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1.
Niger J Clin Pract ; 24(7): 1044-1051, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34290181

RESUMO

Background: Body mass index is a major determinant of cardiac annular valvar dimension and left ventricular mass index in children with sickle cell anemia. Objectives: The study is aimed at ascertaining the impact of Body Mass Index on Left ventricular mass index, right ventricular function and cardiac dimension of children with sickle cell anemia. Methods: A case control study in which echocardiographic measurement of cardiac function and structures were ascertained among children with sickle cell anemia compared with hemoglobin AA genotype. Results: There were 51 subjects and 50 controls. The subjects comprised 54.9% males and controls, 52.0% male. There was a strong positive correlation between BMI and most cardiac structure diameters among children with normal hemoglobin genotype (Pearson's correlation coefficient value, P < 0.001) There was also statistically significant positive correlation between BMI and LV mass among the subjects (n = 50, r = 0.5, P < 0.001). There was significant positive correlation between BMI and TAPSE in both subjects and controls as well as between BMI and RVSP among the subjects, but not the controls (p < 0.001). There was no significant difference in the number with left ventricular hypertrophy (LVH) based on their nutritional status (n = 51, χ^2 = 7.03, P = 0.32). The BMI correlated negatively with left ventricular mass index (LVMI) among the subjects, but the correlation was not statistically significant (r = -0.1, P = 0.53). Conclusion: There was significant positive correlation between BMI and TAPSE in both subjects and controls as well as between BMI and RVSP among the subjects, but not the controls. Body mass index correlated negatively with left ventricular mass index (LVMI) among the subjects.


Assuntos
Anemia Falciforme , Função Ventricular Direita , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Índice de Massa Corporal , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Nigéria
2.
Emerg Med Clin North Am ; 39(3): 555-571, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34215402

RESUMO

Pediatric hematologic and oncologic emergencies are in 3 major categories: complications of hematologic disorders, emergencies associated with the new onset of cancers, and treatment-associated oncologic emergencies. The overall number of these patients remains low; however, the mortality associated with these diseases remains high despite significant advances in management. This article presents a review of the most commonly encountered pediatric hematologic and oncologic complications that emergency physicians and providers need to know.


Assuntos
Anemia Falciforme , Antineoplásicos/efeitos adversos , Neoplasias , Púrpura Trombocitopênica Idiopática , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Criança , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/diagnóstico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/terapia , Humanos , Incidência , Neoplasias/diagnóstico , Neoplasias/epidemiologia , Neoplasias/terapia , Medicina de Emergência Pediátrica , Prevalência , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia
3.
Blood Adv ; 5(13): 2717-2724, 2021 07 13.
Artigo em Inglês | MEDLINE | ID: mdl-34196678

RESUMO

Patients with sickle cell disease (SCD) are at high risk of developing serious infections, therefore, understanding the impact that severe acute respiratory syndrome coronavirus 2 infection has on this population is important. We sought to identify factors associated with hospitalization and serious COVID-19 illness in children and adults with SCD.We established the international SECURE-SCD Registry to collect data on patients with SCD and COVID-19 illness. We used multivariable logistic models to estimate the independent effects of age, sex, genotype, hydroxyurea, and SCD-related and -nonrelated comorbidities on hospitalization, serious COVID-19 illness, and pain as a presenting symptom during COVID-19 illness. As of 23 March 2021, 750 COVID-19 illness cases in patients with SCD were reported to the registry. We identified history of pain (relative risk [RR], 2.15; P < .0001) and SCD heart/lung comorbidities (RR, 1.61; P = .0001) as risk factors for hospitalization in children. History of pain (RR, 1.78; P = .002) was also a risk factor for hospitalization in adults. Children with history of pain (RR, 3.09; P = .009), SCD heart/lung comorbidities (RR, 1.76; P = .03), and SCD renal comorbidities (RR, 3.67; P < .0001) and adults with history of pain (RR 1.94, P = .02) were at higher risk of developing serious COVID-19 illness. History of pain and SCD renal comorbidities also increased risk of pain during COVID-19 in children; history of pain, SCD heart/lung comorbidities, and female sex increased risk of pain during COVID-19 in adults. Hydroxyurea showed no effect on hospitalization and COVID-19 severity, but it lowered the risk of presenting with pain in adults during COVID-19.


Assuntos
Anemia Falciforme , COVID-19 , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Criança , Feminino , Hospitalização , Humanos , Fatores de Risco , SARS-CoV-2
5.
Niger J Clin Pract ; 24(6): 874-882, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34121736

RESUMO

Background: Sickle cell disease (SCD) is a monogenic, phenotypically highly variable disease with multisystem pathology. The phenotypic heterogeneity of SCD is attributed to environmental and genetic factors such as fetal hemoglobin and co-inheritance of α-thalassemia. Objectives: To look for different types of α-thalassemia gene mutations among SCD patients and evaluate the influence of the co-inheritance of α-thalassemia on clinical and hematological variables. Methods: This cross-sectional analytical study included 765 SCD patients, and 150 patients (with low mean corpuscular volume (MCV), low mean corpuscular hemoglobin (MCH) and normal serum ferritin levels) were tested for α-thalassemia gene mutations. Multiplex PCR and reverse hybridization and sequencing for both α genes using the Vienna Lab Strip Assay PCR study were performed using conventional PCR technology. Results: Out of 150 patients tested for α-thalassemia gene mutations, 141 patients were found to have one or more of the mutational types, representing 18.4% of all studied SCD patients. The most common mutations found were the -3.7 deletion (76.6%), followed by the -4.2 deletion (12.1%), mutant α2polyA-1 (Saudi type) (9.2%), and --MED double gene deletion (7.8%). Acute painful episodes did not differ significantly in sickle cell anemia (SCA) patients with or without α-thalassemia, although the co-inheritance of α-thalassemia has a protective role against many disease-related complications. However, this role was not observed with other types of SCD. The means of red blood cell count, hemoglobin, and hematocrit were significantly higher, while the MCV, MCH, reticulocyte count, and hemoglobin A2 percentage were significantly lower in patients with α-thalassemia gene mutations than in those without α-thalassemia gene mutations (P < 0.05). Conclusions: The co-inheritance of α-thalassemia and SCA confers protection against many disease-related complications and is associated with improved hematological indices. However, this protection was not noticed in patients with other types of SCD.


Assuntos
Anemia Falciforme , Talassemia alfa , Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Estudos Transversais , Índices de Eritrócitos , Humanos , Mutação , Talassemia alfa/epidemiologia , Talassemia alfa/genética
6.
Artigo em Inglês | MEDLINE | ID: mdl-34069401

RESUMO

BACKGROUND: Sickle cell anemia (SCA) is an inherited blood disorder that affects over 300,000 newborns worldwide every year, being particularly prevalent in Sub-Saharan Africa. Despite being a monogenic disease, SCA shows a remarkably high clinical heterogeneity. Several studies have already demonstrated the existence of some polymorphisms that can provide major clinical benefits, producing a mild phenotype. Moreover, the existence of distinct haplotypes can also influence the phenotype patterns of certain populations, leading to different clinical manifestations. Our aim was to assess the association between polymorphisms in genes previously related to SCA disease severity in an Angolan pediatric population. METHODS: This study analyzed clinical and biological data collected from 192 Angolan children. Using NGS data, we classified the HBB haplotypes based on four previously described SNPs (rs3834466, rs28440105, rs10128556, and rs968857) and the genotype for the SNPs in HBG2 (rs7482144), BCL11A (rs4671393, rs11886868, rs1427407, rs7557939), HBS1L-MYB (rs66650371) and BGLT3 (rs7924684) genes. RESULTS: The CAR haplotype was undoubtedly the most common HBB haplotype in our population. The HbF values and the ratio of gamma chains were statistically significant for almost all of the variants studied. We reported for the first time an association between rs7924684 in the BGLT3 gene and gamma chains ratio. CONCLUSIONS: The current findings emphasize the importance personalized medicine would have if applied to SCA patient care, since some of the variants studied might predict the phenotype and the overall response to treatment.


Assuntos
Anemia Falciforme , Hemoglobina Fetal , Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Criança , Hemoglobina Fetal/análise , Hemoglobina Fetal/genética , Genótipo , Haplótipos , Humanos , Recém-Nascido , Polimorfismo de Nucleotídeo Único , Proteínas Repressoras/genética
7.
J Clin Pediatr Dent ; 45(2): 117-122, 2021 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-33951171

RESUMO

OBJECTIVE: To identify the association of occlusal disorders in patients with sickle cell disease (SCD). STUDY DESIGN: A literature review was conducted, and articles published between 2010 and 2019 were searched on Bireme and PubMed websites and in MEDLINE and LILACS databases, in English, Portuguese, and Spanish, using the keywords "malocclusion," "sickle cell disease," and "cephalometry," combined by Boolean operators AND and OR. One of the criteria for the selection of articles was the presence of adolescents in the sample. This methodology followed the PRISMA recommendations. Seventy-nine articles were found, seven of which were included in the review as they met the inclusion criteria and the study goals. RESULTS: The prevalence of malocclusion in SCD patients ranged from 62.9% to 100%, which was considered very severe in 30.1% to 80.6%. The most common occlusal changes were Angle's class II malocclusion, increased maxillary overjet, and anterior open bite. In addition, class II skeletal pattern was the most prevalent due to mandibular retrusion. CONCLUSION: Malocclusion prevalence in SCD patients is high and considered to be a risk factor, with a significant rate of very severe malocclusion when compared to healthy patients.


Assuntos
Anemia Falciforme , Má Oclusão Classe II de Angle , Má Oclusão , Mordida Aberta , Sobremordida , Adolescente , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Cefalometria , Humanos , Má Oclusão/epidemiologia
8.
BMC Health Serv Res ; 21(1): 294, 2021 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-33794895

RESUMO

BACKGROUND: Sickle cell disease (SCD) is a public health problem in the Democratic Republic of Congo. While reference sickle cell centers have been implemented in capital cities of African countries and have proven to be beneficial for SCD patients. In the Democratic Republic of Congo, they have never been set up in remote areas for families with low or very low sources of income. METHOD: A cohort of 143 children with SCD aged 10 years old (IQR (interquartile range): 6-15 years) (sex ratio male/female = 1.3) were clinically followed for 12 months without any specific intervention aside from the management of acute events, and then for 12 months with a monthly medical visit, biological follow-up, and chemoprophylaxis (folic acid/penicillin), adequate fluids and malaria prevention. RESULTS: The median age of patients at the diagnosis of SCD was 2 years (IQR: 1-5). The implementation of standardized and regular follow-ups in a new sickle cell reference center in a remote city showed an increase in the annual mean hemoglobin level from 50 to 70 g/L (p = 0.001), and a decrease in the lymphocyte count and spleen size (p < 0.001). A significant decrease (p < 0.001) in the average annual number of hospitalizations and episodes of vaso-occlusive crises, blood transfusions, infections, and acute chest syndromes were also observed. CONCLUSIONS: The creation of a sickle cell reference center and the regular follow-up of children with sickle cell disease are possible and applicable in the context of a remote city of an African country and represent simple and accessible measures that can reduce the morbimortality of children with sickle cell disease.


Assuntos
Anemia Falciforme , África , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Transfusão de Sangue , Criança , Pré-Escolar , Congo/epidemiologia , Feminino , Hospitalização , Humanos , Lactente , Masculino
9.
BMC Psychol ; 9(1): 54, 2021 Apr 06.
Artigo em Inglês | MEDLINE | ID: mdl-33823930

RESUMO

BACKGROUND: Sickle cell disease (SCD) as other chronic medical conditions is commonly complicated by depression or other psychiatric symptoms. Results reported in studies present a large variation. Thus, synthetic data are needed to understand impact of depression in adults with SCD. The aim of this literature review is to analyse the methodology used in the studies assessing depression and discuss the different prevalence levels reported. METHODS: Studies involving adults with SCD from 1999 to 2018 were included when providing data on prevalence of depression. It was defined by a psychometric assessment, a structured interview, or a medical record review. PRISMA recommendations were followed. RESULTS: 36 studies are included accordingly to our methodology. Prevalence variation is large, from 0% to more than 85%. We find that the type of assessment tool used plays a major role in this between studies variation. Also, methodological issues arise with respect to psychometric assessment. Moreover, differences emerge between continents, setting of recruitment or time of assessment. CONCLUSION: All these issues are discussed to provide insight on depression in adults with sickle cell disease. TRIAL REGISTRATION: PROSPERO Registration CRD42018100684.


Assuntos
Anemia Falciforme , Depressão , Adulto , Anemia Falciforme/epidemiologia , Depressão/epidemiologia , Humanos , Prevalência , Psicometria
10.
Rev. cuba. med ; 60(1): e505, graf
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1156561

RESUMO

Introducción: La enfermedad por hemoglobina S es una anemia hemolítica crónica hereditaria cuyas manifestaciones clínicas provienen de la tendencia de esta hemoglobina de polimerizar y deformar los eritrocitos dándoles la típica forma de media luna, platanito, drepanocitos o sickle cell; de aquí el nombre de anemia drepanocítica o sicklemia. Objetivo: Describir los nuevos aspectos moleculares, fisiopatológicos y el diagnóstico de la anemia drepanocítica. Métodos: Se realizó una revisión de la literatura, en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google académico de artículos publicados en los últimos 10 años. Se hizo un análisis y resumen de la bibliografía revisada. Conclusiones: La comprensión de la complejidad y multiplicidad de eventos que conducen a complicaciones graves en la anemia drepanocítica y nuestra incapacidad para predecir el curso clínico en cada caso particular ayudaría en la prevención de estos eventos(AU)


Introduction: Hemoglobin S disease is a hereditary chronic hemolytic anemia whose clinical manifestations come from the tendency of this hemoglobin to polymerize and deform erythrocytes, giving the typical crescent, banana, sickle cell or sickle cell shape; hence the name sickle cell anemia or sicklemia. Objective: To describe the new molecular and pathophysiological aspects and the diagnosis of sickle cell anemia. Methods: A literature review was carried out, in English and Spanish, through PubMed website and Google academic search engine for articles published in the last 10 years. An analysis and summary of the revised bibliography was made. Conclusions: Understanding the complexity and multiplicity of events that lead to serious complications in sickle cell anemia and our inability to predict the clinical course in each particular case would help preventing these events(AU)


Assuntos
Humanos , Hemoglobinopatias , Anemia Falciforme/epidemiologia
12.
JBI Evid Synth ; 19(3): 682-688, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33725714

RESUMO

OBJECTIVE: To assess psychosocial risk factors for increased emergency hospital utilization by sickle cell patients. INTRODUCTION: Emergency hospital utilization by sickle cell disease patients is high but heterogeneous between patients and in a given patient over time. Psychosocial factors affect emergency hospital utilization and are a possible target to improve the management of sickle cell disease. INCLUSION CRITERIA: This review will include all original quantitative studies evaluating the impact of psychosocial risk factors on emergency hospital utilization by sickle cell disease patients. There will be no language restriction. METHODS: PubMed, Embase, CINAHL, PubPsych, LiSSa, and Web of Science will be searched using a peer-reviewed search strategy. Study selection and extraction of data will be performed independently by two authors. Discrepancies will be solved by consensus or, if needed, by a third author. The authors will assess study quality, as well as perform a narrative synthesis of included studies, and where possible, meta-analyses with evaluation of heterogeneity and publication bias. SYSTEMATIC REVIEW REGISTRATION NUMBER: PROSPERO CRD42019140435.


Assuntos
Anemia Falciforme , Anemia Falciforme/epidemiologia , Serviço Hospitalar de Emergência , Hospitais , Humanos , Projetos de Pesquisa , Literatura de Revisão como Assunto , Fatores de Risco , Revisões Sistemáticas como Assunto
13.
Int J Infect Dis ; 106: 128-133, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33741487

RESUMO

OBJECTIVES: The study aimed to assess COVID-19 impact on the morbidity and mortality of vasooclusive crisis (VOC) in sickle cell anaemia (SCA) patients. METHODS: A prospective cohort study of 100 SCA patients; 50 with COVID-19 (COVID group) and 50 without (non-COVID group). All patients signed written informed consent. RESULTS: The COVID group had a significantly higher VOC episode median per year; 3 (IQR,1-6) vs 2 (IQR,2-12) (P < 0.05). The need for hospitalisation was similar in both groups. The non-COVID group had more history of culture-proven infection (P = 0.05). The COVID-group had more osteonecrosis (P < 0.05), splenic sequestration, splenomegaly and hepatic crisis (P = 0.05, 0.006, 0.02; respectively) and significantly higher (P < 0.05) symptoms of fever, cough, fatigue, abdominal pain and anosmia. Mean haemoglobin, lymphocyte subset, platelets, and reticulocytes were reduced in both groups, while lactate dehydrogenase and ferritin levels were significantly elevated. In the COVID group, the rise in white blood cell count, reticulocyte percentage, platelets and ferritin was subdued (P < 0.05). Two patients in the COVID group and 3 in the non-COVID group died; there was no statistically significant difference in mortality. CONCLUSIONS: Although COVID-19 may have triggered the onset of VOC, it did not significantly influence VOC-related morbidity or mortality in this SCA cohort.


Assuntos
Síndrome Torácica Aguda/sangue , Síndrome Torácica Aguda/epidemiologia , Anemia Falciforme/sangue , Anemia Falciforme/epidemiologia , COVID-19/sangue , COVID-19/epidemiologia , SARS-CoV-2 , Síndrome Torácica Aguda/mortalidade , Adulto , Anemia Falciforme/mortalidade , COVID-19/mortalidade , Estudos de Coortes , Comorbidade , Feminino , Ferritinas/sangue , Hospitalização , Humanos , L-Lactato Desidrogenase/sangue , Contagem de Leucócitos , Contagem de Linfócitos , Masculino , Contagem de Plaquetas , Estudos Prospectivos , Reticulócitos
14.
BMC Health Serv Res ; 21(1): 229, 2021 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-33712013

RESUMO

BACKGROUND: In well-resourced countries, comprehensive care programs have increased life expectancy of patients with sickle cell disease, with almost all infants surviving into adulthood. However, families affected by sickle cell disease are more likely to be economically disenfranchised because of their racial or ethnic minority status. As every individual child has the right to the highest attainable standard of health under the United Nations Convention on the Rights of the Child, it is essential to identify both barriers and facilitators with regard to the delivery of adequate healthcare. Optimal healthcare accessibility will improve healthcare outcomes for children with sickle cell disease and their families. Healthcare professionals in the field of sickle cell care have first-hand experience of the barriers that patients encounter when it comes to effective care. We therefore hypothesised that these medical professionals have a clear picture of what is necessary to overcome these barriers and which facilitators will be most feasible. Therefore, this study aims to map best practises and lessons learnt in order to attain more optimal healthcare accessibility for paediatric patients with sickle cell disease and their families. METHODS: Healthcare professionals working with young patients with sickle cell disease were recruited for semi-structured interviews. An interview guide was used to ensure the four healthcare accessibility dimensions were covered. The interviews were transcribed and coded. Based on field notes, initial codes were generated, to collate data (both barriers and solutions) to main themes (such as "transportation", or "telecommunication"). Through ongoing thematic analysis, definitive themes were formulated and best practices were reported as recommendations. Quotations were selected to highlight or illustrate the themes and link the reported results to the empirical data. RESULTS: In 2019, 22 healthcare professionals from five different university hospitals in the Netherlands were interviewed. Participants included (paediatric) haematologists, nurses and allied health professionals. Six themes emerged, all associated with best practices on topics related to the improvement of healthcare accessibility for children with sickle cell disease and their families. Firstly, the full reimbursement of invisible costs made by caregivers. Secondly, clustering of healthcare appointments on the same day to help patients seeing all required specialists without having to visit the hospital frequently. Thirdly, organisation of care according to shared care principles to deliver specialised services as close as possible to the patient's home without compromising quality. Fourthly, optimising verbal and written communication methods with special consideration for families with language barriers, low literacy skills, or both. Fifthly, improving the use of eHealth services tailored to users' health literacy skills, including accessible mobile telephone contact between healthcare professionals and caregivers of children with sickle cell disease. Finally, increasing knowledge and interest in sickle cell disease among key stakeholders and the public to ensure that preventive and acute healthcare measures are understood and safeguarded in all settings. CONCLUSION: This qualitative study describes the views of healthcare professionals on overcoming barriers of healthcare accessibility that arise from the intersecting vulnerabilities faced by patients with sickle cell disease and their families. The recommendations gathered in this report provide high-income countries with a practical resource to meet their obligations towards individual children under the United Nations Convention on the Rights of the Child.


Assuntos
Anemia Falciforme , Grupos Étnicos , Adulto , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Criança , Atenção à Saúde , Humanos , Lactente , Grupos Minoritários , Países Baixos
15.
Haematologica ; 106(6): 1535-1544, 2021 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-33626864

RESUMO

Median life expectancy of patients with sickle cell disease has increased to up to 55 years but there are still frequent cases of premature death, mostly in patients with pre-existing organ failure such as pulmonary hypertension, kidney injury, and cerebral vasculopathy. Most organ injuries remain asymptomatic for a long time and can only be detected through early systematic screening. Protocols combining assessment of velocities on transcranial Doppler and regular transfusions in patients with abnormal velocities have been demonstrated to dramatically reduce the risk of stroke. In contrast, no consensus has been reached on systematic screening or therapy for silent cerebral infarcts. The prognostic significance of increased tricuspid regurgitant jet velocity on echocardiography has not yet been identified in children, whereas increased albuminuria is a good predictor of kidney injury. Finally, screening for hip and eye disorder is recommended; however, different countries adopt different screening strategies. Hydroxyurea is probably of potential benefit in preventing chronic organ damage but this requires further study in order to be fully demonstrated. Efficacy and safety of the other new drugs available are also under investigation.


Assuntos
Anemia Falciforme , Acidente Vascular Cerebral , Anemia Falciforme/complicações , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Antidrepanocíticos , Transfusão de Sangue , Criança , Humanos , Hidroxiureia
16.
Curr Pain Headache Rep ; 25(3): 19, 2021 Feb 25.
Artigo em Inglês | MEDLINE | ID: mdl-33630183

RESUMO

PURPOSE OF REVIEW: This review provides an updated discussion on the clinical presentation, diagnosis and radiographic features, mechanisms, associations and epidemiology, treatment, and prognosis of posterior reversible encephalopathy syndrome (PRES). Headache is common in PRES, though headache associated with PRES was not identified as a separate entity in the 2018 International Classification of Headache Disorders. Here, we review the relevant literature and suggest criteria for consideration of its inclusion. RECENT FINDINGS: COVID-19 has been identified as a potential risk factor for PRES, with a prevalence of 1-4% in patients with SARS-CoV-2 infection undergoing neuroimaging, thus making a discussion of its identification and treatment particularly timely given the ongoing global pandemic at the time of this writing. PRES is a neuro-clinical syndrome with specific imaging findings. The clinical manifestations of PRES include headache, seizures, encephalopathy, visual disturbances, and focal neurologic deficits. Associations with PRES include renal failure, preeclampsia and eclampsia, autoimmune conditions, and immunosuppression. PRES is theorized to be a syndrome of disordered autoregulation and endothelial dysfunction resulting in preferential hyperperfusion of the posterior circulation. Treatment typically focuses on treating the underlying cause and removal of the offending agents.


Assuntos
Endotélio/fisiopatologia , Cefaleia/fisiopatologia , Síndrome da Leucoencefalopatia Posterior/fisiopatologia , Convulsões/fisiopatologia , Transtornos da Visão/fisiopatologia , Síndrome Torácica Aguda/epidemiologia , Ácido Aminolevulínico/análogos & derivados , Anemia Falciforme/epidemiologia , Doenças Autoimunes/epidemiologia , Barreira Hematoencefálica/metabolismo , Edema Encefálico/diagnóstico por imagem , Edema Encefálico/fisiopatologia , COVID-19/epidemiologia , Circulação Cerebrovascular/fisiologia , Citocinas/metabolismo , Eclampsia/epidemiologia , Feminino , Homeostase/fisiologia , Humanos , Hipertensão/fisiopatologia , Imageamento por Ressonância Magnética , Síndrome da Leucoencefalopatia Posterior/diagnóstico por imagem , Síndrome da Leucoencefalopatia Posterior/epidemiologia , Síndrome da Leucoencefalopatia Posterior/terapia , Pré-Eclâmpsia/epidemiologia , Gravidez , Prognóstico , Insuficiência Renal/epidemiologia , SARS-CoV-2 , Vasoespasmo Intracraniano/fisiopatologia
17.
BMJ Glob Health ; 6(2)2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33602689

RESUMO

INTRODUCTION: Sickle cell disease (SCD) disproportionately impacts Adivasi (tribal) communities in India. Current research has focused on epidemiological and biomedical aspects but there has been scarce research on social determinants and health systems aspects. Given its fragmented distribution, resources and programmes have emerged in west and central India. This scoping review seeks to identify geographical and evidence gaps for action on SCD from a health systems lens. METHODS: We followed a scoping review protocol, using Google Scholar and PubMed for published literature. Keywords used included sickle cell anaemia/disease, health systems, tribal and India. We used Google search for grey literature. We compiled a list of 55 records (of which 35 were retained), with about half pertaining directly to India and others relevant to similar settings. Results were organised and analysed using the WHO health systems framework to identify geographical and evidence gaps. RESULTS: We found substantial literature on biomedical and clinical aspects of SCD but little on the design and implementation of programmes in community and Adivasi-specific contexts as well as on social determinants of SCD. There were regional gaps in knowledge in southern and northeast India. Wherever community-based programmes exist, they have originated in civil society initiatives and relatively limited state-led primary healthcare-based efforts pointing to weak agenda setting. CONCLUSION: Both research and action on SCD especially among tribal populations need immediate attention. While geospatial epidemiology has been well understood, gaps remain in context-specific knowledge for action in several parts, as well as evidence gaps across several health system building blocks, including governance and financing of care. Despite publication of a draft policy, delayed adoption and lapses in implementation have limited the response largely to local communities and non-governmental organisations.


Assuntos
Anemia Falciforme , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Programas Governamentais , Humanos , Índia/epidemiologia
18.
Hematology ; 26(1): 199-205, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33594960

RESUMO

BACKGROUND: The Democratic Republic of the Congo (DRC) is the third most affected country worldwide by sickle cell disease (SCD). However, this disease is still orphaned in the country; large-scale control actions are rare, and little is known about its management. OBJECTIVE: To assess current practices in the management of SCD in Kisangani, DRC. METHODS: This cross-sectional study was conducted in six health facilities in Kisangani. It involved 198 presumed sickle cell patients attending the above health facilities. The study focused on the sociodemographic and clinical data of the participants, obtained through a clinical examination and their medical records. Diagnostic confirmation of SCD was made by high-performance liquid chromatography coupled to mass spectrometry. Data were analyzed using SPSS 20.0. RESULTS: The diagnosis of SCD was confirmed in 194 (98.0%; 95% CI: 94.9-99.2) participants, while it was not confirmed in 4 (2.0%; 95% CI: 0.8-5.1) participants. The diagnosis was mainly made by the Emmel test (42.9%). 45.8% of participants had previously been transfused with the blood of their parents. Folic acid was taken by 48.5% of participants and the previous intake of hydroxyurea was reported in 5.1% of participants. The participants vaccinated against Pneumococcus were 13.6% and against Haemophilus influenzae type b 28.3%. Penicillin prophylaxis was received by only 1.5% and malaria prophylaxis by 11.6% of participants. CONCLUSION: Standard-care practices for SCD patients in Kisangani are insufficient. The Congolese government should regard this disease as a health priority and consider actions to improve its management.


Assuntos
Anemia Falciforme/epidemiologia , Padrões de Prática Médica , Adolescente , Adulto , Idoso , Anemia Falciforme/diagnóstico , Anemia Falciforme/etiologia , Anemia Falciforme/terapia , Biomarcadores , Criança , Pré-Escolar , Cromatografia Líquida de Alta Pressão , Tomada de Decisão Clínica , Comorbidade , Estudos Transversais , República Democrática do Congo/epidemiologia , Gerenciamento Clínico , Feminino , Pesquisas sobre Serviços de Saúde , Humanos , Masculino , Espectrometria de Massas , Pessoa de Meia-Idade , Prevalência , Inquéritos e Questionários , Adulto Jovem
19.
Br Dent J ; 230(3): 143-147, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33574535

RESUMO

Sickle cell disease is a multisystem disease associated with episodes of acute illness and progressive organ damage. It is one of the most common severe monogenic disorders worldwide and is now the fastest growing genetic disorder in the UK. Dental awareness of orofacial manifestation varies geographically due to the inheritance pattern and patient population affected by the disease. This article details the epidemiology, pathophysiology and the orofacial manifestations in light of current literature.


Assuntos
Anemia Falciforme , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Humanos
20.
BMC Pulm Med ; 21(1): 46, 2021 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-33514354

RESUMO

BACKGROUND: Sickle-cell anaemia is a widespread genetic disease prevalent worldwide among African and African-American populations. The pathogenesis is most often revealed by pulmonary conditions, including acute thoracic syndrome, which is affecting the life expectancy of these populations. The global spread of CoV2-SARS infection with a respiratory tropism, endothelial damages and procoagulant status endangers the SCD population. However, with only a few case reports, consequences of the Covid-19 pandemic on SCD population remain poorly known. CASE PRESENTATION: We report a case of a 33-year-old man with a history of homozygous SS homozygous sickle cell anemia who consulted on March 24, 2020 for febrile dyspnea 11 days after the onset of symptoms. A nasopharyngeal swab was positive for SARS-CoV-2. His respiratory status worsened rapidly in the emergency room and then in ICU leading to severe ARDS requiring intubation, curarization, and venovenous ECMO. Hematologically, severe hemolysis associated with major thrombocytopenia without documented spinal cord injury was noted. Several transfusion exchanges are performed. The evolution was finally slowly favorable and led to discharge from the intensive care unit and then from the hospital. CONCLUSIONS: This case recalls the importance of an increased prevention policy against COVID-19among the SCD population. In addition, from a therapeutic point of view, it advocates (1) a high preventive anticoagulation from the outset according to the level of D-dimers (2) the use of venovenous ECMO in this particular case, whereas this technique has had rather disappointing results in acute chest syndromes. (3) Unexpectedly, our patient did not develop pulmonary arterial hypertension (PAH) and acute cor pulmonale (ACP), whereas this is a common feature of ARDS during SCD. These last two observations suggest a different pathophysiology of pulmonary disorders in SCD patients in the case of SARS COv2. It could be associated with marked hypoxemia secondary to pulmonary vascular vasodilation.


Assuntos
Anemia Falciforme/epidemiologia , COVID-19/epidemiologia , Oxigenação por Membrana Extracorpórea/métodos , Respiração Artificial/métodos , Síndrome do Desconforto Respiratório/etiologia , SARS-CoV-2 , Adulto , Anemia Falciforme/terapia , COVID-19/complicações , COVID-19/terapia , Humanos , Masculino , Pandemias , Radiografia Torácica , Síndrome do Desconforto Respiratório/diagnóstico , Síndrome do Desconforto Respiratório/terapia
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