Haematological values in steady-state sickle cell anaemia patients and matched heamoglobin AA Controls in a Rural Area of Eastern Gabon

Background. Sickle cell nephropathy (SCN) is a serious complication of sickle cell anaemia (SCA) with asymptomatic onset in childhood and possible progression to chronic kidney disease (CKD). In Southeast Nigeria, few studies have evaluated renal function in paediatric SCA patients for early detection of renal impairment and early intervention to reduce morbidity and mortality. Therefore, this study evaluated the renal function of paediatric SCA patients in a steady state based on glomerular filtration rate and urinalysis findings (proteinuria and haematuria). Methods. A cross-sectional study of consecutively recruited sixty haemoglobin SS (HbSS) children in a steady state and sixty age- and sex-matched haemoglobin AA (HbAA) controls aged 2–18 years was done. Renal function of HbSS subjects was evaluated using estimated glomerular filtration rate (eGFR) which was compared with healthy HbAA subjects. The prevalence of significant proteinuria and haematuria, its association with eGFR, and the effect of past sickle cell crisis (in the preceding 24 months) on renal function were also evaluated. Results. Mean eGFR was significantly higher in HbSS subjects than in the HbAA subjects (p=0.001) and decreased with age. Significant proteinuria and haematuria were more prevalent in the HbSS group (3.4% and 6.7%, respectively) compared to the HbAA subjects (0% and 0%, respectively) (p=0.496 and 0.119, respectively). No significant association was observed between eGFR and proteinuria (p=1.000) or haematuria (p=1.000). There was a positive correlation between eGFR and frequency of past painful crisis that required hospitalization (r=0.138, p=0.295) and between eGFR and frequency of blood transfusion (r=0.679, p≤0.001). Conclusions. Asymptomatic paediatric HbSS (SCA) patients had higher mean eGFR indicating an increased risk of nephropathy. There was no association between eGFR and proteinuria or haematuria. Frequent sickle cell crises especially one requiring transfusion were positively correlated with hyperfiltration.

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ADULT SICKLE CELL ANAEMIA PATIENTS IN BONE PAIN CRISIS HAVE ELEVATED PRO-INFLAMMATORY CYTOKINES

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2018.017 ◽

2018 ◽

Vol 10 (1) ◽

pp. e2018017 ◽

Cited By ~ 4

Author(s):

Adekunle Emmanuel Alagbe ◽

John Ayodele Olaniyi ◽

Oladapo Wale Aworanti

Keyword(s):

Steady State ◽

Sickle Cell ◽

Plasma Levels ◽

Bone Pain ◽

Sickle Cell Anaemia ◽

Cross Sectional Study ◽

Cross Sectional ◽

Tnf Α ◽

Pain Crisis ◽

State Group

Background and Objective: Inflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - α, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients.Methods and Materials: Sixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study. The severity of BPC was assessed clinically, and questionnaires were filled. Plasma levels of TNF- α, IL-8 and ET-1 were quantified by ELISA, and haematological parameters were determined using a 5-part auto-analyzer. Plasma levels were correlated with the severity of bone pain crisis. Results were considered statistically significant if p<0.05.Results: Plasma TNF-α, IL-8, and ET-1 were significantly elevated in the BPC group than in the steady state group and the controls. Plasma TNF-α, IL-8 and ET-1 were markedly higher in the severe BPC groups than the steady state and control groups, There was a positive correlation between TNF-α and ET-1 in the bone pain crisis group.Conclusion: Elevated levels of plasma TNF-α, IL-8, and ET-1 further establish the chronic inflammatory state in SCA and equally affirm their significant contribution, not only to pathogenesis but also to the severity of pain in SCA. Keywords: Sickle cell anaemia, Cytokines, Bone pain crisis, Severity, Steady state.

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Ventricular hypertrophy and ischaemic changes in Children with Sickle Cell Anaemia and its relationship with the Haemoglobin Concentration in Steady State

Medical Science and Discovery ◽

10.36472/msd.v8i1.458 ◽

2021 ◽

Vol 8 (1) ◽

pp. 1-8

Author(s):

Charles Ikegwuonu ◽

Obumneme Ezeanosike ◽

Chuka Manyike ◽

Fortune Ujunwa ◽

Cordis Ikegwuonu

Keyword(s):

Steady State ◽

Sickle Cell ◽

Ventricular Hypertrophy ◽

Sickle Cell Anaemia ◽

Haemoglobin Concentration ◽

Left Ventricular ◽

Cardiac Structure ◽

Cardiac Abnormality ◽

St Segment ◽

The Relationship

Objective: The prevalence and burden of Sickle Cell Anaemia (SCA) in Nigeria are high and they contribute to childhood morbidity and mortality. Chronic anaemia and vaso-occlusion usually involve different organs. The involvement of the heart is a common complication of SCA, thus the need for early detection of cardiac abnormalities in children with SCA. To assess cardiac structure using ECG in children with SCA in steady-state and to determine the relationship between abnormal ECG findings and the participants’ haemoglobin concentration. Material and Method: It was a cross-sectional study done in one of the tertiary hospitals in southeast Nigeria. The study participants were 164 children with SCA in steady state within the ages of 2 -17 years. A 12-lead ECG was carried out on the participants and their haemoglobin concentrations determined. The relationship between the presence of cardiac abnormality and independent variables like haemoglobin concentration, age and gender were analyzed. Result: The prevalence of cardiac abnormality was 59.1%. The commonest cardiac structure abnormality was Left ventricular hypertrophy (LVH) (39.6%). Abnormality in the P-R interval was seen in 14.6%, while ST segment abnormality was seen in 18.3%. The mean Hb of those with abnormal ECG was lower than that of those without abnormal ECG, across each age group. Severe anaemia was a significant (p< 0.01) predictor of LVH. However, there was no significant association between the degree of anaemia and the occurrence of ST-segment (p: 0.26) and PR interval abnormalities (p: 0.52). Conclusion: Cardiac anomalies are common findings in SCA children.

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Assessment of Some Haemostatic Parameters in Sickle Cell Anaemia Subjects Resident in Rivers and Bayelsa States

Journal of Advances in Medicine and Medical Research ◽

10.9734/jammr/2021/v33i1130925 ◽

2021 ◽

pp. 53-64

Author(s):

Barinaaziga S. Mbeera ◽

Susanna O. Akwuebu ◽

A. C. U. Ezimah ◽

Nancy C. Ibeh ◽

Evelyn M. Eze

Keyword(s):

Steady State ◽

Sample Size ◽

Sickle Cell ◽

Teaching Hospital ◽

Sickle Cell Anaemia ◽

Control Group ◽

Port Harcourt ◽

The Mean ◽

The University ◽

D Dimer

Aim: The aim of this study was to assess some haemostatic parameters in sickle cell anaemia subjects in Rivers and Bayelsa States. Study Design: This study is a cross-sectional observational study. Place and Duration of Study: This study was carried out at the University of Port Harcourt Teaching Hospital, Rivers State, and the Federal Medical Centre, Yenagoa, Bayelsa State, between the months of February and August, 2020. Methodology: A total of four hundred and fifty (450) subjects with age range of 1-50 years were randomly selected. There were about 200 registered patients (adults and children alike) at the sickle cell clinics of the University of Port Harcourt Teaching Hospital, and the Federal Medical Centre, Yenagoa, with an average of 4 new patients per month. The sample size was obtained using a prevalence of sickle cell anaemia of 2% and the sample size was calculated using Cochran sample size formula. Five milliliters (5ml) of venous blood sample was withdrawn from the peripheral vein in the upper limb of subjects using a standard venipuncture technique. The sample was rocked gently to mix and kept at room temperature and the haemostatic parameters (vWF, FVIII, D-dimer, L-arginine, fibrinogen, ADAMTS13) were assayed quantitatively with Bioassay Technozym kit using Microplate Reader (Labtech microplate auto ELISA plate reader, an IS0 13485:2003 CE and WHO compliance Co., Ltd. Shanghai International Holding Corp. GmbH; Europe) calibrated to a wavelength of 450 nm with strict adherence to the manufacturer's instructions, while PT and APTT were analysed with Fortress reagent and Uniscope SM801A Laboratory using water bath.Data management and statistical analyses were conducted using Statistical Analyses System SAS 9.4 (SAS Institute, Cary, North Carolina, USA) and p values less than .05were considered statistically significant. Results: The results showed the mean comparison of haemostatic parameters in sickle cell anaemia and control subjects. The comparison of haemostatic parameters showed significant(p<.05) increasesand decreases inVaso-Occlusive Crisis (VOC) and steady state respectively compared with the control group. There was statistically significantreduction in the mean comparison of L-Arginine (p<.01) in VOC) condition than steady state in relation to the control group in our study population, while D-Dimer, ADAMTS13 were also significantly reduced statistically (p<.01) in VOC condition than steady state compared with the control group. However, the mean FVIII inhibitor, Fibrinogen, PT (INR) and APTT were significantly higher (p<.01) in VOC than steady state when compared to controls with normal haemoglobin (HbAA).Correlations of haemostaticparameters by sickle cell anaemia subjects’ condition showed more significant positive correlations in VOC than steady state. Conclusion: This study showed a heightened hypercoagulability in Sickle Cell Snaemia(SCA)subjects, and further pave way for better understanding particularly the diagnostic variables underlying SCA, specific to each subject condition (steady state and VOC). Subjects with SCA, particularly during VOC, undergo significant haemostatic alterations that increase their risk of developing coagulation activation-related complications. Thus, though selected markers of coagulation were significantly different between the subject conditions, they were often significantly higher in the SCA.

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