Definition:
Hereditary coproporphyria (HCP) is a form of hepatic porphyria linked with a deficiency of the enzyme coproporphyrinogen III oxidase.
Diagnosis:
Physicians establish the diagnosis of coproporphyria by demonstrating excess secretion of coproporphyrins in the feces.
Treatment:
While there is no cure for this condition, there are preventative measures people can take to control symptoms. A diet high in carbohydrates, as well as avoidance of aggravating factors (such as alcohol and drug use) can hinder attacks
Symptoms and Signs:
The initial symptom is a condition known as prophyria. Those afflicted experience a range of symptoms including reddish-purple urine, light sensitivity of the skin, and attacks of acute abdominal/nerve pain. These symptoms manifest themselves differently in patients but can be triggered by dieting, infections, or hormonal changes. The use of alcohol and certain drugs such as barbiturates and birth control pills can also trigger these attacks
Causes:
Coproporphyria is the result of a combination of a genetic enzyme defect and acquired causes that become symptomatic in rare cases. In individuals with coproporphyria, the function of coproporphyrinogen oxidase is only 40-60% of normal. Also, most patients with defects in coproporphyrinogen oxidase never display any symptoms. The typical inducers of porphyria are chemicals or situations that boost heme synthesis. This includes fasting and many medications, among others.
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