Marchiafava-Micheli disease - определение. Что такое Marchiafava-Micheli disease
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Что (кто) такое Marchiafava-Micheli disease - определение

DISEASE OF THE BLOOD CHARACTERIZED BY DESTRUCTION OF RED BLOOD CELLS BY THE COMPLEMENT SYSTEM, A PART OF THE BODY'S INNATE IMMUNE SYSTEM
Paroxysmal nocturnal haemoglobinuria; Marchiafava-Micheli Syndrome
  • CD59 protein/Protectin structure
  • CD55 protein/Decay Accelerating Factor structure

Paroxysmal nocturnal hemoglobinuria         
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occurs due to deficiency of the red blood cell surface protein DAF, which normally inhibits such immune reactions.
Marchiafava–Bignami disease         
MEDICAL CONDITION
Marchiafava Bignami disease; Marchiafava disease; Marchiafava syndrome; Central demyelination of corpus callosum; Marchiafava-Bignami disease; Marchiafava-bignami disease
Marchiafava–Bignami disease is a progressive neurological disease of alcohol use disorder, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. The disease was first described in 1903 by the Italian pathologists Amico Bignami and Ettore Marchiafava in an Italian Chianti drinker.
Jacques-Barthélemy Micheli du Crest         
  • Jacques-Barthélemy Micheli du Crest
  • du Crest's panorama of the Alps
GENEVAN CARTOGRAPHER (1690-1766)
Jacques-Barthelemy Micheli du Crest
Jacques-Barthélemy Micheli du Crest (September 28, 1690 – March 29, 1766) was a military engineer, physicist and cartographer, born in Geneva and so a citizen of the then Republic of Geneva, now in Switzerland.According to the Historical Dictionary of Switzerland: Micheli, Jacques-Barthélemy (du Crest) was a citizen of the Republic of Geneva; his family Micheli (du Crest) obtained full citizenship ("bourgeoisie") in 1664.

Википедия

Paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occurs due to deficiency of the red blood cell surface protein DAF, which normally inhibits such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the circulatory system, the red blood cell destruction (hemolysis) is considered an intravascular hemolytic anemia. Other key features of the disease, such as the high incidence of venous blood clot formation, are incompletely understood.

PNH is the only hemolytic anemia caused by an acquired (rather than inherited) intrinsic defect in the cell membrane (deficiency of glycophosphatidylinositol or GPI) leading to the absence of protective exterior surface proteins that normally attach via a GPI anchor. It may develop on its own ("primary PNH") or in the context of other bone marrow disorders such as aplastic anemia ("secondary PNH"). Only a minority of affected people have the telltale red urine in the morning that originally gave the condition its name.

Allogeneic bone marrow transplantation is the only cure, but has significant rates of additional medical problems and death. The monoclonal antibody eculizumab reduces the need for blood transfusions and improves quality of life for those affected by PNH. Eculizumab dramatically alters the natural course of PNH, reducing symptoms and disease complications as well as improving survival to the extent that it may be equivalent to that of the general population. Eculizumab costs at least US$440,000 for a single year of treatment and has been reported as one of the world's most expensive drugs.