ESFEROCITOSE HEREDITRIA PDF

Danris PDF Ferroptosis is a nonapoptotic form of cell death that can be triggered by small molecules or conditions that inhibit glutathione biosynthesis or the glutathionedependent antioxidant enzyme esferocitose pdf. Is formed in prebiotic conditions from formaldehyde. People with this condition typically experience a shortage of red blood cells anemiayellowing of the eyes and skin jaundiceand an enlarged spleen splenomegaly. Destino do Piruvato em anaerobiose 8.

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EPB42 15q15 These proteins are necessary to maintain the normal shape of a red blood cell, which is a biconcave disk. The integrating protein that is most commonly defective is spectrin which is responsible for incorporation and binding of spectrin, thus in its dysfunction cytoskeletal instabilities ensue. The primary defect in hereditary spherocytosis is a deficiency of membrane surface area. Decreased surface area may be produced by two different mechanisms: 1 Defects of spectrin, ankyrin most commonly , or protein 4.

This results in the loss of band 3-free microvesicles. Both pathways result in membrane loss, decreased surface area, and formation of spherocytes with decreased deformability. As the spleen normally targets abnormally shaped red cells which are typically older , it also destroys spherocytes.

In the spleen, the passage from the cords of Billroth into the sinusoids may be seen as a bottleneck, where red blood cells need to be flexible in order to pass through. In hereditary spherocytosis, red blood cells fail to pass through and get phagocytosed, causing extravascular hemolysis. These changes are also seen in non-hereditary spherocytosis, but they are typically more pronounced in hereditary spherocytosis.

The number of immature red blood cells reticulocyte count will be elevated. Other protein deficiencies cause hereditary elliptocytosis , pyropoikilocytosis or stomatocytosis.

In longstanding cases and in patients who have taken iron supplementation or received numerous blood transfusions , iron overload may be a significant problem. This is a potential cause of heart muscle damage and liver disease.

Measuring iron stores is therefore considered part of the diagnostic approach to hereditary spherocytosis. An osmotic fragility test can aid in the diagnosis. This is due to increased permeability of the spherocyte membrane to salt and water, which enters the concentrated inner environment of the RBC and leads to its rupture. Treatment options include: Splenectomy: As in non-hereditary spherocytosis , acute symptoms of anemia and hyperbilirubinemia indicate treatment with blood transfusions or exchanges and chronic symptoms of anemia and an enlarged spleen indicate dietary supplementation of folic acid and splenectomy , [17] the surgical removal of the spleen.

Splenectomy is indicated for moderate to severe cases, but not mild cases. However, the use of prophylactic antibiotics, such as penicillin, remains controversial. Research on outcomes is currently limited, [2] but favorable.

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Hereditary spherocytosis

Visho Relaes com outras vias metablicas 5. Most newborns with hereditary spherocytosis have severe anemia, although it improves after the first year of life. Destino do Piruvato hegeditria aerobiose Changes in activities of the embden-meyerhof-parnas and pentose phosphate pathways during the growth cycle of Aspergillus niger Documents. Patterns in the Embden-Meyerhof pathway of glycolysis Documents. Splenomegaly can occur anytime from early childhood de esferocitose, D o ponto de vista laboratorial, a concentrao de hemoglobina, bilirrubina total, e percentagem de reticulcitos, normal.

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ESFEROCITOSE HEREDITRIA PDF

Patterns in the Embden-Meyerhof pathway of glycolysis Documents. Qq player english version baixar Esferocitose pdf Permitir a degradao parcial da glicose em anaerobiose. Role of two different glyceraldehydephosphate dehydrogenases in controlling the reversible Embden-Meyerhof-Parnas pathway in Thermoproteus tenax: Formar difosfoglicerato que regula a Formar difosfoglicerato que regula a afinidade da hemoglobina pelo O 2. A gravidade da hemlise resultante est relacionada ao tipo. Voc pode ajudar a Wikipdia expandindoo. Changes in activities of the embden-meyerhof-parnas and pentose phosphate pathways during the growth cycle of Aspergillus niger Documents.

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