scholarly journals Sickle cell disease and the adherence to guidelines for the use of blood transfusions in Duhok, Kurdistan, Iraq

2021 ◽  
Vol 25 (1) ◽  
pp. 486-492
Author(s):  
Fahad Jameel ◽  
Adnan Sadeeq
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Cross Sectional ◽  
Medical Visits ◽  
Transfusion Guidelines

Background and objective: One of the important health problems in Duhok city, Iraqi Kurdistan Region, is sickle cell disease. Blood transfusion remains a significant therapeutic intervention in patients with sickle cell disease that reduces complications related to vaso-occlusions. This study aimed to assess compliance to guidelines for the use of blood transfusionsin Duhok, Kurdistan, Iraq. Methods: This is a cross-sectional study that included 135 patients with sickle cell disease registered at Jeen center of pediatric hematological diseases in Duhok, Kurdistan, Iraq. Between April 1st and July 31st, 2019, 205 medical visits of sickle cell disease were registered. Every patient was evaluated to record the clinical setting and explanations behind visiting and indications for transfusion. Results: Of 135 patients,65.9% had sickle cell anemia (HbSS disease), 33.3% had sickle beta thalassemia, and one patient (0.7%) had Sickle/D disease. A total of 205 medical visits of sickle cell disease were registered with 84 blood transfusion decisions. The most common indicated guideline reasons for transfusion were symptomatic anemia and acute hemolytic crisis with a drop of hemoglobin >2 g/dl below steady state hemoglobin and severe painful crisis only accounted for 38.1%. Conclusion: In this study of patients with sickle cell disease, most blood transfusions were not indicated according to the transfusion guidelines (British Committee for Standards in Haematology – BCSH, 2017). Keywords: Transfusion guidelines for sickle cell disease (BCSH 2017); Sickle cell disease; Iraq.

scholarly journals The prevalence of human immunodeficiency and of hepatitis B viral infections is not increased in patients with sickle cell disease in Tanzania

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Grace Shayo ◽  
Irene Makundi ◽  
Lucio Luzzatto
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Hepatitis B ◽  
Sickle Cell ◽  
Viral Infections ◽  
Cross Sectional Study ◽  
Dar Es Salaam ◽  
Categorical Variables ◽  
Cell Disease ◽  
Cross Sectional

Abstract Background Tanzania ranks as the fourth country in the world with respect to the number of sickle cell disease (SCD) births; it is also endemic to the human immunodeficiency virus (HIV) and the hepatitis B virus (HBV). This study was done to determine the prevalence of HIV and HBV infections among SCD patients in Dar es Salaam, Tanzania. Methods A multicenter hospital-based descriptive cross sectional study was carried out among participants aged ≥ 16 years with a proven diagnosis of SCD. Socio-demographic and clinical data were recorded. Blood samples were drawn for HIV and HBV diagnosis. All categorical variables were summarized into frequencies. Results There were 185/325 (56.9 %) females. The mean age (SD) was 23.0 ± 7.5 years. The prevalence of HIV was 1.8 %; the prevalence of HBV was 1.2 %. Conclusions The prevalence of both HIV and HBV in SCD patients is no greater than in the general population of Dar es Salaam or Tanzania. For associations, a large study would be needed. From a detailed blood transfusion history of SCD patients we found no evidence that HIV or HBV infection was transmitted through blood transfusion.

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. 

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