الفهرس 
Evaluation of Cardiac Functions and Lipid Profile in Patients with Sickle Cell Anemia
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Abstract
Introduction: Sickle cell disease is a point mutation in the B-globin gene leading to the synthesis of abnormal hemoglobin S (HbS). This type of hemoglobinopathy may be associated with lipid dysfunction and cardiac dysfunction which need close follow up and monitoring.
Aim of work: to assess the blood lipid profiles in SCD patients and its association with disease related complications especially cardiac affection and other disease related complications.
Patients and methods: This cross-sectional study included 50 SCA patients following up at the Hematology Outpatient clinic, Cairo University Children Hospital. This specialized clinic works 3 days/ week. Thirty healthy subjects who were age and sex matched were enrolled as a control group. Lipid profile (measured by TC, LDL, HDL, TG) and cardiac functions (measured by M- mode which indicates late stages of cardiac affection when abnormal and tissue Doppler which is advanced study to reveal subtle dysfunction at early stages) were assessed for these groups. This study was conducted over a duration of 1 year from August 2020 till July 2021
Results: There was statistically significant lower Hb (mg/dl), BUN (mg/dl), creatinine (mg/dl), HDL (mg/dl), in SCD patients compared to control group with p-value <0.001. There was statistically significant higher LDL (mg/dl), AST (u/L), ALT (u/l), TSB (mg/dl), DSB (mg/dl), ferritin (ng/ml) and LDH (IU/L) in SCD patients compared to control with p-value <0.001. There was statistically significant higher LVlDd, LVlDs (dilated ventricles), LA diameter (diastolic dysfunction), TAPSE (due to stretch of RV), right side Eˋ/cm/s, septal wall E’/cm/s, and left side wall E’/cm/s in SCD compared to control group with p-value <0.001. There was statistically significant lower LVPwd, EF/%, FS/%, Ao diameter/mm, right side A’/cm/s, septal wall S’/cm/s (Right systolic dysfunction), septal wall A’/cm/s, left side wall A’/cm/s, and left side wall S’/cm/s(Left systolic dysfuntion), in SCD compared to control group with p-value <0.001. Tissue Doppler of the included subjects shows statistically significant higher MV E/cm/s, and MV E/A in SCD compared to control group with p-value <0.001. There was statistically significant difference between SCD patients with dyslipidemia and SCD patients without dyslipidemia as regards family pedigree of SCD with p-value =0.012 being most of SCD patients who had history of SCD in 2nd and 3rd pedigree family were more susceptible to dyslipidemia. There was statistically significant lower Ao diameter/mm, LVIDd, right side Eˋ, lower right side A’, lower septal S’, lower septal Eˋ, left S̍, left A̍ in SCD patients with cardiac dysfunction compared to SCD patients with no cardiac dysfunction with p-value =0.003, 0.015, 0.029, <0.001, 0.004, 0.035, 0.041 respectively. There was statistically significant higher septal E/Eˋ in SCD patients with cardiac dysfunction compared to SCD patients with no cardiac dysfunction with p-value =0.021, in echocardiography. In tissue Doppler there was statistically significant higher HR/B in SCD patients with cardiac dysfunction compared to SCD patients with no cardiac dysfunction with p-value =0.047. HDL had a statistically significant predictor of cardiac dysfunction in sickle cell patients with 74.19% sensitivity, 63.16% specificity, and p-value =0.023.
Conclusion: Dyslipidemia (especially elevated LDL, TG and low HDL) is an important complication associated with sickle cell disease which can have great impact on cardiac function of the affected patients. Family pedigree of SCD was the only significant predictor of dyslipidemia. Echocardiographic parameters and tissue Doppler can be helpful in determination
of cardiac dysfunction associated with sickle cell disease