A cross-sectional clinic-based study exploring whether variants within the glutathione S-transferase, haptoglobin and uridine 5′-diphospho-glucuronosyltransferase 1A1 genes are associated with interindividual phenotypic variation in sickle cell anaemia in

2017 ◽  
Vol 100 (2) ◽  
pp. 147-153 ◽  
Author(s):  
Sharon Howell ◽  
Kwesi Marshall ◽  
Marvin Reid ◽  
Norma McFarlane-Anderson ◽  
Colin McKenzie
Keyword(s):  
Sickle Cell ◽  
Phenotypic Variation ◽  
Sickle Cell Anaemia ◽  
Glutathione S Transferase ◽  
Cross Sectional

scholarly journals Relationship between disease severity and folate status of children with sickle cell anaemia in Enugu, South East Nigeria

2021 ◽  
Vol 21 (2) ◽  
pp. 759-764
Author(s):  
Uchenna C Nnajekwu ◽  
Chukwubike O Nnajekwu ◽  
Vivian O Onukwuli ◽  
Ndubuisi A Uwaezuoke ◽  
Osita U Ezenwosu ◽  
...  
Keyword(s):  
Disease Severity ◽  
Sickle Cell ◽  
Severity Score ◽  
Sickle Cell Anaemia ◽  
Life Time ◽  
Folate Deficiency ◽  
Relevant Information ◽  
Folate Supplementation ◽  
Cross Sectional ◽  
Folate Status

Background: Repeated crises in children with sickle cell anaemia (SCA), which is a manifestation of disease severity, results in depletion of their minimal tissue folate stores, with higher likelihood of folate deficiency. The study aimed to determine the relationship between disease severity and the folate status of children with SCA attending University of Nigeria Teaching Hospital (UNTH), Enugu. Methods: This was a hospital based, cross-sectional study conducted between September 2018 and March 2019. One hundred participants were recruited, consisting of 50 children having sickle cell crisis and 50 age and gender matched hae- moglobin AA genotype controls. Relevant information was documented using a pretested questionnaire. Sickle cell severity score was determined using frequency of crisis, admissions and transfusions in the preceding one year, degree of liver and splenic enlargement, life-time cummulative frequency of specific complications of SCA, leucocyte count and haematocrit. Results: Folate deficiency was observed in eight percent of the subjects and none of the controls. The difference was not significant (Fisher’s exact = 4.167, p=0.117). The odds of being folate deficient was 8.5 times more likely during anaemic crisis than in vaso-occlusive crisis, though not significant (95% C.I 0.05 – 89.750, p = 0.075). The mean SCA severity score was 8.06 ± 3.64, signifying a moderate SCA severity in the study population. There was a no relationship between folate status and severity of SCA (Fisher’s exact = 0.054, p = 0.949) Conclusion: Folate status in children with SCA is not affected by their disease severity. Therefore, there may be no need for additional folate supplementation with increasing severity of sickle cell anaemia. Keywords: Sickle cell anaemia; disease severity; folate status; children; Enugu.

scholarly journals Microalbuminuria risks and glomerular filtration in children with sickle cell anaemia in Nigeria

2019 ◽  
Vol 45 (1) ◽  
Author(s):  
I. E. Ocheke ◽  
S. Mohamed ◽  
E. S. Okpe ◽  
F. Bode-Thomas ◽  
M. I. McCullouch
Keyword(s):  
Risk Factors ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Filtration Rate ◽  
Sickle Cell Anaemia ◽  
Estimated Glomerular Filtration Rate ◽  
Kidney Damage ◽  
Strong Positive Correlation ◽  
Cross Sectional

Abstract Introduction Evidence of kidney damage is observed in children with sickle cell anaemia (SCA) and this continues through adulthood with progression to severe functional impairment in some. One of the earliest features of kidney damage associated with SCA is microalbuminuria. Our objective was to determine the risk factors of microalbuminuria in these children and its relationship with estimated glomerular filtration rate. Methods This was a cross-sectional and comparative study involving three hundred and twenty three children with SCA in steady state and equal numbers of apparently healthy age and sex matched haemoglobin AA (HbAA) control, aged 6 months to 18 years. They were consecutively recruited over a 6 month period. Result Microalbuminuria was present in 26% of the study subjects compared with 1.85% of control P = 0.001). Anaemia and high estimated glomerular filtration rate (eGFR) showed strong positive correlation with microalbuminuria (OR = 3.19, CI 0.953–1.116, p = 0.003 and OR = 1.7, CI 1.042–1.066, p = 0.001 respectively). Similarly, eGFR was higher in subjects with SCA than in controls and as well as in those with microalbuminuria compared with those who do not (p = < 0.01). Conclusions The two most important risk factors for microalbuminuria were anaemia and high eGFR. Age category was associated more with microalbuminuria than just age as a variable. Glomerular filtration rate was higher in children with microalbuminuria than those who do not and it was also higher in children with SCA than in control.

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

2018 ◽  
Vol 10 (1) ◽  
pp. e2018017 ◽  
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.

scholarly journals The Iron Status of Sickle Cell Anaemia Patients in Ilorin, North Central Nigeria

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Musa A. Sani ◽  
James O. Adewuyi ◽  
Abiola S. Babatunde ◽  
Hannah O. Olawumi ◽  
Rasaki O. Shittu
Keyword(s):  
Sickle Cell ◽  
Serum Ferritin ◽  
Transferrin Receptor ◽  
Sickle Cell Anaemia ◽  
Iron Status ◽  
Serum Transferrin ◽  
Cross Sectional ◽  
Serum Transferrin Receptor ◽  
Iron Deficient ◽  
The Mean

Objectives. Sickle cell anaemia (SCA) is one of the commonest genetic disorders in the world. It is characterized by anaemia, periodic attacks of thrombotic pain, and chronic systemic organ damage. Recent studies have suggested that individuals with SCA especially from developing countries are more likely to be iron deficient rather than have iron overload. The study aims to determine the iron status of SCA patients in Ilorin, Nigeria.Methods. A cross-sectional study of 45 SCA patients in steady state and 45 non-SCA controls was undertaken. FBC, blood film, sFC, sTfR, and sTfR/log sFC index were done on all subjects.Results. The mean patients’ serum ferritin (589.33 ± 427.61 ng/mL) was significantly higher than the mean serum ferritin of the controls (184.53 ± 119.74 ng/mL). The mean serum transferrin receptor of the patients (4.24 ± 0.17 μg/mL) was higher than that of the controls (3.96 ± 0.17 μg/mL) (p=0.290). The mean serum transferrin receptor (sTfR)/log serum ferritin index of the patients (1.65 ± 0.27 μg/mL) was significantly lower than that of the control (1.82 ± 0.18 μg/mL) (p=0.031).Conclusion. Iron deficiency is uncommon in SCA patients and periodic monitoring of the haematological, biochemical, and clinical features for iron status in SCA patients is advised.

scholarly journals Arterial Blood Pressure in Children with Sickle Cell Anaemia and Controls: A Cross Sectional Study

2020 ◽  
Vol 17 (2) ◽  
pp. 1-9
Author(s):  
Abubakar Garba Farouk ◽  
Halima Umar Ibrahim ◽  
Bello Abdullahi Ibrahim ◽  
Hassan Abdullahi Elechi ◽  
Jamila Audu Idrisa ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Arterial Blood Pressure ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional ◽  
Arterial Blood

Predicted Reference Equations for Spirometry Indices as a Proxy from Anthropometry Measurements of Nigerian Children with Sickle Cell Anaemia Aged 5–12 Years

2020 ◽  
Author(s):  
Olufemi S Akodui ◽  
Abiodun O Faleti ◽  
Folashade A Adekanmbi ◽  
Tinuade A Ogunlesi
Keyword(s):  
Sickle Cell ◽  
Standard Error ◽  
Sickle Cell Anaemia ◽  
Prediction Models ◽  
Coefficient Of Determination ◽  
Linear Regression Models ◽  
Cross Sectional ◽  
Nigerian Children ◽  
Arm Span ◽  
Reference Equations

Abstract Background Significant morbidity and mortality in patients with sickle cell disease are accounted for by lung complications. To facilitate good respiratory care for children with sickle cell anaemia the generation of local predicted values is highly important. Objective To determine the reference equations for spirometry indices estimation in children with sickle cell anaemia, which can be readily used as proxy when there is no easy accessibility to spirometer. Methods A cross-sectional study with linear regression models developed to estimate reference values for spirometric indices in Nigerian children with sickle cell anaemia aged 5–12 years. Results Age as independent variables for estimation of forced expiratory volume in 1 s (FEV1) was associated with lowest coefficient of determination (R2) and highest standard error. The coefficient of determination (R2) and standard error was highest and lowest, respectively, when arm span was used to determine peak expiratory flow rate (PEFR). Prediction models for PEFR and FEV1 gave the closest mean estimates that were 0.18 and 0.16 lower and higher than the actual mean PEFR and FEV1, respectively, but the differences was significant only in FEV1. On the contrary the prediction models for forced vital capacity (FVC) gave mean estimates that was 1.02 higher than the actual mean FVC, however, the finding was not significant. Conclusion Preferred proxy for spirometry indices in children with sickle cell anaemia may be arm span.

scholarly journals Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia

2019 ◽  
Vol 113 (8) ◽  
pp. 489-496
Author(s):  
Igoche D Peter ◽  
Mustafa O Asani ◽  
Shehu U Abdullahi ◽  
Ibrahim Aliyu ◽  
Stephen K Obaro ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Sickle Cell ◽  
Right Ventricular Function ◽  
Sickle Cell Anaemia ◽  
Clinical Laboratory ◽  
P Value ◽  
Cross Sectional ◽  
Mean Pulmonary Arterial Pressure

Abstract Background Pulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA. Methods We performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3–14 y of age in their steady state with matched haemoglobin AA phenotype controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant. Results Of the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls. The median TAPSE was significantly lower in the PH group (2.55 cm [interquartile range {IQR} 2.2–2.8]) compared with the no PH group (2.77 cm [IQR 2.4–3.2]) (p=0.03). No significant correlation existed between mPAP and age, nor any laboratory parameters studied. The odds ratio (OR) suggested PH significantly increased with an increase in the frequency of hospitalizations for vaso-occlusive crises within a 12-month period (OR 15.15 [95% CI 1.57 to 146.35], p=0.02) and a lifetime history of blood transfusion (OR 5.44 [95% CI 1.09 to 27.24], p=0.04). Conclusions Echocardiography-suggested PH is common in children with SCA and is associated with poorer right ventricular function, frequent vaso-occlusive crises and blood transfusions.

Higher prevalence of wheezing and lower FEV1 and FVC percent predicted in adults with sickle cell anaemia: A cross-sectional study

Respirology ◽  
2016 ◽  
Vol 22 (2) ◽  
pp. 284-288 ◽  
Author(s):  
Baba M. Musa ◽  
Najibah A. Galadanci ◽  
Mark Rodeghier ◽  
Michael R. Debaun
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional

scholarly journals Effect of Age on Blood Rheology in Sickle Cell Anaemia and Sickle Cell Haemoglobin C Disease: A Cross-Sectional Study

PLoS ONE ◽  
2016 ◽  
Vol 11 (6) ◽  
pp. e0158182 ◽  
Author(s):  
Céline Renoux ◽  
Marc Romana ◽  
Philippe Joly ◽  
Séverine Ferdinand ◽  
Camille Faes ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Blood Rheology ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional ◽  
Effect Of Age ◽  
Haemoglobin C
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