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Endothelial dysfunction profile in patients of sickle cell anemia

Authors:Ekta Khandelwal , Sumeet Tripathi, Pritam Wasnik
Int J Biol Med Res. 2020; 11(4): 7132-7134  |  PDF File


Introduction: Sickle cell disease (SCD) is a haemoglobin disorder characterized by mutation of valine for glutamic acid at 6th position of the beta globin chain of hemoglobin. Sickle hemoglobin (HbS) causes a distorted sickle shape of RBCs which are prone to haemolysis and vaso-occlusion of sickled red cells. Vaso-occlusive crises (VOC) leads to acute pain syndrome and multi-organ dysfunction in patients of SCD which is one of the major cause of morbidity. This study is to establish the role of endothelial dysfunction (EF) in pathophysiology of vaso-occlusive crises. Aim & objectives: To assess & quantify Endothelial Function by reactive hyperemia in adult patients of sickle cell anemia & compare the vascular reactivity by using parameters of PPG in healthy subjects & SCD patients. Methods: 15 young adult patients of age group 18 to 40 yrs diagnosed with SCD 15 healthy age-matched controls were tested for endothelial function by reactive hyperemia. PPG parameters like systolic peak amplitude, upstroke slope, maximum systolic peak to peak interval (PPI), time difference between the successive systolic peak instances of PPG. In addition, the beat-to-beat changes of PTT were computed. After determining the distribution of data appropriate statistical analysis were done by using SPSS 20 software. Result & Conclusion: There was a significant (p> 0.005) change in PPG parameters like Amplitude, Upstroke Slope, PPI & PTT in the control group as compared to SCD patients before and after RH. Amplitude & upstroke slope suggestive of an effective metabolic response in controls than in patients group. Pulse transit time (PTT) suggestive of intact endothelial function due to release of Endothelial NOS in controls than in patients group.