Edera Methemoglobinemia

Eder Methemoglobinemia: Description and Treatment

Eder Methemoglobinemia is a rare disease that causes a decrease in the blood's ability to carry oxygen. This occurs due to improper formation of methemoglobin, a form of hemoglobin that cannot bind to oxygen. The disease was first described by German pathologist Eder in the 1950s.

Methemoglobinemia can be inherited (genetic form) or result from exposure to certain chemicals in the body (nitrate intolerance, aniline dyes, sulfonamides, etc.). In most people, the level of methemoglobin is less than 1% of the total hemoglobin, but in people with methemoglobinemia the level can reach 10-20% or even higher.

Symptoms of methemoglobinemia can vary depending on how high your methemoglobin levels are. Mild methemoglobinemia may have no symptoms or cause mild shortness of breath and fatigue. High levels of methemoglobin can cause short-term loss of consciousness, seizures, palpitations and headache.

The diagnosis of methemoglobinemia can be made using a blood test to measure methemoglobin levels. Treatment depends on the cause of the disease. If methemoglobinemia is caused by genetic factors, treatment may include blood transfusions or transfusions. If the cause is contact with chemicals, contact with them must be avoided.

If drug-induced methemoglobinemia occurs, the offending drug should be stopped. If this is not possible, then treatment of methemoglobinemia with special medications may be required.

Overall, methemoglobinemia is a rare disease that can have serious consequences. However, with early diagnosis and proper treatment, most people with methemoglobinemia can live normal lives. If you suspect you have methemoglobinemia, see your doctor for diagnosis and treatment.

Edera-Methemoglobin-Acidemia or endogenous porphyria is a rare genetically determined disorder of bilirubin metabolism (more precisely, the prometabolite - porphobilinogen) in which there is a pathological accumulation of methemoglobin and toxic metabolites (protoporphyrins) in tissues.

The disease was first described by American pediatrician Frederick Edwards in 1893. However, this disorder was classified as a disease only at the beginning of the last century - on May 1, 1905, physician Peter Abelson published a brief report in a British medical journal about two cases of porphyria.

The disease manifests itself as severe attacks of intense abdominal pain and is accompanied by signs of liver and cardiovascular failure, jaundice. It occurs in a wide variety of countries and equally in both sexes around the world, but the highest incidence of diagnosis occurs in East and South Asia. In Russia this is an extremely rare disease.

Attacks of Eder-Methemalgia can be provoked by: - ​​excessive ultraviolet radiation, - strong physical activity, including during sports, - injuries, even minor ones, - nervous overstrain, stress. During an attack, increased pressure leads to a crisis. Stroke and heart failure are also dangerous. Spasms of the vessels through which blood comes from the intestines to the liver can cause fatty hepatosis. Attacks can also be triggered by intoxication of the body. For example, lead poisoning against the background of EP can be extremely acute: within a few hours, the body temperature increases by more than 0.5 degrees Celsius, and jaundice develops. It stays at the level of hyperchromia for quite a long time. For diagnosis, a special test for elevated bilirubin in the blood from a finger prick is used, as well as tests for genetic abnormalities that lead to the development of EC. Treatment of the disease involves dietary nutrition and giving up bad habits.