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Porphyria / p ɔːr ˈ f ɪr i ə / is a group of disorders in which substances called porphyrins build up in the body, adversely affecting the skin or nervous system. [1] The types that affect the nervous system are also known as acute porphyria, as symptoms are rapid in onset and short in duration. [1]
Porphyria cutanea tarda is the most common subtype of porphyria. [1] The disease is named because it is a porphyria that often presents with skin manifestations later in life. The disorder results from low levels of the enzyme responsible for the fifth step in heme production. Heme is a vital molecule for all of the body's organs.
The mainstays of treatment are removal from the source of lead and, for people who have significantly high blood lead levels or who have symptoms of poisoning, chelation therapy. [232] Treatment of iron, calcium, and zinc deficiencies, which are associated with increased lead absorption, is another part of treatment for lead poisoning. [233]
Hematin and heme arginate is the treatment of choice during an acute attack. Heme is not a curative treatment, but can shorten attacks and reduce the intensity of an attack. Side-effects are rare but can be serious. [citation needed] Pain is extremely severe and almost always requires the use of opiates to reduce it to tolerable levels. Pain ...
Erythropoietic protoporphyria (or commonly called EPP) is a form of porphyria, which varies in severity and can be very painful.It arises from a deficiency in the enzyme ferrochelatase, leading to abnormally high levels of protoporphyrin in the red blood cells (erythrocytes), plasma, skin, and liver. [2]
Symptoms generally appear from 12 to 24 hours after exposure and typically last 24 to 72 hours. It is highly contagious, and people can continue spreading the virus for two days after symptoms ...
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Heme arginate (or haem arginate) is a compound of heme and arginine used in the treatment of acute porphyrias. [1] [2] This heme product is only available outside the United States and is equivalent to hematin. [3] Heme arginate is a heme compound, whereby L-arginine is added to prevent rapid degradation.