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the treatment method of porphyria disease, how to do porphyria disease, porphyria disease medication

the treatment method of porphyria disease, how to do porphyria disease, porphyria disease medication

Knowledge of diagnosis and treatment of porphyria

Visiting Department: Dermatology DepartmentGeneral treatment of porphyria

Western medicine treatment of porphyria

1. Erythropoietic protoporphyria

(1) Oral β-carotene: It is the only effective drug to control this disease. Taking 3mg/kg daily for 4 ~ 6 weeks continuously, and reducing the dose for 2 ~ 3 months after yellow staining of palms and plantars can obviously prolong the tolerance time of patients to sunlight and make them adapt to normal life and work. The mechanism may be through the formation of a protective layer of β-carotene in the skin and the quenching of oxygen free radicals, which blocks the phototoxic damage caused by porphyrin. Since it has no effect on porphyrin metabolic disorder of this disease, it is necessary to take this drug regularly during and before the onset season every year. Except for skin yellowing (which can subside by itself after reduction and suspension of administration), there are generally no other adverse reactions.

(2) Vitamin B6 high-dose therapy: 100mg is taken orally once in the morning, and then once every hour, and taken continuously for 10 times, which can improve symptoms. Its mechanism may be related to promoting the production of nicotinic acid and nicotinamide and alleviating photoallergy.

(3) Oral administration of nicotinamide and vitamin E.

(4) Coleenamine (cholestylamine) 4g, 3 times a day, taken before meals. The combination of protoporphyrin and protoporphyrin in the intestine blocks the enterohepatic circulation of protoporphyrin thus promoting the elimination of excessive protoporphyrin which is effective in preventing the progress of hepatobiliary lesions of this disease.

2. Delayed cutaneous porphyria

(1) Chloroquine and hydroxychloroquine:

It can increase porphyrin release and excretion in liver, thus improving symptoms. Generally, long-term small-dose oral administration is adopted, and the usage of chloroquine is 0.125 g each time, twice a week, and it is taken continuously for at least 10 months. Clinical symptoms are expected to be relieved in about 4 months, followed by a decrease in uroporphyrin in urine. In recent years, a small dose of hydroxychloroquine has been used for treatment, that is, 0.1 g hydroxychloroquine is taken orally twice a day for 2 weeks, and then reduced to only 2 days a week for 4 ~ 8 months. The disease can be controlled.

(2) Venous bloodletting:

Its mechanism is to deplete heme, which makes porphyrin intermediate products produced excessively in this disease form heme, and to deplete iron stored in the body and improve iron deposition in the liver. Bloodletting is usually performed once every 1 ~ 2 weeks, 400ml each time, 8 ~ 12 times continuously, until hemoglobin drops to 110 ~ 120g/L, which can relieve the disease for a long time. It can be used except for severe cardiopulmonary diseases and anemia, and is a safe and effective therapy.

(3) Others:

Deferoxamine was injected subcutaneously once a day, with a dose of 1.5 g, 5 times a week. Or oral administration of deferoxamine 30mg/kg every day for 1 week every 3 months for 6 ~ 12 months can improve liver iron deposition. Patients who have serious complications and are not suitable for the above two methods can use recombinant erythropoietin, which is effective for PCT patients induced by long-term hemodialysis, and 150U/kg is given intravenously during each dialysis; In addition, oral administration of sodium bicarbonate at the same time can facilitate porphyrin excretion with urine. Others such as nicotinamide, vitamin B6, vitamin E, etc. can also be taken orally.

3. Congenital erythropoietic porphyria

Mainly for symptomatic treatment. Severe long-term hemolysis is a clear indication of splenectomy. After splenectomy, hemolytic anemia can be improved, erythropoiesis can be inhibited, excessive formation of porphyrin can be reduced, and photosensitivity of skin can be reduced, which can play a more lasting relieving role in this disease.

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