scholarly journals Awareness of Retinopathy among Sickle Cell Patients

2021 ◽  
pp. 73-80
Author(s):  
Shahad Alruwaili ◽  
Yasser Aleliwi ◽  
Shahd Al Mahfud ◽  
Mohammad Alshehri ◽  
Hamad Al Ghathber ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Knowledge Score ◽  
Cross Sectional Study ◽  
Eastern Region ◽  
Cell Disease ◽  
Cross Sectional ◽  
Self Administered Questionnaire ◽  
Autosomal Recessive Pattern

Background: Sickle cell disease (SCD) is a haemoglobinopathy that is inherited in an autosomal recessive pattern. Retinopathy is a complication of SCD, which varies from mild peripheral retinopathy to severe proliferative retinopathy that can cause loss of vision. Methods: A cross-sectional study was conducted from June 2020 to January 2021 aiming to determine the knowledge of retinopathy among patients with sickle cell disease using a validated self-administered questionnaire. Result: A total of 166 respondents completed the questionnaire. Females constituted the majority of participants (62.7%). The age group 31–40 years consitituted 28.3% and were associated with higher knowledge scores. The average knowledge score was also significantly higher in respondents from the Eastern region (B = 1.82, P < 0.05) compared to respondents from Riyadh. The main reason for not having an eye check was the perceived lack of importance of visits among 56.8%. Conclusion: Sickle cell disease patients are prone to complications such as retinopathy which can be detected by frequent follow-up. Almost half of the studied sample showed above average knowledge of retinopathies related to sickle cell disease.

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 Sexual Maturity in Adolescents Suffering from Sickle Cell Disease: A Cross-sectional Study

2021 ◽  
Author(s):  
Vijay Shah ◽  
Akash Patel ◽  
Praful Bambharoliya ◽  
Jigisha Patadia
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sexual Maturity ◽  
Tanner Stage ◽  
Cross Sectional Study ◽  
Reproductive Capacity ◽  
Sectional Study ◽  
Cell Disease ◽  
Cross Sectional ◽  
The Mean

Introduction: Sickle Cell Disease (SCD) is an inherited chronic haemolytic anaemia. The diseased person suffers from various complications such as anaemia, frequent infection, fever, hand-foot syndrome, stroke, etc. Puberty changes includes the appearance of the secondary sexual characteristics, increase in height, change in body composition and development of reproductive capacity. Aim: To study the sexual maturity and effect of multiple blood transfusions in adolescents suffering from SCD. Materials and Methods: It was a cross-sectional study conducted on 35 adolescents of age group 11 to 15 years, suffering from SCD. Study was conducted over a period of six months from March 2018 to September 2018 at Department of Paediatrics. SCD was diagnosed by Haemoglobin (Hb) electrophoresis. Weight and height were measured of all the participants. For assessing the sexual maturity, Tanners staging was used. Unpaired t-test was done for data analysis. Results: The mean age of the patients was 13.03±1.7 years. There were 25 males and 10 females. The mean age of male patients between Tanner stage 2(14.63±0.52 years) and Tanner stage 3 (14.75±0.5 years) was significantly higher than the Indian data for males (11.3 and 12.8 years, respectively). The mean age of female patients between Tanner stages 2 (13.5±2.12 years) and Tanner stage 3 (14.33±1.16 years) was higher than the Indian reference data for girls (10.2 and 11.6 years respectively). Conclusion: This study concluded that adolescents with SCD were significantly shorter in height and weight than the standard reference population. Sexual maturity is delayed in adolescents with sickle cell anaemia.

scholarly journals Asymmetric Dimethyl Arginine Level in Children with Sickle Cell Disease: A Cross Sectional Study

2021 ◽  
pp. 93-98
Author(s):  
Seham Fathy Khedr ◽  
Mohamed Hosny El Bradaey ◽  
Hala Mohamed Nagy ◽  
Mohamed Ramadan El-Shanshory ◽  
Eslam Elhawary
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cell Disease ◽  
Adma Level ◽  
Cross Sectional ◽  
Asymmetric Dimethyl Arginine ◽  
Dimethyl Arginine

Background: Sickle cell disease (SCD) consists of a group of hemoglobinopathies in which individuals inherit hemoglobin variants derived from single point mutations. Asymmetric dimethylarginine (ADMA) contributes to limiting Nitric Oxide (NO)  bioavailability in SCD. The aim of the present study was to assess the level of the Asymmetric Dimethyl Arginine in children with sickle cell. Methods: This cohort cross-sectional study was carried out on 60 children which were divided in to 3 equal groups. Group I: SCD children with sickle retinopathy. Group II: SCD children without retinopathy. Group III: healthy control children who were selected from the outpatient clinic. Results: There was a significant increase in ADMA level among participants withSCD. There was a positive significant correlation between ADMA  level and family history as well as the  incidence of hepatomegaly. There was no significant correlation between ADMA level and demographic and laboratory parameters except LDH. Conclusions: The level of ADMA is elevated in children with sickle cell anemia. High plasma ADMA level is a risk for hepatomegaly in children with sickle cell anemia.

scholarly journals A cross sectional study of growth of children with sickle cell disease, aged 2 to 5 years in Yaoundé, Cameroon

2019 ◽  
Vol 34 ◽  
Author(s):  
Suzanne Sap Ngo Um ◽  
Judith Seungue ◽  
Anastasie Yanda Alima ◽  
Ritha Mbono ◽  
Hubert Mbassi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cell Disease ◽  
Cross Sectional

scholarly journals Placenta growth factor in sickle cell disease: association with hemolysis and inflammation

Blood ◽  
2010 ◽  
Vol 115 (10) ◽  
pp. 2014-2020 ◽  
Author(s):  
Julia E. Brittain ◽  
Ben Hulkower ◽  
Susan K. Jones ◽  
Dell Strayhorn ◽  
Laura De Castro ◽  
...  
Keyword(s):  
Growth Factor ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Predictive Accuracy ◽  
Cross Sectional Study ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Placenta Growth Factor ◽  
Cross Sectional

Abstract Placenta growth factor (PlGF) is released by immature erythrocytes and is elevated in sickle cell disease (SCD). Previous data generated in vitro suggest that PlGF may play a role in the pathophysiology of SCD-associated pulmonary hypertension (PHT) by inducing the release of the vasoconstrictor, endothelin-1. In this cross-sectional study of 74 patients with SCD, we confirm that PlGF is significantly elevated in SCD compared with healthy control subjects. We found significantly higher levels of PlGF in SCD patients with PHT but observed no association of PlGF with the frequency of acute pain episodes or history of acute chest syndrome. The observed correlation between PlGF and various measures of red cell destruction suggests that hemolysis, and the resultant erythropoietic response, results in the up-regulation of PlGF. Although relatively specific, PlGF, as well as N-terminal pro-brain natriuretic peptide and soluble vascular cell adhesion molecule, has low predictive accuracy for the presence of PHT. Prospective studies are required to conclusively define the contribution of PlGF to the pathogenesis of PHT and other hemolytic complications in SCD.

scholarly journals Relationship between painful manifestations and the use of hydroxyurea in children and adolescents with sickle cell disease

2020 ◽  
Vol 9 (10) ◽  
pp. e6949109046
Author(s):  
Suzana Silva Lira ◽  
Caroline Maria Igrejas Lopes ◽  
Gabriella Aguiar Rodrigues Veras ◽  
Valdenice Aparecida de Menezes
Keyword(s):  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Short Form ◽  
Cross Sectional Study ◽  
High Rate ◽  
Physical Factors ◽  
Cell Disease ◽  
Cross Sectional ◽  
Painful Events

To analyze the profile of pain, its triggering factors, and the relationship with the use of hydroxyurea in children and adolescents with sickle cell disease. This was an analytical cross-sectional study of 80 patients with sickle cell disease, both male and female, aged 6 to 18 years, seen at the Center for Hematology and Hemotherapy of Pernambuco, Brazil. To assess the pain profile, forms with the adapted visual scale from the “Fear of Dental Pain Questionnaire – Short Form” were used. Fisher's exact test was used to evaluate the association between pain manifestations and the use of hydroxyurea. Pain was reported by 68.7% of the patients and 52.7% of them reported severe pain, which eventually affected their daily routines. Physical triggers presented the highest rate (78.2%). The most frequent sites of pain were the trunk (80%) and lower extremities (54.5%), and constricting (40%) and deep (40%) pain were the most frequent types. Thirty percent of the patients reported being treated with hydroxyurea for prevention of painful events. It is concluded that the patients with sickle cell disease showed a high rate of painful events and physical factors, such as cold temperature, trauma and physical effort, were considered to be the most prevalent. There was no association between the use of hydroxyurea and pain improvement.

scholarly journals Patterns of eating associated with the chronic diseases in Al-Ahsa, Saudi Arabia

2017 ◽  
Vol 4 (10) ◽  
pp. 3517 ◽  
Author(s):  
Hassan Ali Al Sayegh ◽  
Abdulaziz Adel Al Qurini ◽  
Abdul Sattar Khan ◽  
Sayed Ibrahim
Keyword(s):  
Sickle Cell Disease ◽  
Chronic Diseases ◽  
Sickle Cell ◽  
Eating Behaviors ◽  
Metabolic Diseases ◽  
Cross Sectional Study ◽  
Primary Concern ◽  
Cell Disease ◽  
Cross Sectional ◽  
Endemic Diseases

Background: There is increase in the limitation of daily activity of people with chronic diseases. This may be due to multiple factors one of them is the nature of the lifestyle for instance, patterns of eating that the person is following. It is important to sum the dietary behaviors in specific population to see how much is it associated with chronic diseases. Specially, obesity, cardiovascular diseases, diabetes and sickle cell disease which are endemic diseases in Al-Ahsa. Moreover, no studies have been conducted in this area before. It is known that there is increased obesity, cardiovascular and diabetes prevalence in Al-Ahsa. The study aims to see the association between chronic diseases and eating patterns. Methods: Cross sectional study, self-reported questionnaire distributed to the population under supervision of the data collectors in Al-Ahsa, during the summer of 2017. Results: The most prevalent chronic disease is obesity. Then comes hypertension and G6PD. Obesity showed most association with other chronic diseases. Also, eating frozen canned food show associations with obesity, hypertension, G6PD and diabetes. Conclusions: We concluded that there are certain eating behaviors influences some chronic diseases; there should be a focus on these behaviors to prevent the occurrence of these chronic diseases, especially metabolic diseases. Obesity is the primary concern which may facilitate the developing of hypertension, asthma, diabetes, high cholesterol, G6PD, hypothyroidism, hyperthyroidism, diabetes, sickle cell disease and cardiovascular events. 

scholarly journals Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study

PLoS ONE ◽  
2021 ◽  
Vol 16 (3) ◽  
pp. e0248649
Author(s):  
Marie Gerardin ◽  
Morgane Rousselet ◽  
Marie-Laure Couec ◽  
Agathe Masseau ◽  
Marylène Guerlais ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Descriptive Analysis ◽  
Specific Treatment ◽  
Cross Sectional Study ◽  
Social Consequences ◽  
Cell Disease ◽  
Cross Sectional ◽  
Link Type ◽  
The Impact

Background Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose—drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables. Methods The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use. Results The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences. Conclusions The impact of SCD on patients’ lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis. Trial registration Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.

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