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Pathophysiology Of Sickle Cell Disease

Pathophysiology of sickle cell disease has to do it with its adverse effects and protean or variable complications, which has led to recent advances that are targeted towards molecular therapies for sickle cell disease patients. 

In sickle cell disease, a single amino acid substitution in the beta-globin β-globin chain leads to the polymerization of the mutant hemoglobin S that leads to the impairment of the erythrocyte (red blood cells) rheology and survival. Medically, as recorded when seen, erythrocyte abnormalities of sickle cell disease manifest as hemolytic anemia and several cycles of microvascular vaso occlusion that result in end-organ ischemia-reperfusion injury and infarction. 

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Photo Credits: ResearchGate

Hemoglobin S which is the sickle cell gene undergoes polymerization. The intraerythrocytic hemoglobin S deoxygenation in tissues with high oxygen demand promotes the exposure of hydrophobic (water-hating) motifs on the individual deoxygenated hemoglobin tetramers (four states). The globin chains on the beta S globin on different deoxygenated hemoglobin tetramers bind one to the other to hide the exposed hydrophobic motifs; this then initiates the production of nucleation of a hemoglobin S polymer. 

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Photo Credits: Frontiers

These polymers elongate or grow rapidly to form long fibers that would increase cellular rigidity and also cause a distortion of the erythrocyte (red blood cells) membrane, leading to erythrocyte (red blood cells) sickling, cellular energetic failure and stress, dehydration, impaired rheology, and premature hemolysis. 


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Photo Credits: McMaster

Vaso occlusion or blood vessel occlusion that leads to ischemia is the predominant pathophysiology of sickle cell disease that is responsible for acute systemic painful vaso occlusive crisis VOC endothelial dysfunction and sterile inflammation are also known hallmarks of the manifestation of sickle cell disease. 

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Photo Credits: PBworks

Sickle cell disease patients are known to have an increased range level of neutrophils, monocytes, and platelets at baseline, and an elevated level of circulating neutrophil platelet and monocyte platelet which aggregates in sickle cell disease patient blood and correlates with disease severity. Thrombocytopenia is a progression of VOC.

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Photo Credits: ResearchGate

Content created and supplied by: Dr-Kikiope (via Opera News )

Hemoglobin S Sickle Cell beta-globin

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