The most common risk factor for porphyria is having a family member with this disease. Also, whites are at greater risk than blacks or Asians.
If you have porphyria, certain factors can increase your risk of a porphyria attack. These include: drugs, chemicals, alcohol, dieting, and sunlight. Triggers for specific types of porphyria are listed below:
Acute Intermittent Porphyria
Drugs, such as:
Barbiturates
Sulfa drugs
Seizure drugs
Steroid hormones such as:
Estrogen
Progesterone
Hormonal changes related to the menstrual cycle
Weight-loss diets or fasting
Infections
Alcohol
Stress
Surgery
Cigarette smoke
Porphyria Cutanea Tarda
Iron
Alcohol
Estrogens
Hydrocarbons
Certain pesticides or chemicals
Erythropoietic Protoporphyria
Sunlight
Weight-loss diets or fasting
Congenital Erythropoietic Protoporphyria
Sunlight
Symptoms
Porphyria can cause skin or nervous system problems. Specific symptoms depend on the type.
Acute Intermittent Porphyria(AIP) -- Inherited autosomal dominant trait
Nervous system symptoms usually occur after puberty. Gastrointestinal symptoms are caused by nerves that affect the intestinal tract. Attacks can last from days to weeks. Symptoms of future attacks resemble the initial episode, and may include:
Abdominal pain and cramping
Nausea and vomiting
Constipation
Painful urination or urinary retention
Pain in limbs, head, neck, or chest
Muscle weakness
Loss of sensation
Tremors
Sweating
Rapid heart rate
High blood pressure
Breathing problems
Heart arrhythmia
Seizures
Mental symptoms such as:
Hallucinations
Restlessness
Depression
Anxiety
Insomnia
Confusion
Paranoia
Porphyria Cutanea Tarda (PCT) -- The most common porphyria; most are not inherited, but some are autosomal dominant trait
Symptoms may include:
Sun sensitivity
Sun-exposed skin is fragile
Minor injury may damage the skin
Blisters on the face, hands, arms, feet, and legs
Skin heals slowly
Skin susceptible to infection
Skin thickens and scars
Skin color changes
Excess hair growth
Reddish urine, in infancy or childhood
Erythropoietic Protoporphyria(EPP) -- Inherited as an autosomal dominant trait
Symptoms may include:
Sun sensitivity
Redness or swelling, but usually no blisters
Itching or burning sensation
Long-term skin and nail changes
Gallstones
Congenital Erythropoietic Protoporphyria(CEP) -- Extremely rare Inherited autosomal recessive
Symptoms may include:
Reddish urine, in infancy
Sun sensitivity, beginning in early infancy
Sun-exposed skin is fragile
Blisters on sun-exposed skin
Blisters open and are prone to infection
Skin color may change
Skin thickens
Excess hair growth
Scarring
Reddish-brown teeth
Enlarged spleen
Hemolytic anemia
Diagnosis
The doctor will ask about symptoms as well as medical and family history. A physical examination will also be done.
Tests differ for the various types of porphyria, and may include blood, urine, and/or stool tests to check for excess porphyrin or to check for a specific enzyme deficiency. In some cases specific genetic testing may be available as well.
Treatment
For all types of porphyria, treatment includes the following:
Avoiding known triggers and drugs that can precipitate an attack
Eating a high-carbohydrate diet
Porphyria that affects the skin, require special attention to protect the skin from injury and/or infection.
Specific treatment depends on the type of porphyria.
Acute Intermittent Porphyria
You may need to be hospitalized during an attack. In the hospital, you may be given the following:
Heme by vein (intravenous) in the form of hematin, heme albumin, or heme arginate
Glucose by vein
Drugs to control symptoms such as pain, nausea, anxiety, and insomnia
Porphyria Cutanea Tarda
Treatment may include:
Blood removal weekly to monthly
Low doses of antimalarial drugs, such as:
Chloroquine (Aralen Phosphate)
Hydroxychloroquine (Plaquenil)
Radiology imaging to monitor for increased risk of liver cancer
Erythropoietic Protoporphyria
Treatment may include:
Oral beta-carotene
Maintaining normal iron levels with food or supplements
Medication to aid excretion of porphyrins in stool, such as:
Activated charcoal
Cholestyramine
Blood transfusions
Heme by vein in the form of hematin, heme albumin, or heme arginate
Spleen removal
Congenital Erythropoietic Protoporphyria
Treatment may include:
Oral beta-carotene
Oral charcoal, to aid excretion of porphyrins in stool
Blood transfusions
Spleen removal
Bone marrow transplantation
Prevention
Genetic testing may identify people at risk for porphyria. If there are people in your family with the diagnosis of porphyria, you may be eligible for testing if that family member has had genetic testing and that testing showed a change in the DNA; that change can then be looked for in you.
A genetic counselor can review your family history and risks for this disorder in you and your offspring and discuss appropriate testing that is available for you.
Although the genetic mutation cannot be corrected, attacks can be anticipated, prevented, or controlled. Steps to avoid porphyria attacks and complications include the following:
Avoid drugs and other triggers
Protect skin from injury or infection
 

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