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1.
Estima (Online) ; 19(1): e0321, jan.-dez. 2021.
Artigo em Português | BDENF - Enfermagem | ID: biblio-1178276

RESUMO

Objetivo:caracterizar o perfil de mulheres com úlceras da perna decorrentes da doença falciforme atendidas em uma instituição pública de saúde do Brasil. Método: estudo de coorte histórica realizado no setor ambulatorial com amostra de mulheres com doença falciforme e úlcera da perna, acompanhadas de 1998 a 2014. Os dados foram extraídos do prontuário e contemplaram variáveis sociodemográficas, clínicas e relacionadas à úlcera, e passaram por análise estatística descritiva. Resultados: das 17 mulheres, 64,7% obtiveram cura, 58,8% tinham idade entre 30 e 39 anos, 64,7% se autodeclaram pardas, 70,6% estavam sem parceiros, 41,2% possuíam ensino fundamental incompleto, 52,9% tinham renda de 2 a 3 salários mínimos, 70,6% eram eutróficas e 82,4% faziam uso de suplementos vitamínicos. As mulheres apresentaram o total de 29 úlceras, 52,9% tinham úlcera única, 41,4% localizadas na região maleolar, 55,17% com existência entre 2 e 36 meses, todas classificadas em estágio 3. O tratamento foi com coberturas e bota de Unna. Conclusão: as mulheres receberam cuidados de enfermeiros especialistas e a maioria obteve a cura com até oito meses de acompanhamento que demandou a consulta de enfermagem, contemplando a avaliação clínica, o tratamento tópico e aplicação da terapia de compressão.


Assuntos
Perfil de Saúde , Anemia Falciforme , Úlcera da Perna
2.
BMC Infect Dis ; 21(1): 1028, 2021 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-34592961

RESUMO

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.


Assuntos
Anemia Falciforme , Infecções por HIV , Hepatite B , Adolescente , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Estudos Transversais , Feminino , Infecções por HIV/complicações , Infecções por HIV/epidemiologia , Hepatite B/epidemiologia , Vírus da Hepatite B , Humanos , Masculino , Prevalência , Tanzânia/epidemiologia , Adulto Jovem
3.
J Med Case Rep ; 15(1): 504, 2021 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-34625105

RESUMO

BACKGROUND: The occurrence of acute leukemia in patients with sickle cell anemia is uncommon. The Philadelphia chromosome is the hallmark of chronic myeloid leukemia. However, it may also be associated with acute lymphoblastic leukemia and acute myeloblastic leukemia. The common BCR-ABL1 transcripts seen in acute lymphoblastic leukemia are e1a2, e13a2, and e14a2, while other transcripts such as e1a3, e13a3, and e6a2 occur rarely. This report describes the presentation, management, and outcome of the occurrence of B-cell acute lymphoblastic leukemia with the rare e1a3 BCR-ABL1 transcript in a patient with sickle cell anemia. CASE PRESENTATION: A 19-year-old male Nigerian, a known sickle cell anemia patient was admitted on account of severe vaso-occlusive crisis. Examination revealed fever, palor, and jaundice. Full blood count showed anemia and leukocytosis. Peripheral blood and bone marrow smears revealed numerous large and small lymphoblasts in keeping with the L2 subtype of acute lymphoblastic leukemia based on the French-American-British classification. Further evaluation was in keeping with a diagnosis of BCR-ABL1-positive mature B-cell acute lymphoblastic leukemia associated with the rare e1a3 transcript. He was commenced simultaneously on induction chemotherapy and Imatinib while being prepared for allogeneic stem cell transplantation. However, he died  six  months after diagnosis from meningoencephalitis. CONCLUSION: The occurrence of acute lymphoblastic leukemia with a rare BCR-ABL1 e1a3 transcript in association with sickle cell anemia is uncommon and associated with poor prognosis.


Assuntos
Anemia Falciforme , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/genética , Linfócitos B , Proteínas de Fusão bcr-abl/genética , Humanos , Masculino , Nigéria , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Adulto Jovem
4.
Trials ; 22(1): 690, 2021 Oct 10.
Artigo em Inglês | MEDLINE | ID: mdl-34629091

RESUMO

BACKGROUND: Young children with sickle cell anaemia (SCA) often have slowed processing speed associated with reduced brain white matter integrity, low oxygen saturation, and sleep-disordered breathing (SDB), related in part to enlarged adenoids and tonsils. Common treatments for SDB include adenotonsillectomy and nocturnal continuous positive airway pressure (CPAP), but adenotonsillectomy is an invasive surgical procedure, and CPAP is rarely well-tolerated. Further, there is no current consensus on the ability of these treatments to improve cognitive function. Several double-blind, randomised controlled trials (RCTs) have demonstrated the efficacy of montelukast, a safe, well-tolerated anti-inflammatory agent, as a treatment for airway obstruction and reducing adenoid size for children who do not have SCA. However, we do not yet know whether montelukast reduces adenoid size and improves cognition function in young children with SCA. METHODS: The Study of Montelukast In Children with Sickle Cell Disease (SMILES) is a 12-week multicentre, double-blind, RCT. SMILES aims to recruit 200 paediatric patients with SCA and SDB aged 3-7.99 years to assess the extent to which montelukast can improve cognitive function (i.e. processing speed) and sleep and reduce adenoidal size and white matter damage compared to placebo. Patients will be randomised to either montelukast or placebo for 12 weeks. The primary objective of the SMILES trial is to assess the effect of montelukast on processing speed in young children with SCA. At baseline and post-treatment, we will administer a cognitive evaluation; caregivers will complete questionnaires (e.g. sleep, pain) and measures of demographics. Laboratory values will be obtained from medical records collected as part of standard care. If a family agrees, patients will undergo brain MRIs for adenoid size and other structural and haemodynamic quantitative measures at baseline and post-treatment, and we will obtain overnight oximetry. DISCUSSION: Findings from this study will increase our understanding of whether montelukast is an effective treatment for young children with SCA. Using cognitive testing and MRI, the SMILES trial hopes to gain critical knowledge to help develop targeted interventions to improve the outcomes of young children with SCA. TRIAL REGISTRATION: ClinicalTrials.gov NCT04351698 . Registered on April 17, 2020. European Clinical Trials Database (EudraCT No. 2017-004539-36). Registered on May 19, 2020.


Assuntos
Anemia Falciforme , Quinolinas , Acetatos/efeitos adversos , Anemia Falciforme/diagnóstico , Anemia Falciforme/tratamento farmacológico , Anti-Inflamatórios , Criança , Pré-Escolar , Ciclopropanos , Humanos , Quinolinas/efeitos adversos , Ensaios Clínicos Controlados Aleatórios como Assunto , Sulfetos
5.
Emerg Med Clin North Am ; 39(4): 839-850, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34600641

RESUMO

Abdominal pain is a common reason for emergency department visits, with many patients not receiving a definitive diagnosis for their symptoms. Non-gastrointestinal causes need to be considered in the workup of abdominal pain. A high index of suspicion is needed in order to develop a broad differential, and a thorough history and physical examination is paramount. This article will discuss some of these diagnoses, including can't miss diagnoses, common non-abdominal causes, and rare etiologies of abdominal pain.


Assuntos
Dor Abdominal/etiologia , Síndrome Coronariana Aguda/diagnóstico , Doenças das Glândulas Suprarrenais/diagnóstico , Anemia Falciforme/diagnóstico , Angioedemas Hereditários/diagnóstico , Doenças da Aorta/diagnóstico , COVID-19/diagnóstico , Cetoacidose Diabética/diagnóstico , Diagnóstico Diferencial , Serviço Hospitalar de Emergência , Feminino , Insuficiência Cardíaca/complicações , Herpes Zoster/diagnóstico , Humanos , Intoxicação por Chumbo/diagnóstico , Transtornos de Enxaqueca/diagnóstico , Torção Ovariana/diagnóstico , Doença Inflamatória Pélvica/diagnóstico , Pneumonia/diagnóstico , Porfiria Aguda Intermitente/diagnóstico , Gravidez , Gravidez Ectópica/diagnóstico , Embolia Pulmonar/diagnóstico , Púrpura de Schoenlein-Henoch/diagnóstico , Tireotoxicose/diagnóstico , Uremia/diagnóstico
6.
BMC Med Inform Decis Mak ; 21(1): 268, 2021 09 18.
Artigo em Inglês | MEDLINE | ID: mdl-34537047

RESUMO

BACKGROUND: The glycated hemoglobin (A1c) test is not recommended for sickle cell disease (SCD) patients. We examine ordering patterns of diabetes-related tests for SCD patients to explore misutilization of tests among this underserved population. METHODS: We used de-identified electronic health record (EHR) data in the Cerner Health Facts™ (HF) data warehouse to evaluate the frequency of A1c and fructosamine tests during 2010 to 2016, for 37,151 SCD patients from 393 healthcare facilities across the United States. After excluding facilities with no A1c data, we defined three groups of facilities based on the prevalence of SCD patients with A1c test(s): adherent facilities (no SCD patients with A1c test(s)), minor non-adherent facilities, major non-adherent facilities. RESULTS: We determined that 11% of SCD patients (3927 patients) treated at 393 facilities in the US received orders for at least one A1c test. Of the 3927 SCD patients with an A1c test, only 89 patients (2.3%) received an order for a fructosamine test. At the minor non-adherent facilities, 5% of the SCD patients received an A1c test while 58% of the SCD patients at the least adherent facilities had at least one A1c test. Overall, the percent of A1c tests ordered for SCD patients between 2010 and 2016 remained similar. CONCLUSIONS: Inappropriate A1c test orders among a sickle cell population is a significant quality gap. Interventions to advance adoption of professional recommendations that advocate for alternate tests, such as fructosamine, can guide clinicians in test selection to reduce this quality gap are discussed. The informatics strategy used in this work can inform other largescale analyses of lab test utilization using de-identified EHR data.


Assuntos
Anemia Falciforme , Diabetes Mellitus , Anemia Falciforme/diagnóstico , Registros Eletrônicos de Saúde , Frutosamina , Hemoglobina A Glicada , Humanos , Estados Unidos
7.
Free Radic Biol Med ; 175: 95-107, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34478834

RESUMO

Hemopexin (Hpx) is a crucial defense protein against heme liberated from degraded hemoglobin during hemolysis. High heme stress creates an imbalance in Hpx bioavailability, favoring heme accumulation and downstream pathophysiological responses leading to cardiopulmonary disease progression in sickle cell disease (SCD) patients. Here, we evaluated a model of murine SCD, which was designed to accelerate red blood cell sickling, pulmonary hypertension, right ventricular dysfunction, and exercise intolerance by exposure of the mice to moderate hypobaric hypoxia. The sequence of pathophysiology in this model tracks with circulatory heme accumulation, lipid oxidation, extensive remodeling of the pulmonary vasculature, and fibrosis. We hypothesized that Hpx replacement for an extended period would improve exercise tolerance measured by critical speed as a clinically meaningful therapeutic endpoint. Further, we sought to define the effects of Hpx on upstream cardiopulmonary function, histopathology, and tissue oxidation. Our data shows that tri-weekly administrations of Hpx for three months dose-dependently reduced heme exposure and pulmonary hypertension while improving cardiac pressure-volume relationships and exercise tolerance. Furthermore, Hpx administration dose-dependently attenuated pulmonary fibrosis and oxidative modifications in the lung and myocardium of the right ventricle. Observations in our SCD murine model are consistent with pulmonary vascular and right ventricular pathology at autopsy in SCD patients having suffered from severe pulmonary hypertension, right ventricular dysfunction, and sudden cardiac death. This study provides a translational evaluation supported by a rigorous outcome analysis demonstrating therapeutic proof-of-concept for Hpx replacement in SCD.


Assuntos
Anemia Falciforme , Hemopexina , Anemia Falciforme/tratamento farmacológico , Animais , Heme , Hemoglobinas , Hemólise , Humanos , Camundongos
8.
N C Med J ; 82(5): 312-320, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34544765

RESUMO

BACKGROUND Sickle cell disease (SCD) is a complex disease associated with many complications and a shortened lifespan. In 2016, the National Heart, Lung and Blood Institute funded 8 centers in the United States to form the Sickle Cell Disease Implementation Consortium, with the goal of improving SCD care. The aim of our study was to describe SCD self-efficacy, pain interference, and barriers to care from the perspective of persons with SCD in the North Carolina center.METHODS Persons with SCD, aged 15 and older were recruited from a large SCD center in North Carolina. Surveys, focus groups, and interviews were completed.RESULTS Fifty-one people completed a survey, and 14 people completed an interview or focus group. Barriers identified in the survey included self-care barriers, misconceptions related to hydroxyurea (an oral medication that reduces rates of pain crisis), limited provider knowledge, and stigma. Concerning self-efficacy, participants reported that they were able to manage their pain symptoms most of the time. Pain interfered most with the ability to participate in social and day-to-day activities.Common themes from the focus groups and interviews included misconceptions about hydroxyurea, pain, provider knowledge, stigma, co-management, transportation, and insurance. Recommendations to improve care included the use of case managers, utilization of treatment guidelines, individualized pain protocols, and effective co-management by providers.LIMITATIONS Participants were recruited from 1 SCD center and may not be representative of the entire SCD population in North Carolina.CONCLUSIONS Participants described many perceived barriers to care, and their responses suggest a need for improvements in patient hydroxyurea education, provider knowledge, and care coordination.


Assuntos
Anemia Falciforme , Anemia Falciforme/tratamento farmacológico , Humanos , Hidroxiureia , Determinação de Necessidades de Cuidados de Saúde , North Carolina , Dor , Estados Unidos
9.
Immunohematology ; 37(3): 131-137, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34591378

RESUMO

Sickle cell disease (SCD) poses a major public health challenge in sub-Saharan Africa, including Nigeria. Blood transfusion is a mainstay in SCD treatment. Erythrocyte alloimmunization is known to complicate the transfusional care of patients with SCD. Immune alloantibodies are associated with hemolytic transfusion reactions and transfusion refractoriness. We aimed to determine the prevalence, specificities, and clinical associations/risk factors of immune erythrocyte alloantibodies among adult patients with SCD compared with healthy blood donors in Lagos, Nigeria, through a cross-sectional study. All participants were interviewed using a structured questionnaire to obtain details on bio-data, hemoglobin phenotype, blood transfusion history, and SCD history where relevant. Blood specimens obtained from each participant were subjected to antibody screening/identification using tube agglutination method. The mean age of the SCD participants and healthy blood donors was 27.92 and 29.04 years, respectively. The majority (72.5%) of the SCD participants had received at least 1 unit of red blood cell (RBC) transfusion in their lifetime, compared with only 7.5 percent of blood donors. Six SCD participants (7.5%) tested positive for atypical erythrocyte alloantibodies, with none among blood donors. Most of the antibodies (75%) belonged to the Rh blood group system. The most frequent antibody was anti-E, followed by anti-C and anti-D. Advancing age (30 years or more), recent transfusions (last 4 weeks), higher transfusion rates, and established renal disease were significantly associated with alloimmunization (p values of 0.026, 0.043, 0.002, and 0.043, respectively). This study suggests blood transfusion as a strong risk factor for RBC alloimmunization in SCD patients. Extended RBC phenotyping is recommended for all patients with SCD, especially those receiving regular transfusions.Sickle cell disease (SCD) poses a major public health challenge in sub-Saharan Africa, including Nigeria. Blood transfusion is a mainstay in SCD treatment. Erythrocyte alloimmunization is known to complicate the transfusional care of patients with SCD. Immune alloantibodies are associated with hemolytic transfusion reactions and transfusion refractoriness. We aimed to determine the prevalence, specificities, and clinical associations/risk factors of immune erythrocyte alloantibodies among adult patients with SCD compared with healthy blood donors in Lagos, Nigeria, through a cross-sectional study. All participants were interviewed using a structured questionnaire to obtain details on bio-data, hemoglobin phenotype, blood transfusion history, and SCD history where relevant. Blood specimens obtained from each participant were subjected to antibody screening/identification using tube agglutination method. The mean age of the SCD participants and healthy blood donors was 27.92 and 29.04 years, respectively. The majority (72.5%) of the SCD participants had received at least 1 unit of red blood cell (RBC) transfusion in their lifetime, compared with only 7.5 percent of blood donors. Six SCD participants (7.5%) tested positive for atypical erythrocyte alloantibodies, with none among blood donors. Most of the antibodies (75%) belonged to the Rh blood group system. The most frequent antibody was anti-E, followed by anti-C and anti-D. Advancing age (30 years or more), recent transfusions (last 4 weeks), higher transfusion rates, and established renal disease were significantly associated with alloimmunization (p values of 0.026, 0.043, 0.002, and 0.043, respectively). This study suggests blood transfusion as a strong risk factor for RBC alloimmunization in SCD patients. Extended RBC phenotyping is recommended for all patients with SCD, especially those receiving regular transfusions.


Assuntos
Anemia Falciforme , Doadores de Sangue , Adulto , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Estudos Transversais , Eritrócitos , Humanos , Isoanticorpos , Nigéria
10.
Lancet Haematol ; 8(10): e677-e678, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34481548
11.
Acta Biomed ; 92(4): e2021410, 2021 09 02.
Artigo em Inglês | MEDLINE | ID: mdl-34487058

RESUMO

Starting from 2021, Acta Biomedica Parmensis will dedicate an annual update to the "Advances in Hemoglobinopathies". The section editor of this new editorial initiative is prof. Ashraf T Soliman, Pediatrician and Endocrinologist at Hamad Medical Center (HMC) of Doha. Prof Soliman is a pionier in the study of endocrine complications in hemoglobinopathies and effects of blood transfusions on spermatogenesis. He collaborates strictly with prof. Mohamed Yassin, Hematologist-Oncologist at MCH and the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine (ICET-A). This issue of Acta Biomedica contains three articles on: The different patterns of insulin response during Oral Glucose Tolerance Test (OGTT) in transfused young patients with ß- Thalassemia; Immigration and screening programs for hemoglobinopathies in Italy, Spain and Turkey and The effects of treatment with blood transfusion, iron chelation and hydroxyurea on puberty, growth and spermatogenesis in sickle cell disease,.


Assuntos
Anemia Falciforme , Hemoglobinopatias , Talassemia , Talassemia beta , Emigração e Imigração , Hemoglobinopatias/terapia , Humanos , Masculino , Talassemia beta/terapia
12.
Acta Biomed ; 92(4): e2021386, 2021 09 02.
Artigo em Inglês | MEDLINE | ID: mdl-34487059

RESUMO

Sickle cell disease (SCD) is traditionally associated with growth failure and delayed puberty. Wasting and stunting are still prevalent in children and adolescents with SCD, especially in developing countries. In addition, sperm abnormalities are frequent in males with SCD, with high rates of low sperm density, low sperm counts, poor motility, and increased abnormal morphology.  Severe anemia, vaso-occlusive attacks with ischemic injury to different organs including the pituitary gland and testis, and nutritional factors are incriminated in the pathogenesis of defective growth, puberty, and spermatogenesis. There is great phenotypic variability among patients with SCD. The variability in the clinical severity of SCA can partly be explained by genetic modifiers, including HbF level and co-inheritance of α-thalassemia. In the past, severe disease led to early mortality. Advancements in treatment have allowed patients with SCD to have a longer and better quality of life. For most patients, the mainstays of treatment are preventive and supportive. For those with severe SCD, three major therapeutic options are currently available: erythrocyte transfusion or exchange, hydroxyurea, and hematopoietic stem cell transplantation. In this mini-review, the authors tried to recognize, delineate, and update knowledge on abnormalities due to SCD from those created by the use of different treatment modalities.


Assuntos
Anemia Falciforme , Hidroxiureia , Adolescente , Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos , Transfusão de Sangue , Humanos , Hidroxiureia/uso terapêutico , Quelantes de Ferro , Masculino , Puberdade , Qualidade de Vida , Espermatogênese
13.
Pan Afr Med J ; 39: 140, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34527156

RESUMO

Introduction: the spectrum of pulmonary complications in sickle cell anemia (SCA) comprises mainly of acute chest syndrome (ACS), pulmonary hypertension (PH) and airway hyper-responsiveness (AHR). This study was conducted to examine the abnormalities in pulmonary function tests (PFTs) seen in children with SCA. Methods: electronic databases (Cochrane library, PubMed, EMBASE, Scopus, Web of Science) were used as data sources. Two authors independently reviewed studies. All case-control studies with PFT performed in patients with SCA and normal controls were reviewed. Pulmonary functions were assessed with the help of spirometry, lung volume and gas diffusion findings. Results: nine studies with 788 SCA children and 1101 controls were analyzed. For all studies, the pooled mean difference for forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, peak expiratory flow rate (PEFR), total lung capacity (TLC) and carbon mono-oxide diffusing capacity (DLCO) were -12.67, (95% CI: -15.41,-9.94), -11.69, (95% CI: -14.24, -9.14), -1.90, (95% CI: -4.32, 0.52), -3.36 (95% CI: -6.69, -0.02), -7.35, (95% CI: -14.97, -0.27) and -4.68, (95% CI -20.64, -11.29) respectively. FEV1 and FVC and were the only parameters found to be significantly decreased. Conclusion: sickle cell anemia was associated with lower FEV1 and FVC, thus, supporting the role of routine monitoring for the progression of lung function decline in children with SCA with ACS. We recommend routine screening and lung function monitoring for early recognition of pulmonary function decline.


Assuntos
Anemia Falciforme/complicações , Testes de Função Respiratória/métodos , Síndrome Torácica Aguda/diagnóstico , Síndrome Torácica Aguda/etiologia , Criança , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Medidas de Volume Pulmonar , Hipersensibilidade Respiratória/diagnóstico , Hipersensibilidade Respiratória/etiologia , Espirometria
14.
Lancet Haematol ; 8(10): e744-e755, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34481550

RESUMO

Sickle cell disease can be life-threatening or chronically debilitating for both children and adults. Worldwide, more than 300 000 children are born with sickle cell disease every year, over 75% of whom in sub-Saharan Africa. Increased awareness and early interventions, such as neonate screening and comprehensive care, have led to considerable reductions in mortality in children younger than 5 years in high-income countries. However, sickle cell disease prevention and care have largely been neglected in Africa. Without intervention, 50-90% of affected children in many sub-Saharan African countries die before their fifth birthday. Fortunately, increasing initiatives in sub-Saharan Africa are piloting interventions such as neonate screening and comprehensive care, and as mortality declines, quality of life and increased life expectancy become major targets for interventions. Hydroxyurea (hydroxycarbamide) and haematopoietic stem-cell transplantation have already been shown to be effective therapies in high-income countries, but are either not widely accessible or too expensive for most African populations. These challenges are being alleviated by numerous networks evolving through international collaborations that are positively changing the outlook of sickle cell disease management in sub-Saharan Africa. In this Series paper, we describe the epidemiology, pathophysiology, clinicobiological profile, and psychosocial effects of sickle cell disease in sub-Saharan Africa. We highlight transferable strategies already used for the successful management of the condition and key strategies and recommendations for affordable and comprehensive care on the continent. TRANSLATION: For the French translation of the abstract see Supplementary Materials section.


Assuntos
Anemia Falciforme/prevenção & controle , África ao Sul do Saara/epidemiologia , Anemia Falciforme/epidemiologia , Anemia Falciforme/fisiopatologia , Anemia Falciforme/psicologia , Humanos , Qualidade de Vida
15.
Pan Afr Med J ; 39: 163, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34548892

RESUMO

Introduction: Sickle Cell Disease (SCD) is the leading genetic disease in sub-Saharan Africa and therefore remains a global public health threat. Use of complementary and alternative medicines (CAM) most especially herbal medicine (HM) in chronic diseases such as sickle cell disease has widely been reported in Africa where advanced technologies are greatly lacking. Despite a large presence of the sickle cell disease in Uganda, the extent to which herbal medicines are used in management of children with sickle cell disease has not been documented. This study purposed to determine the prevalence of herbal medicine (HM) use and associated factors among caregivers of children with SCD at Mulago National Referral Hospital. Methods: a total of 384 child caretakers were interviewed in a descriptive cross-sectional quantitative study conducted at the Mulago Sickle cell clinic in March 2019. Enrolment was done consecutively and a structured interviewer administered questionnaire administered to collect data from the caretakers which was managed using SPSS version 23. Multivariate logistic regression was used to identify the factors associated with herbal medicine (HM) use. Factors with p-value <0.05 were regarded significant. Results: the rate of herbal use was 77.6% (298 of 384 caregivers). At multivariate analysis, the odds of a caregiver who agreed that; HM cures symptoms faster than conventional medicine (CM) were 3 times those who disagreed with this statement (AOR =3.439, 95% CI: 1.447 - 8.176). The odds that a caregiver who agreed that HM has fewer side effects than CM were almost 4 times those that disagreed with this statement (AOR = 3.528, 95% CI: 1.917 -6.494). The odds that a caregiver who agreed that marketing HM through televisions adverts encourages HM use were 4 times those who disagreed with this statement (AOR = 4.185, 95% CI: 2.036 -8.603). Conclusion: this study reports a high prevalence of HM use among caregivers of children with SCD at Mulago Hospital, in Uganda. The practice is significantly influenced by caretakers´ perception that HM cures symptoms faster than CM, has fewer side effects and that telemarketing has greatly facilitated its use over CM. More effort is therefore needed to encourage clinic attendances and CM use and limit the unfounded TV adverts on HM. There is also need for studies to identify the common HM used so that their efficacy and safety are well studied.


Assuntos
Anemia Falciforme/tratamento farmacológico , Cuidadores/estatística & dados numéricos , Fitoterapia/estatística & dados numéricos , Preparações de Plantas/administração & dosagem , Adolescente , Adulto , Idoso , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Uganda , Adulto Jovem
16.
J Med Microbiol ; 70(9)2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34477545

RESUMO

Introduction. Sickle cell disease (SCD) children have a high susceptibility to pneumococcal infection. For this reason, they are routinely immunized with pneumococcal vaccines and use antibiotic prophylaxis (AP).Hypothesis/Gap Statement. Yet, little is known about SCD children's gut microbiota. If antibiotic-resistant Enterobacterales may colonize people on AP, we hypothesized that SCD children on AP are colonized by resistant enterobacteria species.Objective. To evaluate the effect of continuous AP on Enterobacterales gut colonization from children with SCD.Methodology. We analysed 30 faecal swabs from SCD children on AP and 21 swabs from children without the same condition. Enterobacterales was isolated on MacConkey agar plates and identified by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) (bioMérieux, Marcy l'Etoile, France). We performed the antibiogram by Vitek 2 system (bioMérieux, Marcy l'Etoile, France), and the resistance genes were identified by multiplex PCR.Results. We found four different species with resistance to one or more different antibiotic types in the AP-SCD children's group: Escherichia coli, Klebsiella pneumoniae, Citrobacter freundii, and Citrobacter farmeri. Colonization by resistant E. coli was associated with AP (prevalence ratio 2.69, 95 % confidence interval [CI], 1.98-3.67, P<0.001). Strains producing extended-spectrum ß-lactamases (ESBL) were identified only in SCD children, E. coli, 4/30 (13 %), and K. pneumoniae, 2/30 (7 %). The ESBL-producing Enterobacterales were associated with penicillin G benzathine use (95 % CI, 22.91-86.71, P<0.001). CTX-M-1 was the most prevalent among ESBL-producers (3/6, 50 %), followed by CTX-M-9 (2/6, 33 %), and CTX-M-2 (1/6, 17 %).Conclusion. Resistant enterobacteria colonize SCD children on AP, and this therapy raises the chance of ESBL-producing Enterobacterales colonization. Future studies should focus on prophylactic vaccines as exclusive therapy against pneumococcal infections.


Assuntos
Anemia Falciforme/prevenção & controle , Antibacterianos/efeitos adversos , Antibioticoprofilaxia , Enterobacteriáceas Resistentes a Carbapenêmicos/efeitos dos fármacos , Infecções por Enterobacteriaceae/microbiologia , Vacinas Pneumocócicas/efeitos adversos , Adolescente , Brasil/epidemiologia , Criança , Pré-Escolar , Farmacorresistência Bacteriana Múltipla , Feminino , Microbioma Gastrointestinal/efeitos dos fármacos , Humanos , Masculino
17.
Hematology ; 26(1): 684-690, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34493173

RESUMO

BACKGROUND: Sickle cell anaemia affects about 4 million people across the globe, making it an inherited disorder of public health importance. Red cell lysis consequent upon haemoglobin crystallization and repeated sickling leads to anaemia and a baseline strain on haemopoiesis. Vaso-occlusion and haemolysis underlies majority of the chronic complications of sickle cell. We evaluated the clinical and laboratory features observed across the various clinical phenotypes in adult sickle cell disease patients. METHODS: Steady state data collected prospectively in a cohort of adult sickle cell disease patients as out-patients between July 2010 and July 2020. The information included epidemiological, clinical and laboratory data. RESULTS: About 270 patients were captured in this study (165 males and 105 females). Their ages ranged from 16 to 55 years, with a median age of 25 years. Sixty-eight had leg ulcers, 43 of the males had priapism (erectile dysfunction in 8), 42 had AVN, 31 had nephropathy, 23 had osteomyelitis, 15 had osteoarthritis, 12 had cholelithiasis, 10 had stroke or other neurological impairment, 5 had pulmonary hypertension, while 23 had other complications. Frequency of crisis ranged from 0 to >10/year median of 2. Of the 219 recorded, 148 of the patients had been transfused in the past, while 71 had not. CONCLUSION: The prevalence of SLU, AVN, priapism, nephropathy and the other complications of SCD show some variations from other studies. This variation in the clinical parameters across different clinical phenotypes indicates an interplay between age, genetic and environmental factors.


Assuntos
Anemia Falciforme , Adolescente , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Anemia Falciforme/metabolismo , Anemia Falciforme/patologia , Colelitíase/etiologia , Colelitíase/metabolismo , Colelitíase/patologia , Feminino , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/metabolismo , Hipertensão Pulmonar/patologia , Nefropatias/epidemiologia , Nefropatias/etiologia , Nefropatias/metabolismo , Nefropatias/patologia , Úlcera da Perna/epidemiologia , Úlcera da Perna/etiologia , Úlcera da Perna/metabolismo , Úlcera da Perna/patologia , Masculino , Pessoa de Meia-Idade , Nigéria/epidemiologia , Osteoartrite/epidemiologia , Osteoartrite/etiologia , Osteoartrite/metabolismo , Osteomielite/epidemiologia , Osteomielite/etiologia , Osteomielite/metabolismo , Osteomielite/patologia , Priapismo/epidemiologia , Priapismo/etiologia , Priapismo/metabolismo , Priapismo/patologia , Estudos Prospectivos , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/metabolismo , Acidente Vascular Cerebral/patologia
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