scholarly journals Psychological Study of the Persons Suffering From Sickle Cell Disease in Raigarh District of Chhattisgarh State

2017 ◽  
Vol 4 (2) ◽  
Author(s):  
Dr. Rajesh Kumar Ajagallay ◽  
Dr. Gaukaran Janghel ◽  
Dr. Vimal Chandra Bhagat ◽  
Viyata Chanda ◽  
Dr. Rakesh Kumar Agrawal ◽  
...  
Keyword(s):  
Mental Health ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Groups ◽  
Emotional Stability ◽  
Two Dimensions ◽  
The Body ◽  
Cell Disease ◽  
Cross Sectional ◽  
Blood Disorder

Background: Sickle cell disease is an inherited blood disorder in which the body produces abnormal shaped red blood cells (RBC). The disease affects both biological and psychosocial aspects of patients. Aim: Present study aimed at investigating the different mental health dimensions used by heterozygous and homozygous sickle cell anemic patients. Method: The cross-sectional study design with the total 100 sickle cell anemic adolescents of both the sexes were selected in 10 to 20 year age groups, from various hospitals and health clinics of Chhattisgarh, India. The correlation analysis was used for analyzing the data. Results: Total 100 patients were selected which consisted of 30 homozygous and 70 heterozygous adolescent patients with sickle cell gene. The Emotional Stability was which higher significantly correlated with the intelligence quotients, IQ (r = .387, p< .001) than the other dimensions. Only two dimensions of mental health viz. emotional stability (t- 2.38; p<.018) and self-concept (t- 2.32; p<.001) of sickle cell patients which differed among heterozygous and homozygous patients.

Prevalence of ocular abnormalities in relation to sickle cell disease severity among children in South-western, Nigeria

2020 ◽  
pp. 112067212095761
Author(s):  
Oluwatoyin I. Oladimeji ◽  
Oluwagbemiga O. Adeodu ◽  
Oluwatoyin H. Onakpoya ◽  
Samuel A. Adegoke
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
The Body ◽  
University Teaching ◽  
Teaching Hospitals ◽  
Cell Disease ◽  
Cross Sectional ◽  
Ocular Abnormalities

Introduction: Sickle cell disease (SCD) ranks high among genetic disorders worldwide. It is characterised by repeated vaso-occlusion with resultant end-organ damage. This process can occur in all vascular beds in the body, including ocular blood vessels and may cause irreversible blindness in advanced stages. Little is known of the relationship between the prevalence of ocular abnormalities among children with SCD and their disease severity. Methods: A descriptive cross-sectional study was carried out at the Paediatric Haematology Clinics and the Eye Centre of the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife. Children with SCD in steady state were recruited from the Haematology Clinics and examined for ocular abnormalities at the Eye Centre of the hospital. The subjects SCD severity grade was determined using a previously validated scoring system. Results: One hundred and twenty (120) children aged 5 to 15 years were examined. Of these, 72 had one or more ocular abnormalities giving the prevalence of ocular abnormalities among them to be 60.0%. Though a higher proportion of children with moderate disease, 23 (65.7%) of 35, compared to those with mild disease, 49 (57.6%) of 85 had ocular abnormalities, this difference was not statistically significant, p = 0.412. Conclusion: Ocular abnormalities among Nigerian children with SCD are common even in steady-state, but not significantly associated with disease severity. Periodic screening for ocular abnormalities should thus be done on them irrespective of disease severity.

scholarly journals Comparative study of hypercoagulability change in steady state and during vaso-occlusive crisis among Sudanese patients living with sickle cell disease

2020 ◽  
Vol 20 (1) ◽  
pp. 392-396
Author(s):  
Elmigdad Abdelgadir Mohamed ◽  
Mamoud Mohamed Elgari ◽  
Asaad Mohammed Babker ◽  
Hisham Ali Waggiallah
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
International Normalized Ratio ◽  
P Value ◽  
Thrombin Time ◽  
Cell Disease ◽  
Cross Sectional ◽  
Blood Disorder ◽  
D Dimer

Background: Sickle cell disease (SCD) is an inherited blood disorder that affects red blood cells. (SCD) is characterized by re- current vaso-occlusive crisis (VOC). Material and methods: This was a descriptive cross sectional study conducted through the period from July 2015 to July 2017 in which a total of seventy two blood specimens were collected in 'EDTA' and citrated vacutainers from Sudanese patients with SCD attending "Fath Elrhman Albasheer" Centre. Both sexes' with different ages were included. Among these samples 49 (68.1%) were in steady state while the remained 23 (31.9%) were in VOC. All samples were tested for coagulation profile Result: There was increase in fibrinogen and D-dimer levels in most patients 67% and 71%, respectively. Significant increase in D-dimers was observed in patients with (VOC) compared with steady state (P. value = 0.006). Protein S was significantly in- creased in males in comparison with females P. value = 0.017. The results of prothrombin time (PT), international normalized ratio (INR) and thrombin time (TT) were within normal range. Conclusion: Significant increase in levels of D-dimer during VOC indicating abnormal coagulation and fibrinolysis activation. Reduced levels of natural anticoagulants proteins C and S can be consider as predictive markers indicate hepatic dysfunction in patients with SCD. Keyword: SCD; VOC; hypercoagulability; Sudanese patients. 

scholarly journals Study of Prevalance of Nephropathy among Sickle Cell Disease Patients in Waghodia Region, Vadodara, Gujarat

2021 ◽  
pp. 607-613
Author(s):  
Charmi C. Thakkar ◽  
Inampudi Sailaja
Keyword(s):  
Renal Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Mean Value ◽  
Cell Disease ◽  
Protein Loss ◽  
Cross Sectional ◽  
Blood Disorder ◽  
The Mean

Introduction: Sickle-cell disease (or drepanocytosis) is a life-long blood disorder Characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickle cell disease (SCD) has several complications, including chronic renal failure, manifesting with hypertension (high blood pressure) proteinuria (protein loss in the urine), hematuria (redblood cells in urine) and worsening anaemia. Progression to end-stage renal failure confers a poor prognosis. Objective: The objective of the study was to determine the Prevalence of Nephropathy among sickle cell disease patients. Materials and Methods: This cross sectional study includes a total 150 participants who suffering from sickle cell anemia and attending our Institute. Renal function test and Urine examination of all participants was done. Estimated Glomerular Filtration Rate (eGFR) calculated using the Cockroft Gault formula. Comparison of results was done between Sickle cell trait and Sickle cell disease Group. Results: The mean age of the SCA patients were 25.54±10 years. Maximum participants are found to be from age group 25-30 yr(n=35) followed by 20-25 yr(n=30). Of the 150 SCA patients, 89 (59.33%), and 61 (40.66%) were males and females, respectively. The Mean value of S.Creatinine of SCT group is 0.73±0.46 mg/dl and SCD is 1.0±0.35 mg/dl, while the Mean value of eGFR is 134.19±87.21 ml/min and 124.20 ±58.25 ml/min in SCT and SCD Group respectively. Conclusions: From our study we conclude that the Derangement of Kidney function in sickle cell disease is frequent in our setting especially among young adult. It concerns SCD as well as SCT patients. Albuminuria is more frequent in homozygote patients and its prevalence increase with age. Age ≥ 25 years is associated with high risk of CKD in SCA group and albuminuria in SCD.

Status of Sickle Cell Disease Among Tharu Population In Banke District of Nepal.

2021 ◽  
Vol 11 (5) ◽  
Author(s):  
Gupta Umesh Prasad ◽  
Bhandari Amrit ◽  
Giri Dhruba ◽  
Adhikari Sushmita ◽  
Paudel Sangita ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Age Groups ◽  
Test Method ◽  
Beta Thalassemia ◽  
Compound Heterozygous ◽  
Cell Disease ◽  
Cross Sectional ◽  
Solubility Test

Sickle cell disease (SCD) is prevalent in malaria-endemic areas because the gene for sickle cell provides its carrier with resistance against malaria. In Nepal, malaria is prevalent in Terai, hence the susceptibility of SCD is high in this region. Being indigenous to the Terai, thousands of people in the Tharu communities of the Banke districting Nepal are believed to have suffered from sickle cell disease. The objective of this study was to find out the status of sickle cell disease among the Tharu population of Banke district, Nepal. A cross-sectional, experimental study was performed among systematically randomly selected 275 samples from 3 Village Development Committee (VDCs). All the samples were first screened for the presence of sickle hemoglobin using the sickle solubility test method in Bheri Zonal Hospital. Then all sickle solubility positive samples were further processed for alkaline hemoglobin electrophoresis by using Interlab GenioS electrophoresis instrument. Out of a total 275 samples, 33 (12.0%) samples were confirmed as sickle solubility test positive. Among which, sickle cell trait was the most common disorder found grossing to 81.8%, followed by homozygous sickle cell disease; (15.2 %). One case (3.0%) of compound heterozygous sickle beta-thalassemia was also found.The Males were found to be more affected than females with ratio of 1.4:1.1. The highest frequency of SCD was found to be in 11-20 age groups comprising about 36.4%. Dangaura Tharu (51.5%) was the most common ethnic group with this disorder. The findings of this study indicate SCD is prevalent among the Tharu population in Banke district of Province-5, Nepal

The Emotional Impact of Sickle Cell Disease Symptoms: A Case Review

2021 ◽  
Vol 6 (2) ◽  
Author(s):  
Hosseini SA ◽  
◽  
Isola S ◽  
Bhatia M ◽  
Thara S ◽  
...  
Keyword(s):  
Mental Health ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Large Scale ◽  
Hospital Admissions ◽  
The Body ◽  
Emotional Impact ◽  
Case Review ◽  
Cell Disease ◽  
Liaison Psychiatry

Background: Sickle Cell Disease (SCD) is a chronic and debilitating disease that can affect almost any organ system in the body; hence, it is associated with a broad range of complications and comorbidities that may have a profound impact on a patients’ quality of life through medical and mental health consequences. Psychosocial and affective comorbidities are prevalent in SCD and could considerably influence disease outcomes; however, treatment options for SCD psychosocial sequelae are suboptimal, and evidence on their effectiveness is limited. Case Report: We report a 21-year-old African American female with a history of SCD and recurrent hospital admissions due to acute SCD complications, who was referred to the consultation-liaison psychiatry service for evaluation of psychiatric comorbidities and management recommendations. Conclusion: This case demonstrates the complexity of the relationship between somatic complications and neuropsychiatric disorders in SCD and highlights the importance of early identification and management of mental health conditions in this patient population. We emphasize treatment challenges and the need for large scale research on pharmacotherapies and nonpharmacological interventions in treating psychosocial comorbidities associated with SCD.

scholarly journals Antiphospholipid Antibodies in Sickle Cell Disease: A Systematic Review and Exploratory Meta-Analysis

2021 ◽  
Vol 27 ◽  
pp. 107602962110029
Author(s):  
Mira Merashli ◽  
Alessia Arcaro ◽  
Maria Graf ◽  
Matilde Caruso ◽  
Paul R. J. Ames ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Antiphospholipid Antibodies ◽  
Meta Analysis ◽  
Age Groups ◽  
Anticardiolipin Antibodies ◽  
Cell Disease ◽  
Pooled Prevalence ◽  
The Relationship

The relationship between antiphospholipid antibodies (aPL) and sickle cell disease (SCD) has never been systematically addressed. Our aim was to evaluate potential links between SCD and aPL in all age groups. EMBASE/PubMed was screened from inception to May 2020 and Peto odds ratios for rare events were calculated. The pooled prevalence (PP) of IgG anticardiolipin antibodies (aCL) was higher in individuals with SCD than in controls (27.9% vs 8.7%, P < 0.0001), that of IgM aCL was similar in the two groups (2.9% vs 2.7%); only individuals with SCD were positive for lupus anticoagulant (LA) (7.7% vs 0%, P < 0.0001). The PP of leg ulcers was similar between aPL positive and negative individuals (44% vs 53%) and between patients in acute crisis and stable patients (5.6% vs 7.3%). Reporting of aPL as a binary outcome and not as a titer precluded further interpretation. The results indicate that a prospective case-control study with serial measurements of a panel of aPL in SCD patients might be warranted, in order to understand further the possible pathogenic role of aPL in SCD.

scholarly journals Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Richard K. D. Ephraim ◽  
Patrick Adu ◽  
Edem Ake ◽  
Hope Agbodzakey ◽  
Prince Adoba ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Total Cholesterol ◽  
Sickle Cell ◽  
Density Lipoprotein ◽  
Hdl Cholesterol ◽  
Lipoprotein Cholesterol ◽  
Cell Disease ◽  
Cross Sectional

Background.Abnormal lipid homeostasis in sickle cell disease (SCD) is characterized by defects in plasma and erythrocyte lipids and may increase the risk of cardiovascular disease. This study assessed the lipid profile and non-HDL cholesterol level of SCD patients.Methods.A hospital-based cross-sectional study was conducted in 50 SCD patients, in the steady state, aged 8–28 years, attending the SCD clinic, and 50 healthy volunteers between the ages of 8–38 years. Serum lipids were determined by enzymatic methods and non-HDL cholesterol calculated by this formula: non-HDL-C = TC-HDL-C.Results.Total cholesterol (TC) (p=0.001) and high-density lipoprotein cholesterol (HDL-C) (p<0.0001) were significantly decreased in cases compared to controls. The levels of non-HDL-C, low-density lipoprotein cholesterol (LDL-C), and triglyceride (TG) were similar among the participants. The levels of decrease in TC and HDL were associated with whether a patient was SCD-SS or SCD-SC. Systolic blood pressure and diastolic blood pressure were each significantly associated with increased VLDL [SBP,p=0.01, OR: 0.74 (CI: 0.6–0.93); DBP,p=0.023, OR: 1.45 (CI: 1.05–2.0)].Conclusion.Dyslipidemia is common among participants in this study. It was more pronounced in the SCD-SS than in SCD-SC. This dyslipidemia was associated with high VLDL as well as increased SBP and DBP.

scholarly journals Reduction of the Six-Minute Walk Distance in Children with Sickle Cell Disease Is Correlated with Silent Infarct: Results from a Cross-Sectional Evaluation in a Single Center in Belgium

PLoS ONE ◽  
2014 ◽  
Vol 9 (10) ◽  
pp. e108922 ◽  
Author(s):  
Laurence Dedeken ◽  
Rudy Chapusette ◽  
Phu Quoc Lê ◽  
Catherine Heijmans ◽  
Christine Devalck ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Single Center ◽  
Cell Disease ◽  
Walk Distance ◽  
Cross Sectional ◽  
Minute Walk Distance ◽  
Minute Walk

scholarly journals The Practices of Anaesthesiologists in the Management of Patients with Sickle Cell Disease: Empirical Evidence from Cameroon

Healthcare ◽  
2021 ◽  
Vol 9 (12) ◽  
pp. 1617
Author(s):  
Dominique Djomo Tamchom ◽  
Aristide Kuitchet ◽  
Raymond Ndikontar ◽  
Serge Nga Nomo ◽  
Hermine Fouda ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Perioperative Management ◽  
Treatment Guideline ◽  
Binary Logistic Regression ◽  
Cross Sectional Study ◽  
Cell Disease ◽  
Binary Logistic Regression Model ◽  
Cross Sectional ◽  
Elective Surgical Procedures

Patients with sickle cell disease are more likely to undergo surgery during their lifetime, especially given the numerous complications they may develop. There is a paucity of data concerning the management of patients with sickle cell disease by anaesthesiologists, especially in Africa. This study aimed to describe the practices of anaesthesiologists in Cameroon concerning the perioperative management of patients with sickle cell disease. A cross-sectional study was carried out over four months and involved 35 out 47 anaesthesiologists working in hospitals across the country, who were invited to fill a data collection form after giving their informed consent. The data were analysed using descriptive statistics and a binary logistic regression model. Among the 35 anaesthesiologists included in the study, most (29 (82.9%)) had managed patients with sickle cell disease for both emergency and elective surgical procedures. Most of them had never asked for a haematology consultation before surgery. Most participants (26 (74.3%)) admitted to having carried out simple blood transfusions, while 4 (11.4%) carried out exchange transfusions. The haemoglobin thresholds for transfusion varied from one practitioner to another, between < 6 g/dl and < 9 g/dl. Only 6 (17.1%) anaesthesiologists had a treatment guideline for the management of patients with sickle cell disease in the hospitals where they practiced. Only 9 (25.7%) prescribed a search for irregular agglutinins. The percentage of haemoglobin S before surgery was always available for 5 (14.3%) of the participants. The coefficient (0.06) of the occurrence of a haematology consultation before surgery had a significant influence on the probability of management of post-operative complications (coefficient 0.06, 10% level of probability). This study highlights the fact that practices in the perioperative management of patients with sickle cell disease in Cameroon vary greatly from one anaesthesiologist to another. We disclosed major differences in the current recommendations, which support the fact that even in Sub-Saharan countries, guidelines applicable to the local settings should be published.

scholarly journals Microfluidics in Sickle Cell Disease Research: State of the Art and a Perspective Beyond the Flow Problem

2021 ◽  
Vol 7 ◽  
Author(s):  
Anupam Aich ◽  
Yann Lamarre ◽  
Daniel Pereira Sacomani ◽  
Simone Kashima ◽  
Dimas Tadeu Covas ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Flow Problem ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Intercellular Interaction ◽  
Blood Disorder ◽  
Resource Setting ◽  
Wide Range

Sickle cell disease (SCD) is the monogenic hemoglobinopathy where mutated sickle hemoglobin molecules polymerize to form long fibers under deoxygenated state and deform red blood cells (RBCs) into predominantly sickle form. Sickled RBCs stick to the vascular bed and obstruct blood flow in extreme conditions, leading to acute painful vaso-occlusion crises (VOCs) – the leading cause of mortality in SCD. Being a blood disorder of deformed RBCs, SCD manifests a wide-range of organ-specific clinical complications of life (in addition to chronic pain) such as stroke, acute chest syndrome (ACS) and pulmonary hypertension in the lung, nephropathy, auto-splenectomy, and splenomegaly, hand-foot syndrome, leg ulcer, stress erythropoiesis, osteonecrosis and osteoporosis. The physiological inception for VOC was initially thought to be only a fluid flow problem in microvascular space originated from increased viscosity due to aggregates of sickled RBCs; however, over the last three decades, multiple molecular and cellular mechanisms have been identified that aid the VOC in vivo. Activation of adhesion molecules in vascular endothelium and on RBC membranes, activated neutrophils and platelets, increased viscosity of the blood, and fluid physics driving sickled and deformed RBCs to the vascular wall (known as margination of flow) – all of these come together to orchestrate VOC. Microfluidic technology in sickle research was primarily adopted to benefit from mimicking the microvascular network to observe RBC flow under low oxygen conditions as models of VOC. However, over the last decade, microfluidics has evolved as a valuable tool to extract biophysical characteristics of sickle red cells, measure deformability of sickle red cells under simulated oxygen gradient and shear, drug testing, in vitro models of intercellular interaction on endothelialized or adhesion molecule-functionalized channels to understand adhesion in sickle microenvironment, characterizing biomechanics and microrheology, biomarker identification, and last but not least, for developing point-of-care diagnostic technologies for low resource setting. Several of these platforms have already demonstrated true potential to be translated from bench to bedside. Emerging microfluidics-based technologies for studying heterotypic cell–cell interactions, organ-on-chip application and drug dosage screening can be employed to sickle research field due to their wide-ranging advantages.

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