scholarly journals Using the DFConhecimento instrument to assess Congolese healthcare professionals’ knowledge on sickle cell disease

2021 ◽  
Vol 5 (1) ◽  
pp. 015-020
Author(s):  
Katamea Tina ◽  
Mukuku Olivier ◽  
Nzala Patient Dinanga ◽  
Nsasi Bénédicte Malonda ◽  
Mpoy Charles Wembonyama ◽  
...  

Introduction: Despite advances in the management of sickle cell disease (SCD), gaps still exist in the knowledge of healthcare professionals (HCPs) about the disease. The objective of this study was to assess the knowledge of HCPs about SCD. Methods: This is a cross-sectional study involving 465 HCPs (physicians and nurses) who responded to the DFConhecimento instrument questionnaire. Performance was tested in terms of average score and proportion of correct response for each questionnaire item topic. Results: The average score for respondents was 4.6 ± 1.9 out of a total of 13 points. Proportions of professionals who responded well were greater than 58% in three topics (Neonatal screening program, Sickle cell conditions, and Sickle cell anemia genotype). In the other topics, rates of good response ranged from 11.6% to 46.0%. There was a statistical association between best performance and medical title: physicians were more knowledgeable than nurses (OR = 6.26; 95% CI: 2.69-14.56). Conclusion: This study highlighted that knowledge of SCD among HCPs is very inadequate. This lack of sufficient information on SCD from HCPs indicates the need to develop continuing education programs.

Healthcare ◽  
2021 ◽  
Vol 9 (12) ◽  
pp. 1617
Author(s):  
Dominique Djomo Tamchom ◽  
Aristide Kuitchet ◽  
Raymond Ndikontar ◽  
Serge Nga Nomo ◽  
Hermine Fouda ◽  
...  

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.


Author(s):  
Vijay Shah ◽  
Akash Patel ◽  
Praful Bambharoliya ◽  
Jigisha Patadia

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.


Author(s):  
Seham Fathy Khedr ◽  
Mohamed Hosny El Bradaey ◽  
Hala Mohamed Nagy ◽  
Mohamed Ramadan El-Shanshory ◽  
Eslam Elhawary

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.


2019 ◽  
Vol 34 ◽  
Author(s):  
Suzanne Sap Ngo Um ◽  
Judith Seungue ◽  
Anastasie Yanda Alima ◽  
Ritha Mbono ◽  
Hubert Mbassi ◽  
...  

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Oladele Simeon Olatunya ◽  
Adefunke Olarinre Babatola ◽  
Ezra Olatunde Ogundare ◽  
Babatunde Ajayi Olofinbiyi ◽  
Olubunmi Adeola Lawal ◽  
...  

Background. Early sickle cell disease (SCD) diagnosis has shown promise in combating SCD in many countries. The aim of this study was to assess the practice and perception of early SCD diagnosis among a group of parents and physicians in Nigeria. Patients and Methods. This was a cross-sectional descriptive study conducted to assess the opinions and practice of early diagnosis of SCD among 135 physicians caring for SCD patients and 164 mothers of children with SCD in a southwestern state of Nigeria. Results. Most physicians 132 (97.8%) were aware of prenatal SCD diagnosis, but only 51 (37.8%) would recommend it. Most physicians 129 (95.6%) routinely recommend premarital SCD genetic counseling and testing, and 89 (65.1%) were aware of the national government newborn screening program but lesser proportion 75 (55.6%) were willing to recommend it. Amongst the mothers, 154 (94%) and 158 (96%) had encountered genetic counseling for SCD and were willing to offer newborn screening to their children, respectively. On the contrary, fewer mothers 42 (25%) were aware of prenatal SCD diagnosis, 28 (17%) were willing to partake in it, and 44 (26%) were undecided. There were discrepancies in the willingness by physicians to practice early SCD diagnosis and its uptake by mothers (p<0.0001). The commonest reason given by both the physicians and mothers for not practicing SCD prenatal diagnosis was the high cost of the procedure. Conclusion. The perceptions and practice of early SCD diagnosis was suboptimal in the study locality. Scaling up awareness and universal coverage are required.


Blood ◽  
2010 ◽  
Vol 115 (10) ◽  
pp. 2014-2020 ◽  
Author(s):  
Julia E. Brittain ◽  
Ben Hulkower ◽  
Susan K. Jones ◽  
Dell Strayhorn ◽  
Laura De Castro ◽  
...  

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.


2020 ◽  
Vol 9 (10) ◽  
pp. e6949109046
Author(s):  
Suzana Silva Lira ◽  
Caroline Maria Igrejas Lopes ◽  
Gabriella Aguiar Rodrigues Veras ◽  
Valdenice Aparecida de Menezes

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.


Author(s):  
Hassan Ali Al Sayegh ◽  
Abdulaziz Adel Al Qurini ◽  
Abdul Sattar Khan ◽  
Sayed Ibrahim

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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