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Essay / hereditary spherocytosis - 1062
Erythrocyte membrane disorders consist of HS where it is identified by the diversity in clinic and laboratory which is also revealed by recent molecular studies. A mutation is found in one of the spherocytosis genes causing erythrocyte membrane abnormalities. The laparoscopic approach has been one of the new surgical splenectomy procedures for the treatment of HS. Partial splenectomy is performed in children to avoid post-splenectomy sepsis. The final management helps in understanding the splenectomy protocol and suggests careful discussion between the patient, family, and healthcare provider. Hereditary spherocytosis (HS) or Minkowski-Chauffard syndrome is a genetic familial hemolytic disease that causes defects in the internal cytoskeleton of the erythrocyte membrane leading to anemia. The cells have a spherical shape instead of being bi-concave lacking flexibility; they therefore become more sensitive to hemolysis because they cannot pass through the vessels without changing shape. According to the genetic defect, the genetic defect is caused by the heterogeneous modification of one of six genes, which codes for the protein involved in the vertical associations that link the cell membrane skeleton to the lipid bilayer. Skeletal abnormalities of the erythrocyte membrane are responsible for various hereditary hemolytic anemias associated with the abnormal shape of erythrocytes. Hemoglobin is released by hemolysis. There are more reticulocytes present in the circulation and the bone marrow tries to produce more red blood cells than usual in order to prevent anemia. Hereditary spherocytosis (HS) occurs worldwide, but is more common in northern European descendants. It affects approximately 1 in 1,000 to 2,500 individuals according to the diagnostic principle that...... middle of paper ......full and preserving enough tissue for this would help it perform the functions desired hematological and immune systems. Partial splenectomy reduces the symptoms of hypersplenism and splenic sequestration in children with hemoglobinopathies, while splenic function is less well preserved than in children with spherocytosis. This can be corroborated by early studies where surgery performed in 11 patients showed that hemoglobin values increased while reticulocyte values decreased. This results in a direct increase in erythrocyte count lifespan after partial splenectomy. ] summarized his experience in Europe by explaining that irregular rates of splenic regrowth are not associated with recurrent hemolysis and that the reasons behind this are unclear and could be due to parenchymal remodeling after partial resection..