Cardiovascular changes in children with sickle cell crisis

2020 ◽  
Vol 30 (2) ◽  
pp. 162-170
Author(s):  
Richard Onalo ◽  
Peter Cooper ◽  
Antoinette Cilliers ◽  
Uche Nnebe-Agumadu
Keyword(s):  
Pulmonary Hypertension ◽  
Steady State ◽  
Sickle Cell ◽  
Myocardial Ischaemia ◽  
Sickle Cell Anaemia ◽  
Cardiac Biomarkers ◽  
Systemic Hypertension ◽  
Cardiovascular Changes ◽  
Sickle Cell Crisis ◽  
The Mean

AbstractBackground:Sickle cell anaemia is characterised by frequent, sometimes serious events referred to as “crisis”. Cardiopulmonary consequences such as pulmonary hypertension and myocardial ischaemia may accompany a serious crisis.Objective:To determine the cardiovascular changes that occur during a severe sickle cell crisis.Methods:A cross-sectional comparative study of sickle cell anaemia in children (5–17 years) admitted during a severe crisis (cases) and those in steady state (controls) was conducted over a 2-year period. Effects of the crisis on the cardiopulmonary system were assessed. The diagnosis of myocardial ischaemia was made using electrocardiography and serological cardiac biomarkers, while cardiac dysfunction and the presence of pulmonary hypertension were determined using echocardiography. The presence of systemic hypertension and tachycardia was also evaluated.Results:A total of 176 patients were recruited, 92 in steady state (male:female ratio, 1.2:1) and 84 in severe crisis (male:female ratio, 1.3:1). The mean age was 10.4 ± 3.2 years for steady state and 10.5 ± 3.4 years for those in crisis. The mean heart rate in crisis was higher than in steady state (p < 0.0001). The blood pressures (systolic, p < 0.0001, diastolic, p < 0.0001, mean, p < 0.0001) as well as myocardial ischaemia scores (p < 0.0001) were higher in patients with crisis than in those in steady state. Similarly, conduction abnormalities, pulmonary hypertension, and ventricular dysfunction were more prevalent in the crisis than in the steady state.Conclusion:The present data suggest that sickle cell crisis results in a derangement of clinical, electrocardiographical, and echocardiographical parameters in children with sickle cell anaemia. Further research on these cardiovascular events may improve the overall care of these patients.

Sickle cell crisis as evaluated from measurements of hydroxybutyrate dehydrogenase and myoglobin in plasma.

1981 ◽  
Vol 27 (2) ◽  
pp. 314-316 ◽  
Author(s):  
E F Roth ◽  
P A Bardfeld ◽  
S J Goldsmith ◽  
E Radel ◽  
J C Williams
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
High Specificity ◽  
Mean Value ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
The Mean ◽  
Low Sensitivity ◽  
Sickle Cell Crises

Abstract Data on plasma hydroxybutyrate dehydrogenase activity (I) and myoglobin concentration were used to evaluate painful sickle cell crises. I was increased during non-crisis steady state in patients with sickle cell disease as compared to normal values (232, SD 79.7 vs 85, SD 33 Sigma units/mL). During crisis, the mean value for I increased further to 379 (SD 139) Sigma units/mL. For 12 patients evaluated both during steady state and crisis, there was a mean increase in plasma I of 131% (SD 76%). Repeated determinations of I in sickle cell disease patients during several months while they were in steady state showed that baseline I varied by no more than 20% from the mean. Plasma myoglobin in patients with sickle cell disease was not above normal, but during crisis 21 of 39 patients tested had increased plasma myoglobin concentrations. Our data suggest that I may be a useful indicator of sickle cell crisis when the patient's own baseline value is available for comparison. Plasma myoglobin measurements give evidence of muscle damage during crisis with high specificity but low sensitivity.

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

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.

scholarly journals Evaluation of Vitamin D in Sickle Cell Anaemia Patients at Steady State

2020 ◽  
Vol 1 (2) ◽  
Author(s):  
Umar Aminu Abdullah ◽  
Mohammed Bashir AbdulRahman ◽  
Bello Aminu ◽  
Abubakar Umar Musa ◽  
Isah Balarabe
Keyword(s):  
Vitamin D ◽  
Steady State ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Public Health Problem ◽  
Bone Fragility ◽  
Sub Saharan Africa ◽  
Major Public Health Problem ◽  
The Mean ◽  
Sub Saharan

Background: Sickle cell anaemia is a major public health problem in sub-Saharan Africa with high morbidities like bony alterations and bone fragility especially in those with vitamin D deficiency. There is paucity of research data on bone biomarkers in patients with sickle cell anaemia especially in Northern Nigeria. The aim of this study was to evaluate vitamin D in adult sickle cell anaemia patients at steady state. Materials and Methods: Seventy-seven patients with sickle cell anaemia and equal number of apparently healthy matched controls were recruited for the study. Various biochemical parameters of bone metabolism were measured. Data were analysed using IBM SPSS version 23.0. Results: The mean age of SCA adult patients and controls wer

scholarly journals Inflammatory mediators in sickle cell anaemia highlight the difference between steady state and crisis in paediatric patients

2017 ◽  
Vol 182 (6) ◽  
pp. 933-936 ◽  
Author(s):  
Magda O. S. Carvalho ◽  
Théo Araujo-Santos ◽  
João H. O. Reis ◽  
Larissa C. Rocha ◽  
Bruno A. V. Cerqueira ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell ◽  
Inflammatory Mediators ◽  
Sickle Cell Anaemia ◽  
Paediatric Patients ◽  
The Difference

scholarly journals Neck circumference is independently associated with relative systemic hypertension in young adults with sickle cell anaemia

2018 ◽  
Vol 24 (1) ◽  
Author(s):  
Lawrence A. Olatunji ◽  
Olatunde P. Olabode ◽  
Olawale M. Akinlade ◽  
Abiola S. Babatunde ◽  
Victoria A. Olatunji ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Neck Circumference ◽  
Systemic Hypertension

Liver function test profile of Nigerian children with sickle cell anaemia in steady state

2014 ◽  
Vol 11 (1) ◽  
pp. 13
Author(s):  
ShehuA Akuyam ◽  
PeterO Anaja ◽  
Yusuf Garba ◽  
Nasir Lawal ◽  
Abdullahi Musa ◽  
...  
Keyword(s):  
Steady State ◽  
Liver Function ◽  
Liver Function Test ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Nigerian Children ◽  
Function Test

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

2019 ◽  
Vol 26 (1) ◽  
pp. 13
Author(s):  
LandryErik Mombo ◽  
Gaël Mabioko-Mbembo ◽  
Cyrille Bisseye ◽  
Kevin Mbacky ◽  
Fatoumata Thiam ◽  
...  
Keyword(s):  
Steady State ◽  
Rural Area ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Haematological Values

scholarly journals Sickle Cell Nephropathy and Associated Factors among Asymptomatic Children with Sickle Cell Anaemia

2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Uchenna Modestus Nnaji ◽  
Christian Chukwukere Ogoke ◽  
Henrietta Uche Okafor ◽  
Kingsley I. Achigbu
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Steady State ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Filtration Rate ◽  
Sickle Cell Anaemia ◽  
Asymptomatic Children ◽  
Increased Risk ◽  
Sickle Cell Nephropathy

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.

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.

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