State Sickle Cell Disease Patients Research Articles

T090 Correlation between NT-proBNP and haemoglobin values in steady state sickle cell disease patients in Nairobi, Kenya

T090 Correlation between NT-proBNP and haemoglobin values in steady state sickle cell disease patients in Nairobi, Kenya

Transcranial Doppler Velocities among Sickle Cell Disease Patients in Steady State

Transcranial Doppler (TCD) screening is an established tool to identify children with sickle cell disease at high risk of stroke. Our objective was to study TCD velocities among sickle cell disease patients while in a steady state. This cross-sectional study included 78 steady state sickle cell disease patients [31 Hb SS (βS/βS) (sickle cell anemia), 47 Hb S/β-thalassemia (HBB: c.20A>T/β-thal)], attending the Pediatric Hematology Clinic at Cairo University Children’s Hospital, Cairo, Egypt. All patients underwent TCD velocity assessment as per the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. In our cohort, TCD velocities were comparable among Hb S/β-thal vs. SS patients. Hemolysis indicators correlated significantly to TCD velocities in Hb S/β-thal patients; positive correlation was found between total bilirubin level and right middle cerebral artery (MCA) and right distal internal carotid artery (dICA) TCD velocities (r = 0.428, p = 0.00, r = 0.360, p = 0.01), respectively as well as between reticulocyte count and right MCA, right dICA and right anterior cerebral artery (ACA) TCD velocities (r = 0.424, p = 0.01), (r = 0.40, p = 0.00), (r = 0.303, p = 0.04), respectively. On the other hand, statistically significant negative correlations were found between hemoglobin (Hb) level and right ACA, right dICA TCD velocities (r = −0.290, p = 0.05), (r = −0.324, p = 0.03). Although Hb F is considered an ameliorating factor for disease severity; hemolysis stands as an indicator of risk for TCD velocity elevation, and in turn, risk for stroke among sickle cell disease patients.

Evaluation of Baseline Hematologic Parameters of Steady State Sickle Cell Disease Patients at The Chantal Biya’s Foundation, Yaounde- Cameroon

Introduction
 Sickle cell disease is a genetic abnormality involving the hemoglobin. Although it is primarily a red blood cell disorder, the white blood cells and platelets are also affected by the mutation. The consequence hemoglobin S causing polymerization of hemoglobin, results in hemolysis and anemia. This study aims to provide baseline hematologic parameters in steady state sickle cell disease patients compared with the reference values of normal healthy subjects used at the Chantal Biya Foundation (CBF), in order to monitor other sicklers in Cameroon.
 Methodology
 A comparative analysis of sickle cell hematologic parameters with control hematologic parameters of normal healthy subjects (reference values of healthy subjects used at the Chantal Biya Foundation) was carried out.
 Results
 A total of 62 sickle cell disease patients in steady state who complied with the selection criteria were recruited. Of the 62, 29 were females and 33 were males. The age range was from 1 year to 19 years and an average age of 6 ± 4.19 SD. Results from sickle cell patients showed an increase in white blood cells (WBCs), neutrophils and lymphocytes and a great decrease in the mean values of hematocrit Hct. as well as RBC indices, but no great or slight difference in the values of basophils compared with the reference values of normal healthy subjects in the CBF Yaoundé, 2015.
 Conclusion
 Sickle cell disease patients in steady state have lower values of red cells parameters, but higher values of white cells and platelets count when compared with the reference values of normal healthy subjects used at the Chantal Biya Foundation.

Evaluation of Tp-e Interval and Tp-e/QTc Ratio among Patients with Steady State Sickle Cell Disease

Sickle cell disease (SCD) has been regarded as an inflammatory and pro- coagulatory disease with profound cardiovascular abnormalities including propensity for ventricular arrhythmogenesis. Tp-e and Tpe/QTc ratio however has been proposed as better indicators of arrythmogenesis and has been shown to be prolonged in many inflammatory conditions and correlate with levels of inflammatory markers. However, correlation between Tpe/QTc ratio and the level of highly sensitive C-reactive protein (hs-CRP) and plasminogen activator inhibitor (PAI) have not been reported in SCD. This study aims at evaluating Tp-e Interval and Tp-e/QTc ratio among steady state Sickle cell disease patients in relationship to the degree of anaemia, inflammatory and profibrotic markers. Methodology: A cross-sectional hospital-based study comprises 30 sickle cell anaemia patients in steady state with an equal number of controls having genotype HbAA and HbAS respectively.Clinical, laboratory and ECG parameters were obtained. Results: A total of 90 participants are with mean age 24.2 ± 5.6. The study showed that sickle cell disease patient had significantly lower level of PCV and higher level of PAI, platelet and total white cell count (p value < 0.05). C-reactive protein was also higher in them. 76.7% of HbSS patients had abnormal ECG. QTc and Tp-e were also prolonged in sickle cell disease patients compared with controls. An association was found between the level of PCV, PAI and prolonged Tp-e and QTc. Conclusion: Sickle cell disease patients have higher levels of inflammatory markers and abnormal ECG patterns are common in them. Moreover, the levels of these inflammatory markers correlate with Tp-e parameters.

Blood Pressure Patterns and Factors Associated with Relative Hypertension among Steady State Sickle Cell Disease Patients in Kinshasa, Democratic Republic of the Congo: A Cross-Sectional Study

Background: Although systemic hypertension is rarely seen in steady state sickle cell disease (SCD), relative hypertension has been reported to be associated with an increased risk of cardiovascular and renal complications. Objective: To determine the prevalence of BP patterns and assess factors associated with relative hypertension insickle cell anemia (SCA) adult patients. Methods: Clinical data and office BP were obtained from 103 consecutive steady-state SCA adult patients (mean age 26 ± 7.9 years, 66% females, 22.3% on hydroxyurea) attending four healthcare centers providing SCD-specific care in Kinshasa. Seated BP was measured using an automated electronic device. Three consecutive blood pressure measurements were taken with 2 minutes interval between readings and the average of the 2 last readings was considered for the analyses. Normal BP, relative hypertension and systemic hypertension were defined as BP < 120/70 mmHg, 120 - 139/70 - 89 mmHg and ≥140/90 mmHg, respectively. Results: Normal BP, relative hypertension and systemic hypertension were observed in 56 (54%), 43(42%) and 4 (4%) of SCA patients, respectively. In multivariate analysis, factors associated with relative hypertension were leg ulcer (aOR 2.05; 95%CI 1.77 - 5.18; p = 0.016), central obesity (aOR 3.32; 95%CI 1.28 - 6.24; p = 0.001), smoking (aOR 5.02; 95%CI 1.51 - 9.50; p = 0.017), and microalbumiuria (aOR 3.44; 95%CI 1.44 - 5.76; p = 0.035). Conclusion: Relative hypertension was a common finding in the present case series and associated with traditional cardiovascular risk factor as well as factors specific to SCD highlighting the need for measures to prevent its progression towards systemic hypertension and associated cardiovascular and renal disease.

LC-MS/MS analysis of plasma polyunsaturated fatty acids in patients with homozygous sickle cell disease.

The aim of this study was to determine circulating omega-6, omega-3 polyunsaturated fatty acids and prostaglandin E2 (PGE2) levels in steady state sickle cell disease (SCD) patients. Blood was collected from healthy hemoglobin volunteers and steady state homozygous HbSS patients who had not received blood transfusions in the last 3months. Plasma levels of arachidonic acid (AA, C20:4n-6), dihomo-gamma-linolenic acid (DGLA, C20:3n-6), eicosapentaenoic acid (EPA, C20:5n-3) and docosahexaenoic acid (DHA, C22:6n-3) were determined by an optimized multiple reaction monitoring method using ultrafast liquid chromatography coupled with tandem mass spectrometry. PGE2 was measured in serum samples by enzyme immunoassay. Plasma AA and DGLA were significantly increased while EPA and DHA were significantly decreased in SCD plasma compared to control. Serum PGE2 levels, AA/DHA and AA/EPA ratio was significantly higher in SCD patients when compared to control group. The significant increase in PGE2 levels, AA/EPA and AA/DHA ratio confirms the presence of a proinflammatory state in SCD patients.

A study of β-thromboglobulin and platelet factor-4 plasma levels in steady state sickle cell patients

To evaluate the platelet function in sickle cell syndromes we measured the beta-thromboglobulin (beta-TG) and platelet factor 4 (PF-4) plasma values of 45 patients suffering from homozygous sickle cell anaemia (10) and sickle cell beta-thalassaemia (35) in steady state. The results were compared to those of 32 normal controls. Both the beta-TG and PF-4 levels were found to be significantly higher in patients than in controls but the beta-TG:PF-4 ratio was significantly lower in the patients group. This finding and the absence of any statistical correlation between platelet number and beta-TG or PF-4 indicate that platelets seem to be somehow activated in sickle cell syndromes, both in homozygotes and sickle cell/beta-thalassaemia heterozygotes. This platelet activation seems to exist even in steady state sickle cell disease patients, regardless of the functional status of the spleen.