Nutritional diet for high urinary porphyrins in urine

       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.  Acute intermittent hematoporphyria is more common and is an autosomal dominant disorder caused by a deficiency in PBG deaminase (uroporphyrinogen synthase). This deficiency reduces the conversion of PBG to uroporphyrinogen III in the liver, and the resulting impaired synthesis of heme causes enhanced action of ALA synthase, resulting in increased synthesis of ALA and PBG and increased excretion from the urine. Late-onset cutaneous hematoporphyria is the most common hematoporphyria and is caused by a deficiency of uroporphyrinogen decarboxylase in the liver. It is autosomal dominant. Cases are sporadically distributed, with more males than females, and most patients have no family history. Some individuals have a biochemical defect of reduced enzymatic activity of uroporphyrinogen decarboxylase, but urinary excretion of uroporphyrins is not necessarily increased and clinically significant symptoms are not always present. The synergistic effect of the genetic defect and acquired factors such as alcoholism, intrahepatic iron overload, liver damage, and female hormones further decreases the activity of uroporphyrin decarboxylase or stimulates the formation of ALA, resulting in a marked increase in the formation of uroporphyrins, leading to the development of delayed cutaneous porphyria.  Such patients should eat high-protein nutritious food; vitamin and mineral-rich food; high-calorie easy-to-digest food; avoid eating greasy and hard-to-digest food; avoid eating fried, smoked, barbecued, raw and cold, stimulating food; avoid eating high-salt and high-fat food. The following is an introduction to its dietary treatment.  1, reasonable nutrition Food should be as diverse as possible, coarse and fine grains with balanced. Eat more beans, mushroom food.  2, nutritious Eat more nutritious, well absorbed, well digested food, more high protein, multivitamin, low animal fat food.  3, fresh and light diet Do not eat stale and spoiled or irritating things; advocate more fresh fruits and vegetables with vitamin C; choose light-tasting, easy-to-digest food to keep the bowels open; drink more yogurt, mushroom soup, seaweed soup, yellow croaker soup as appropriate.  4, eat less spontaneous, stimulating food eat less beef and mutton, dog meat, chicken, fish and shrimp and other spontaneous food, do not eat fried and smoked food, do not eat heavy food. Avoid smoking, alcohol, spicy and rough food.