{"id":13364,"date":"2024-08-23T00:49:05","date_gmt":"2024-08-22T22:49:05","guid":{"rendered":"https:\/\/valintermed.com\/?p=13364"},"modified":"2024-08-23T00:49:05","modified_gmt":"2024-08-22T22:49:05","slug":"porfiriya-pestraya","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/pestilence-porphyria\/","title":{"rendered":"Porphyria motley"},"content":{"rendered":"
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Variegate porphyria is a rare hereditary disorder belonging to the group of porphyrias, characterized by a disorder of hemoglobin synthesis, in particular, inhibition of the activity of enzymes involved in the biosynthesis of porphyrins. The predominant type of porphyria in this case is porphyria caused by a deficiency of enzymes specific to the liver, which leads to the accumulation of porphyrins in the body, as well as their excretion in the urine. The clinical spectrum of this disease is wide and includes acute abdominal pain, psychoneurological disorders, and dermatological manifestations such as photosensitivity and blistering of the skin, which is a consequence of the generation of active oxygen species when accumulated porphyrins are exposed to light.<\/p>\n

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