What are the prevention methods for high urinary porphyrins?

  High levels of urinary porphyrins in the urine are due to porphyria. Porphyria is a disorder of porphyrin metabolism characterized by increased excretion of porphyrins and porphyrin precursors in the urine and feces. Porphyrias are congenital disorders caused primarily by deficiencies in various enzymes related to heme synthesis and have a family history of occurrence. What are the prevention methods for protoporphyria?  The principles of treatment are to avoid sunlight and to reduce the toxicity of protoporphyrin to the liver.  1, avoid light: patients should avoid direct exposure to sunlight as much as possible, wear dark clothing, or wear special titanium, zinc protective clothing, also can be used externally dihydroxybenzone (dihydroxyacetone), henna protective agent.  2, carotene: eat food containing high carotene, or directly take betacarotene (β-carotene), this drug can play a protective role on the skin of photosensitive people and strengthen the skin’s tolerance to sunlight, its clear mechanism of action is not clear, 20 to 60mg/time, 3 times/d, orally. The dose can be increased or decreased appropriately depending on the patient’s efficacy of the drug or the blood carotenoid level. Patients can take the drug in the spring and summer when the sun is strong. The maximum efficacy appears 1 to 3 months after taking the drug, and the efficacy can be maintained for 1 to 2 months after stopping the drug, and there are generally no obvious adverse reactions.  3.Norferric hemoglobin: n-ferric hemoglobin reduces the production of protoporphyrin by inhibiting the activity of δ-aminoketoglutarate synthase. The dose is 4mg/(kg-d), intravenous drip, 1 course of treatment with 6 days.  4, kaufenamide: Because it can bind protoporphyrin and excrete through feces, it can partially reduce the hepatic and intestinal circulation of protoporphyrin, reduce the level of protoporphyrin in red blood cells and plasma, and also improve the liver damage caused by protoporphyrin. Usage: 4g/time, 3 times/d, orally.  5, other drugs: In the past, treatment with certain antimalarial drugs such as miparin, hydroxychloroquine, etc., also has certain effect on some cases. The adsorption effect of medicinal charcoal can block the hepatic and intestinal circulation of porphyrins, so that the clinical symptoms can be completely relieved. 4g/time, 3 times/d, cool boiled water mixed into a paste and taken orally for 9 months. Some patients can also be treated with adenosine monophosphate or inosine to achieve good results.  Most patients have a good prognosis and generally do not develop permanent disability or affect life expectancy. There are more cases with cholelithiasis and individual cases with chronic intrahepatic cholestasis, which can lead to cirrhosis, and attention should be paid to the prevention and treatment of complications. Prevention should be done by avoiding sun exposure and taking sun protection measures. Avoid eating photosensitive foods.