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1.
Blood ; 143(14): 1326-1327, 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38573609
2.
Hematol Oncol Stem Cell Ther ; 17(2): 130-136, 2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38560974

RESUMO

OBJECTIVE: This study aims to compare the polysomnographic features between Arab-Indian and Benin phenotypes of sickle cell disease (SCD). MATERIALS AND METHODS: This prospective cross-sectional study was conducted in the Children's Hospital at King Fahad MedicalCity, in Riyadhwhere childrenwere recruited fromthe pediatric hematology clinic and pediatric sleepmedicine. All families were approached and patients who met the inclusion criteria and agreed to participate were included in the study. RESULTS: Eighty four children (37 of whom were females) with SCD were included in the study. Their median (interquartile) age was 9 (6.65, 11) years and their body mass index z score was -1.45 (-2.195, -1.45). The evidence of obstructive sleep apnea (OSA) was more prominent in the Benin phenotype (66.7%) in comparison to those of the Arab-Indian (35.2%) phenotype ( p = 0.006). Additionally, 56.7% of Benin had moderate to severe OSA whereas Arab-Indian had 18% with a ( p = 0.0003). Controlling for other factors, the odds ratio (confidence interval) of having OSA in Benin phenotype was 4.68 (1.42-15.38) times higher as compared to Arab-Indian phenotype. CONCLUSION: The risk of having OSA as well as the severity of OSA is higher in Benin phenotype as compared to Arab-Indian phenotype which indicates the presence of potential OSA risk factors other than the SCD itself.


Assuntos
Anemia Falciforme , Apneia Obstrutiva do Sono , Feminino , Humanos , Criança , Masculino , Estudos Transversais , Estudos Prospectivos , Polissonografia , Apneia Obstrutiva do Sono/epidemiologia , Anemia Falciforme/complicações , Fenótipo
3.
Hematol Oncol Stem Cell Ther ; 17(2): 120-129, 2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38560971

RESUMO

BACKGROUND AND OBJECTIVES: Bone marrow mesenchymal stromal cells (BM-MSCs) are key elements of the hematopoietic niche and participate in the regulatory mechanisms of hematopoietic stem cells (HSCs). Hematological diseases can affect MSCs and their functions. However, the dysregulations caused by sickle cell disease (SCD) are not fully elucidated. This work explored changes in BM-MSCs and their relationship with age using sickle cell mice (Townes-SS). MATERIALS AND METHODS: BM-MSCs were isolated from Townes-SS, and control groups 30- and 60-day-old Townes-AA and C57BL/6 J. RESULTS: The BM-MSCs showed no morphological differences in culture and demonstrated a murine MSC-like immunophenotypic profile (Sca-1+, CD29+, CD44+, CD90.2+, CD31-, CD45-, and CD117-). Subsequently, all BM-MSCs were able to differentiate into adipocytes and osteocytes in vitro. Finally, 30-day-old BM-MSCs of Townes-SS showed higher expression of genes related to the maintenance of HSCs (Cxcl12, Vegfa, and Angpt1) and lower expression of pro-inflammatory genes (Tnfa and Il-6). However, 60-day-old BM-MSCs of Townes-SS started to show expression of genes related to reduced HSC maintenance and increased expression of pro-inflammatory genes. CONCLUSION: These results indicates age as a modifying factor of gene expression of BM-MSCs in the context of SCD.


Assuntos
Anemia Falciforme , Células-Tronco Mesenquimais , Humanos , Animais , Camundongos , Medula Óssea , Camundongos Endogâmicos C57BL , Células-Tronco Hematopoéticas/metabolismo , Células-Tronco Mesenquimais/metabolismo , Células da Medula Óssea/metabolismo , Diferenciação Celular
4.
Andes Pediatr ; 95(1): 41-52, 2024 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-38587343

RESUMO

Sickle cell anemia (SCA) is the most common genetic disease worldwide. There are countries with massive public health programs for early detection of this condition. In the literature, several specific haplotypes or single-base polymorphic variants (SNPs) have been associated with the SCA prognosis. OBJECTIVE: To demonstrate the significant correlation of SNPs relevant to the diagnosis and prognosis of SCA among different ethnic groups. METHODOLOGY: we analyzed population frequencies and correlations of several SNPs related to the prognosis of SCA (i.e., baseline fetal hemoglobin levels), response to hydroxyurea treatment, and response to other drugs used in the SCA treatment, collected from validated genomic databases among different ethnic groups. RESULTS: The calculation of the Hardy-Weinberg equilibrium and the logistic regression was successful in classifying the ethnic groups as African (0 = 0.78, 1 = 0.89), and with a lower efficiency as American (AMR) (0 = 0.88, 1 = 0.00), East Asian (EAS) (0 = 0.80, 1 = 0.00), European (EUR) (0 = 0.79, 1 = 0.00), and South Asian (SAS) (0 = 0.80, 1 = 0.00). CONCLUSIONS: The results extend those from previous reports and show that the profile of most of the SNPs studied presented statistically significant distributions among general ethnic groups, pointing to the need to carry out massive early screening of relevant SNPs for SCA in patients diagnosed with this disease. It is concluded that the application of a broad mutation detection program will lead to a more personalized and efficient response in the treatment of SCA.


Assuntos
Anemia Falciforme , Medicina de Precisão , Humanos , Anemia Falciforme/diagnóstico , Anemia Falciforme/genética , Anemia Falciforme/terapia , Mutação , Etnicidade/genética , Prognóstico
6.
AANA J ; 92(2): 87-92, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38564204

RESUMO

Enhanced recovery after surgery (ERAS) is a patient-centered, evidence-based, multidisciplinary team-developed approach to a surgical stress response that is implemented to optimize physiological function and facilitate recovery for the best possible outcomes from surgery. Although there are currently well-known published guidelines for the perioperative management of patients with sickle cell disease, there are currently no specific and evidencebased ERAS protocols that address the needs of these patients. A novel mechanistic model has recently been found that could change ERAS protocols for patients with sickle cell disease with regard to a current preoperative carbohydrate loading drink recommendation, nutrition and intravenous fluid management. ERAS has great benefits for most patient populations, but emerging research suggests that patients with sickle cell disease may process and respond differently to varying concentrations of serum glucose and serum cations (hyperglycemia and hypertonic states). This adverse response involves actin, a cytoskeletal protein, in the red blood cell and how increased hemoglobin glycosylation may lead to a malfunction in this protein and a transition to vaso-occlusive crises in patients with sickle cell disease. Further research is warranted with this new mechanistic model to develop more meticulous and customized perioperative management plans to address risk mitigation in patients with sickle cell disease.


Assuntos
Anemia Falciforme , Recuperação Pós-Cirúrgica Melhorada , Humanos , Administração Intravenosa
7.
BMJ Open ; 14(4): e080235, 2024 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-38580373

RESUMO

INTRODUCTION: Children with sickle cell disease show a significant decrease in bone mineral density, an increase in resting energy expenditure of more than 15%, a decrease in fat and lean mass as well as a significant increase in protein turnover, particularly in bone tissue. This study aims to evaluate the effectiveness of an increase in food intake on bone mineral density and the clinical and biological complications of paediatric sickle cell disease. METHODS AND ANALYSIS: The study is designed as an open-label randomised controlled clinical trial conducted in the Paediatrics Unit of the Orléans University Hospital Centre. Participants aged 3-16 years will be randomly divided into two groups: the intervention group will receive oral nutritional supplements (pharmacological nutritional hypercaloric products) while the control group will receive age-appropriate and gender-appropriate nutritional intake during 12 months. Total body less head bone mineral density will be measured at the beginning and the end of the trial. A rigorous nutritional follow-up by weekly 24 hours recall dietary assessment and planned contacts every 6 weeks will be carried out throughout the study. A school absenteeism questionnaire, intended to reflect the patient's school productivity, will be completed by participants and parents every 3 months. Blood samples of each patient of both groups will be stocked at the beginning and at the end of the trial, for future biological trial. Clinical and biological complications will be regularly monitored. ETHICS AND DISSEMINATION: The protocol has been approved by the French ethics committee (Comité de Protection des Personnes Sud-Ouest et Outre-Mer 2, Toulouse; approval no: 2-20-092 id9534). Children and their parents will give informed consent to participate in the study before taking part. Results will be disseminated through peer-reviewed journals or international academic conferences. TRIAL REGISTRATION NUMBER: NCT04754711.


Assuntos
Anemia Falciforme , Densidade Óssea , Humanos , Criança , Suplementos Nutricionais , Osso e Ossos , Anemia Falciforme/terapia , Ensaios Clínicos Controlados Aleatórios como Assunto
8.
Braz Oral Res ; 38: e026, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38597546

RESUMO

This study validated the content of an instrument designed to assess the knowledge, involvement (attitudes) and management (practice) of dentists relative to sickle-cell disease (KAPD-SCD). The instrument consisted of five domains composed of a total of thirteen items: I. Dentist's self-assessment relative to sickle-cell disease; II. Dentist's knowledge of the repercussions of sickle-cell disease on the stomatognathic system; III. Dentist's knowledge of the complications of sickle-cell disease in the stomatognathic system; IV. Dentist's knowledge concerning the dental management of sickle-cell disease patients; and V. Dentist's involvement in an approach to sickle-cell disease. Twelve experts assigned scores to each item of the instrument. The criteria were clarity, understanding and appropriateness, leaving open fields for comments. Descriptive and content analyses of the data were made. Each expert analyzed 39 assessment units. The percentages considered for agreement were high (>80%), medium (70%-80%), or low (<70%), and each item was maintained or revised according to the percentage observed. There was high consensus in 74% of the assessment units (the corresponding items were maintained), medium consensus in 24% of them (the corresponding items were revised), and disagreement in 2% of them, namely as regards the "appropriateness" of item 5 ("Are there oral complications in sickle-cell disease?"), which was revised. The final version of the instrument had 16 items for different applications such as in the clinical care program, teaching program, or research program, with different cut-off scores for each application. In conclusion, the level of agreement among experts showed evidence of the content validity of the instrument.


Assuntos
Anemia Falciforme , Humanos , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Odontólogos , Inquéritos e Questionários
9.
BMC Res Notes ; 17(1): 100, 2024 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-38589908

RESUMO

OBJECTIVES: Hypoxia is a known feature of sickle cell anaemia (SCA) which results from chronic anaemia and recurrent vaso-occlusive crisis (VOC) which can cause tissue ischaemia that leads to an end organ damage. The hallmark of SCA is chronic anaemia and recurrent vaso-occlusive crisis. The aim of this study is to compare the oxygen saturation of sickle cell anaemic individuals with the normal haemoglobin type (Hb AA) control and also to determine the prevalence of hypoxemia among SCA. RESULTS: Two-hundred and twenty-two (136 Hb SS and 86 Hb AA) participated in the study. The mean ± SD of age (years), oxygen saturation (%) and pulse rate (bpm) of participants with sickle cell anaemia and Hb AA control were 21.85 ± 3.04 and 22.14 ± 3.18 (t = 0.701, p = 0.436), 95.21 ± 3.02 and 98.07 ± 0.81 (t=-8.598, p < 0.0001) and 77.10 ± 9.28 and 73.16 ± 8.52 (t = 3.173, p = 0.002) respectively. The prevalence of hypoxemia among SCA participants was 47.1%. Prevalence of hypoxemia in males with SCA was 60.9% while 39.1% of the females had hypoxemia.


Assuntos
Anemia Falciforme , Masculino , Feminino , Humanos , Adulto Jovem , Nigéria/epidemiologia , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Hipóxia/epidemiologia , Estudos Transversais
11.
Sci Rep ; 14(1): 7315, 2024 03 27.
Artigo em Inglês | MEDLINE | ID: mdl-38538687

RESUMO

Sickle cell disease (SCD) is a genetic disorder causing painful and unpredictable Vaso-occlusive crises (VOCs) through blood vessel blockages. In this study, we propose explosive synchronization (ES) as a novel approach to comprehend the hypersensitivity and occurrence of VOCs in the SCD brain network. We hypothesized that the accumulated disruptions in the brain network induced by SCD might lead to strengthened ES and hypersensitivity. We explored ES's relationship with patient reported outcome measures (PROMs) as well as VOCs by analyzing EEG data from 25 SCD patients and 18 matched controls. SCD patients exhibited lower alpha frequency than controls. SCD patients showed correlation between frequency disassortativity (FDA), an ES condition, and three important PROMs. Furthermore, stronger FDA was observed in SCD patients with a higher frequency of VOCs and EEG recording near VOC. We also conducted computational modeling on SCD brain network to study FDA's role in network sensitivity. Our model demonstrated that a stronger FDA could be linked to increased sensitivity and frequency of VOCs. This study establishes connections between SCD pain and the universal network mechanism, ES, offering a strong theoretical foundation. This understanding will aid predicting VOCs and refining pain management for SCD patients.


Assuntos
Anemia Falciforme , Dor , Humanos , Dor/etiologia , Anemia Falciforme/complicações , Manejo da Dor/efeitos adversos , Encéfalo
13.
Sultan Qaboos Univ Med J ; 24(1): 58-62, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38434462

RESUMO

Objectives: Despite the numerous advances in management strategies, treating osteomyelitis in individuals with sickle cell disease (SCD) remains a significant challenge, leading to severe long-term consequences. This study aimed to assess the key factors potentially linked to a complex progression of osteomyelitis in patients diagnosed with SCD. Methods: A cohort of 34 patients was identified and their progress was monitored over a span of 12 months during a 10-year period (2010-2020). The variables under investigation encompassed demographic and clinical traits, laboratory analyses and imaging data, as well as the treatment strategies employed. Results: The risk prediction model pinpointed 5 factors (severity of SCD, involvement of lower limbs, presence of bacteraemia, magnetic resonance image [MRI] findings and utilisation of surgical debridement) that exhibited an area under the curve (AUC) exceeding 0.7. Causative organisms were identified in 9 out of the total 34 patients (26.47%). A total of 17 patients displayed a severe course of SCD (AUC = 7.88), with MRI being highlighted as a valuable contributing factor (AUC = 7.88). Furthermore, 13 patients (38.2%) underwent surgical debridement, a procedure that yielded a statistically significant P value of 0.012 and an AUC of 0.714. Conclusion: Osteomyelitis within the context of severe SCD, particularly when accompanied by lower extremity infection, bacteraemia, positive MRI findings and the need for surgical debridement, emerges as a cluster of risk factors predisposing individuals to osteomyelitis relapse and a more complex disease course.


Assuntos
Anemia Falciforme , Bacteriemia , Osteomielite , Humanos , Anemia Falciforme/complicações , Área Sob a Curva , Extremidade Inferior
14.
BMJ Open ; 14(3): e083688, 2024 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-38448082

RESUMO

INTRODUCTION: Sickle cell disease (SCD) poses a significant global health burden, particularly affecting individuals in developing countries with constrained healthcare resources. While research on self-management in the context of SCD is emerging, it has predominantly focused on primary studies, and there is a notable dearth of evidence synthesis on SCD self-management in developing countries. This scoping review aims to identify and map self-management needs of individuals living with SCD, the strategies they employed to meet those needs, and the support systems available to them. METHODS AND ANALYSIS: The review will be conducted following the Arksey and O'Malley's (2005) 29 framework to comprehensively examine the landscape of SCD self-management research. Searches will be performed in PubMed, Scopus, Embase and Dimensions AI, with additional searches in other databases and grey literature. Indexed literature published in English from inception to January 2024 will be included. Reference list from included studies will also be searched manually. Two teams will be constituted to independently screen titles, abstracts and full text against the eligible criteria. Data will be extracted from included studies onto a customised data extraction form. ETHICS AND DISSEMINATION: Ethical approval is not required for this review due to the fact that it synthesises information from available publications. The findings will be disseminated through publication in a peer-reviewed journal. Also, the findings will possibly be presented at relevant international and national conferences. This protocol has already been registered with the Open Science Framework. The study characteristics such as design and setting will be descriptively analysed and presented as graphs, tables and figures. Thematic analysis will also be conducted based on the study objectives and presented as a narrative summary.


Assuntos
Anemia Falciforme , Autogestão , Humanos , Países em Desenvolvimento , Anemia Falciforme/terapia , Bases de Dados Factuais , Literatura Cinzenta , Literatura de Revisão como Assunto
15.
Cien Saude Colet ; 29(3): e06752023, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38451648

RESUMO

The aim was to associate living, health and oral health conditions with the quality of life (QL) of children and adolescents (CA) with sickle cell disease (SCD). Of the 120 eligible users of a public hematological service, 106 CA with SCD from 6 to 18 years of age, and their caregivers, answered semi-structured questions about socio-demographic, health and oral health conditions. For QL, we used the validated instrument PedsQLSCD™. The oral clinical examination occurred according to the guidelines of WHO and SB Brazil 2010. The majority of CA were non-white people (88%), mean age of 10.4 (±2.9) years, family income of up to two monthly minimum wages, for 03 to 05 members, with diagnosis of sickle cell anemia by neonatal screening, hospitalizations were due allergic crises, polypharmacy and dental caries (51%) were present. "About the Impact of My Pain" was the best-fit model for the QLSCD (adjusted R²=56%; AIC=28.67; p=0.04). Dental caries in permanent dentition worsened the QLSCD (OR=0.53; IC95%=0.35-0.78; p<0.05) and was associated with the type of school, car ownership, number of family members, of complications and of the medications. To overcome this scenario, programmatic actions are required, and implementation of public policies specifically directed towards these groups.


Assuntos
Anemia Falciforme , Cárie Dentária , Criança , Recém-Nascido , Humanos , Adolescente , Dentição Permanente , Qualidade de Vida , Cárie Dentária/epidemiologia , Anemia Falciforme/epidemiologia , Brasil/epidemiologia
16.
Cien Saude Colet ; 29(3): e06772023, 2024 Mar.
Artigo em Português, Inglês | MEDLINE | ID: mdl-38451649

RESUMO

Sickle cell disease (SCD) is an emblematic case of historical health neglect in Brazil and reflects how institutional racism produces health inequalities. This article engaged in a historical journey of this disease, showing the delayed implementation of health policies for people with sickle cell disease, often concealed in Public Power's (in)actions and omissions. The lack of commitment to implement the recommendations of the Brazilian Ministry of Health, such as neonatal screening, and the difficulty in incorporating technologies for health care result from this modus operandi. The advances and setbacks in programmatic actions and the constant pressure on several governmental entities have characterized the reported saga in the last twenty years. The present text discusses the policies for people with SCD, appropriating the Sankofa symbol, meaning that building the present is only possible by remembering past mistakes. Thus, we recognize this trajectory and this historical moment in which there is a concrete possibility of moving forward and achieving the longed-for comprehensive care for people with SCD. There is an invitation to glance at a new perspective, one in which hope is the trigger for the movements needed to guarantee the rights of people with SCD.


A doença falciforme (DF) é um caso emblemático de negligência histórica em saúde no Brasil e reflete como o racismo institucional produz iniquidades em saúde. Este artigo fez um percurso histórico até os dias atuais e mostra atraso na implementação de políticas de saúde voltadas para as pessoas com DF, tantas vezes encoberto em (in)ações e omissões do poder público. O descompromisso para a efetivação das recomendações do Ministério da Saúde, a exemplo da triagem neonatal, e a dificuldade de incorporar as tecnologias para a assistência à saúde resultam desse modus nada operandi. Os avanços e retrocessos nas ações programáticas, bem como a pressão constante sobre os diversos entes governamentais, caracterizaram a saga dos últimos 20 anos. O texto disserta sobre as políticas voltadas para as pessoas com DF, apropriando-se da simbologia Sankofa, já que só é possível construir o presente pelo aprendizado dos erros do passado. Assim, reconhecemos essa trajetória e esse momento histórico em que há possibilidade concreta de avançar e concretizar o tão almejado cuidado integral para pessoas com DF. Concluiu-se que há um convite para um novo olhar, em que esperançar seja o disparador das movimentações necessárias para a garantia do direito para as pessoas com DF.


Assuntos
Anemia Falciforme , Humanos , Recém-Nascido , Anemia Falciforme/epidemiologia , Brasil , Assistência Integral à Saúde , Governo , Instalações de Saúde
17.
Cien Saude Colet ; 29(3): e11782023, 2024 Mar.
Artigo em Português, Inglês | MEDLINE | ID: mdl-38451654

RESUMO

This is a study on sickle cell disease, a chronic illness that affects many Brazilians, that aims to understand and analyze how people address the adversities arising from the diagnosis and the biographical rupture. The description of people's experiences and strategies conjures a picture that expresses the respondents' habitus, built in a dialectical relationship with the vulnerability determined by the disease. We adopted a qualitative approach and focused interviews as proposed by Merton, combined with the snowball technique, applied to groups related to sickle cell disease on social networks. Seven participants were selected because they were privileged informants with the disease, were over eighteen, lived in the Federal District, and were non-exclusive users of the Unified Health System. The interview material was categorized from the focal groups employed. The results indicated the following categories: biographical rupture, experience and coping strategies, and healthcare. It is necessary to sensitize professionals and the population about the challenging living conditions of people with sickle cell disease and the consolidation of public policies and care networks to accommodate this population.


Trata-se de um estudo sobre a doença falciforme, enfermidade crônica que afeta muitas pessoas no Brasil. O objetivo foi compreender como as pessoas lidam com as adversidades oriundas do diagnóstico e a ruptura biográfica. A descrição das experiências e estratégias empregadas pelas pessoas compuseram um quadro que expressa os habitus dos entrevistados, construídos em relação dialética com a vulnerabilidade determinada pela doença. A abordagem foi qualitativa e empregou entrevistas focadas como propostas por Merton, a técnica bola de neve aplicada em grupos relacionados à doença falciforme em redes sociais. Sete participantes foram selecionados por serem informantes privilegiados, portadores da doença, maiores de 18 anos, moradores no Distrito Federal e usuários não exclusivos do Sistema Único de Saúde. O material das entrevistas foi categorizado a partir dos núcleos focais empregados. Os resultados apontaram as categorias: ruptura biográfica, experiência e estratégias de enfrentamento, atenção à saúde. Concluímos que é necessária uma sensibilização dos profissionais e da população sobre as dificuldades da condição de vida das pessoas com doença falciforme e a consolidação das políticas públicas e das redes de atendimento para acolher essa população.


Assuntos
Anemia Falciforme , População da América do Sul , Humanos , Anemia Falciforme/epidemiologia , Brasil , Grupos Focais
18.
Med Sci Monit ; 30: e944204, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38425279

RESUMO

In 2020, Emmanuelle Charpentier and Jennifer Doudna were awarded the Nobel Prize in Chemistry for their research on the endonuclease, clustered regularly interspaced short palindromic repeats (CRISPR) and the CRISPR-associated protein 9 (CRISPR-Cas9) method for DNA editing. On 16 November 2023, the UK Medicines and Healthcare Products Regulatory Agency (MHRA) was the first to approve the CRISPR-Cas9 gene editing therapy, Casgevy (exagamglogene autotemcel), for the treatment of patients with transfusion-dependent b-thalassemia and the treatment of sickle cell disease in patients aged ≥12 years with recurrent vaso-occlusive crises. On 8 December 2023, the US Food and Drug Administration (FDA) approved both Casgevy and Lyfgenia (lovotibeglogene autotemcel) for patients with sickle cell disease. On 15 December 2023, the European Medicines Agency (EMA) approved Casgevy for sickle cell disease and transfusion-dependent ß-thalassemia. This Editorial aims to present an update on the landmark first regulatory approvals of CRISPR-Cas9 for patients with sickle cell disease and transfusion-dependent b-thalassemia and the potential challenges for therapeutic gene (DNA) editing.


Assuntos
Anemia Falciforme , Talassemia beta , Estados Unidos , Humanos , Edição de Genes/métodos , Sistemas CRISPR-Cas/genética , Talassemia beta/genética , Talassemia beta/terapia , Anemia Falciforme/genética , Anemia Falciforme/terapia , DNA
19.
Afr J Prim Health Care Fam Med ; 16(1): e1-e8, 2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38426772

RESUMO

BACKGROUND:  In Kinshasa, Democratic Republic of Congo, there is a low evocation of the diagnosis of sickle cell disease (SCD) by first-level healthcare providers (HCPs), most likely because of poor knowledge of the disease. AIM:  To assess the levels of knowledge and practices of SCD and to identify determinants of the practices among primary HCPs. SETTING:  Healthcare facilities in Selembao health zone in Kinshasa, Democratic Republic of the Congo. METHODS:  A cross-sectional study of HCPs randomly selected through a two-stage sampling design. Data were collected using a pre-tested interviewer-administered questionnaire. Univariate and bivariate analysis were done to describe the levels of knowledge and practices of SCD. Factors associated with better practices on SCD were determined using multiple linear regression. The threshold for statistical significance was p Ë‚ 0.05. RESULTS:  A total of 318 HCPs, which included 80 physicians and 238 nurses, participated in the study. The participants showed different scores on the components of the knowledge. All the participants showed poor practices on SCD. Multiple linear regression retained overall knowledge of SCD as a significant predictor of better practice for physicians. Knowledge of SCD and duration of work experience were significant predictors of better practices among nurses. CONCLUSION:  The practices of healthcare providers on SCD were far from optimal. These practices were significantly associated with knowledge and experience of healthcare providers.Contribution: This study highlighted the need for continuing professional education to enhance the management of SCD in the setting.


Assuntos
Anemia Falciforme , Médicos , Humanos , República Democrática do Congo , Estudos Transversais , Pessoal de Saúde , Anemia Falciforme/terapia
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