Hereditary Coproporphyria (HCP)

Hereditary coproporphyria is genetic. It shows a reduction of the enzyme coproporphyrinogen oxidase, and there will be coproporphyrinogen type III in the urine and the stool. The ALA (aminolevulinic acid) and PGP (porphobilinogen) can only be measured in the urine during an attack.

The symptoms comes with an attack and may be seen as: stomach-ache, nausea, vomiting, constipation, rapid pulse and hypertension. Some patients get confused and get hallucinations. By deterioration muscle-weakness may occur. If the peripheral nerves have been hit by muscle-weakness/paralysation ( symmetric or asymmetric), it will take some years before this will disappear. In some cases it may never disappear.
HCP may be light-sensible. If so, there will be blisters and perhaps wounds on the back of the hands. Some patients will be very easily sun-burned.
Attacks in HCP may occur after taken medicine, for example barbiturates and sulfonamides. Furthermore you can see a list of dangerous and unsecure medicine here. Chemicals, hunger, sun-bathing, hormones (cyclus) and stress may also cause symptoms.

The treatment is sugar taken through the mouth or directly into the blood, depending on the intensity of the attack. It may be necessary to transfuse haem. Besides this the treatment will be directed against the present symptoms, considering which medicine the patient may stand.

You ought to consult your doctor: if you have the symptoms of HCP and need treatment.

Prophylaxis: Attacks may be prevented by eating food with lots of fine carbohydrates, avoiding dangerous and unsafe drugs, and avoiding fast and stress. By light-sensibility you ought to be carefull with the combination: skin and light.

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