Thesis (Ph.D.) - Cairo University - Faculty of Medicine - Department of Pediatrics

Background: A Chronic pro-inflammatory state exists in sickle cell disease (SCD) patients, even in the clinically asymptomatic phase. In SCD patients, the relation between the chronic pro-inflammatory state and oxidative stress is bidirectional. Aim of work: Assessment of the oxidant {u2013}anti oxidant status in SCD patients in its relation to transcranial Doppler (TCD) velocities and estimated pulmonary artery pressure (ESPAP). Patients and Methods: This case-control study included 78 SCD patients (31 SS, 47 SB) in steady state as well as 40 age and sex matched healthy controls. Serum nitrite, malondialdhyde (MDA), paraoxinase (PON), catalase and total antioxidant capacity (TOA) were estimated calorimetrically.TCD was done via transtemporal approach according to STOP trial and pulmonary hypertension was assessed as an estimate from tricuspid regurge (TR) jet velocity using the modified Bernoulli equation. Results: Serum nitrite and serum MDA were significantly higher in cases than controls (P {u02C2}0.01, {u02C2}0.01, respectively) while serum catalase and serum PON and TOA were significantly lower in cases than controls (P=0.03, {u02C2}0.01, {u02C2}0.01, respectively). Serum nitrite showed significant negative correlation with TOA and serum catalase (P {u02C2}0.01, 0.03 respectively). Serum PON showed significant negative correlation with age of patient, age at 1st dose of hydroxyurea (HU) among all patients (P{u02C2}0.05,{u02C2}0.05 respectively).TOA significantly correlated to TCD velocities in right dICA among all patients and SB patients(P{u02C2}0.05, {u02C2}0.01 respectively). Among SS group, serum nitrite significantly correlated to Hb F (P{u02C2}0.05) while serum catalase showed statistically significant negative correlations with Hb F (P {u02C2}0.05). Among SB group, serum PON showed significant negative correlation with duration of chelation (P{u02C2}0.05) and significantly correlated to Hb F (P{u02C2}0.05). No correlation was found between all measured oxidants-antioxidants and ESPAP among our patients

Issued also as CD