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2.
Arq. bras. cardiol ; 118(3): 565-575, mar. 2022. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1364343

ABSTRACT

Resumo Fundamento Pacientes com anemia falciforme (AF) têm risco aumentado de complicações cardiovasculares. O teste ergométrico é usado como marcador de prognóstico em uma série de doenças cardiovasculares. Entretanto, há uma escassez de evidências sobre exercícios em pacientes com AF, especialmente em relação à sua segurança, viabilidade e possível função prognóstica. Objetivos Usamos o teste em esteira máximo para determinar a segurança e a viabilidade do teste ergométrico em pacientes com AF. Além disso, os fatores associados à duração do exercício, bem como o impacto das alterações causadas pelo exercício em resultados clínicos, também foram avaliados. Métodos 113 pacientes com AF que passaram pelo teste ergométrico e por uma avaliação cardiovascular abrangente incluindo um ecocardiograma e os níveis do peptídeo natriurético do tipo B (BNP). O desfecho de longo prazo foi uma combinação de eventos incluindo morte, crises álgicas graves, síndrome torácica aguda ou internações hospitalares por outras complicações associadas â doença falciforme. A análise de regressão de Cox foi realizada para identificar as variáveis associadas ao resultado. Um p valor <0,05 foi considerado estatisticamente significativo. Resultados A média de idade foi de 36 ± 12 anos (intervalo, 18-65 anos), e 62 pacientes eram do sexo feminino (52%). A presença de alterações isquêmicas ao esforço e resposta pressórica anormal ao exercício foram detectadas em 17% e 9 % da´população estudada respectivamente. Dois pacientes apresentaram crise álgica com necessidade de internação hospitalar no período de 48 horas da realização do exame. Fatores associados à duração do exercício foram idade, sexo, velocidade máxima de regurgitação tricúspide (RT), e relação E/e', após a padronização quanto aos marcadores da gravidade da doença. Durante o período médio de acompanhamento de 10,1 meses (variando de 1,2 a 26), 27 pacientes (23%) apresentaram desfechos clínicos adversos. Preditores independentes de eventos adversos foram a concentração de hemoglobina, velocidade do fluxo transmitral tardio (onda A), e a resposta da PA ao exercício. Conclusões A realização de testes ergométricos em pacientes com AF, clinicamente estáveis, é viável. A duração do exercício estava associada à função diastólica e a pressão arterial pulmonar. A resposta anormal da PA foi um preditor independente de eventos adversos.


Abstract Background Patients with sickle cell disease (SCD) are at increased risk for cardiovascular complications. Exercise testing is used as a prognostic marker in a variety of cardiovascular diseases. However, there is a lack of evidence on exercise in SCD patients, particularly regarding its safety, feasibility, and possible prognostic role. Objectives We used the maximal treadmill test to determine safety and feasibility of the exercise testing in SCD patients. Additionally, the factors associated with exercise duration, as well as the impact of exercise-induced changes on clinical outcome, were also assessed. Methods One-hundred thirteen patients with SCD, who underwent exercise testing, were prospectively enrolled. A comprehensive cardiovascular evaluation, including echocardiography and B-type natriuretic peptide (BNP) levels, were obtained. The long-term outcome was a composite endpoint of death, severe acute painful episodes, acute chest syndrome, or hospitalization for other SCD-related complications. Cox regression analysis was performed to identify the variables associated with the outcome. A p-value<0.05 was considered to be statistically significant. Results The mean age was 36 ± 12 years (range, 18-65 years), and 62 patients were women (52%). Ischemic electrocardiogram and abnormal blood pressure (BP) response to exercise were detected in 17% and 9%, respectively. Two patients experienced pain crises within 48 hours that required hospitalization. Factors associated with exercise duration were age, sex, tricuspid regurgitation (TR) maximal velocity, and E/e' ratio, after adjustment for markers of disease severity. During the mean follow-up of 10.1 months (ranging from 1.2 to 26), the endpoint was reached in 27 patients (23%). Independent predictors of adverse events were hemoglobin concentration, late transmitral flow velocity (A wave), and BP response to exercise. Conclusions Exercise testing in SCD patients who were clinically stable is feasible. Exercise duration was associated with diastolic function and pulmonary artery pressure. Abnormal BP response was an independent predictor of adverse events.


Subject(s)
Humans , Female , Adult , Young Adult , Exercise Test , Anemia, Sickle Cell/complications , Prognosis , Echocardiography , Feasibility Studies , Middle Aged
4.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(4): 468-475, Oct.-Dec. 2021. tab, graf
Article in English | LILACS | ID: biblio-1350824

ABSTRACT

ABSTRACT Introduction: Although the efficacy of hydroxyurea (HU) in inhibiting erythrocyte sickling has been well demonstrated, the action of this drug on human neutrophils and the mechanism by which it improves the manifestations of the disease have not been studied thoroughly. We aimed to investigate the cell viability, along with inflammatory and oxidative markers in the neutrophils of sickle cell anemia (SCA) patients and the effects of HU therapy on these cells, by evaluating the dose-responsiveness. Methods: In the present study, 101 patients (45 men and 56 women, aged 18-69 years) with SCA were divided into groups according to the use or not of HU: the SS group (without HU treatment, n = 47) and the SSHU group (under HU treatment, n = 54). The SSHU group was further stratified into subgroups according to the daily dose of the drug that patients already used: SSHU - 0.5 g (n = 19); SSHU - 1 g (n = 26) and SSHU - 1.5-2 g (n = 9). A control group (AA) comprised 50 healthy individuals. Neutrophils isolated from whole blood were analyzed using Trypan Blue, monoiodotyrosine (MTT) and lactate dehydrogenase (LDH) toxicity assays. Myeloperoxidase (MPO), superoxide dismutase (SOD), glutathione peroxidase (GSH-Px) activities and concentrations of interleukin 10 (IL-10), tumor necrosis factor alpha (TNF-α) and malonaldehyde (MDA) were also measured. Results: Neutrophils from SCA patients showed membrane fragility and a significant decrease in cell viability when analyzed by Trypan Blue (p < 0.05), MTT (p < 0.001) and LDH (p = 0.011), compared to the AA group. Levels of inflammatory (MPO, TNF-α, and IL-10) and oxidative markers (SOD, GSH-Px, and MDA) were also altered (p < 0.05) in these cells, showing a significant difference in the SSHU-1g and SSHU - 1.5-2 g groups, compared to the SS group. Treatment with HU reverted the levels of all markers to concentrations similar to those in healthy individuals in a positive dose-effect relationship. Conclusion: The HU did not generate a cytotoxic effect on neutrophils in SCA patients, but it modulated their oxidative and inflammatory mechanisms, promoting cytoprotection with a positive dose-effect.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Aged , Hydroxyurea , Anemia, Sickle Cell , Tumor Necrosis Factor-alpha , Oxidative Stress , Cytotoxicity, Immunologic , Dosage , Inflammation , Malondialdehyde , Neutrophils
5.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(4): 443-452, Oct.-Dec. 2021. tab, graf, ilus
Article in English | LILACS | ID: biblio-1350818

ABSTRACT

ABSTRACT Introduction: Inspiratory muscle training (IMT) has been shown to be an efficient method of improving exercise tolerance and inspiratory and expiratory muscle strength in several diseases. The effects of IMT on patients with sickle cell anemia (SCD) are relatively unknown. Our study aimed to evaluate the effects of IMT on adult SCD patients, regarding respiratory muscle strength (RMS) variables, lung function, exercise tolerance, blood lactation concentration, limitation imposed by dyspnea during daily activities and impact of fatigue on the quality of life. Methods: This was a randomized single-blind study, with an IMT design comprising true load (TG) and sham load (SG) groups. Initial assessment included spirometry, volumetric capnography (VCap) and measurement of RMS by maximal inspiratory and expiratory pressure (PImax and PEmax). The Medical Research Council dyspnea scale and modified fatigue impact scale were also applied and blood lactate concentration was measured before and after the 6-minute walk test. After this initial assessment, the patient used the IMT device at home daily, returning every 6 weeks for RMS reassessment. Both groups used the same device and were unaware of which group they were in. After a period totaling 18 weeks, patients underwent the final evaluation, as initially performed. Results: Twenty-five patients in total participated until the end of the study (median age 42 years). There were no significant differences between TG and SG based on age, sex, body mass index or severity of genotype. At the end of the training, both groups showed a significant increase in PEmax and PImax, improvement in Vcap and in exercise tolerance and dyspnea reduction while performing daily life activities. The same was observed in patients grouped according to disease severity (HbSS and HbSβ0 vs HbSC and HbSβ+), without differences between groups. Conclusion: Home-based inspiratory muscle training benefits outpatients with SCD, including the sham load group. Trial registration:http://www.ensaiosclinicos.gov.br; registration number: RBR-6g8n92.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Breathing Exercises , Anemia, Sickle Cell , Inspiratory Capacity , Exercise Tolerance , Capnography , Maximal Respiratory Pressures
6.
Estima (Online) ; 19(1): e0321, jan.-dez. 2021.
Article in English, Portuguese | LILACS, BDENF | ID: biblio-1178276

ABSTRACT

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.


Objective:to characterize the profile of women with leg ulcers resulting from sickle cell disease treated at a public health institution in Brazil. Method: historical cohort study conducted in the outpatient sector with a sample of women with sickle cell disease and leg ulcer, followed from 1998 to 2014. The data were extracted from the medical record and included sociodemographic, clinical and ulcer-related variables, and underwent descriptive statistical analysis. Results: of the 17 women, 64.7% were cured, 58.8% were aged between 30 and 39 years, 64.7% declared themselves brown, 70.6% were without partners, 41.2% had incomplete elementary education, 52, 9% had an income of 2 to 3 minimum wages, 70.6% were eutrophic and 82.4% used vitamin supplements. Women had a total of 29 ulcers, 52.9% had a single ulcer, 41.4% were located in the malleolar region, 55.17% were between 2 and 36 months, all classified as stage 3. The treatment was with coverings and Unna's boot. Conclusion: the women received care from specialist nurses and most were cured with up to eight months of follow-up, which required a nursing consultation, including clinical evaluation, topical treatment and application of compression therapy


Objetivo:caracterizar el perfil de mujeres con úlceras de la pierna consecuente de enfermedad de células falciformes atendidas en una institución pública de salud de Brasil. Método: estudio de cohorte histórica realizado en el sector ambulatorio con muestra de mujeres con enfermedad de células falciformes y úlcera de pierna, seguidas de 1998 a 2014. Los datos fueron extraídos de la ficha médica y abarcaron las variables sociodemográficas, clínicas y relacionadas con la úlcera y pasaron por análisis estadístico descriptivo. Resultados: de las 17 mujeres, el 64,7% obtuvieron curación, el 58,8% tenían edades entre 30 y 39 años, el 64,7% se autodeclararon morenas, el 70,6% no tenían pareja, el 41,2% no habían completado la educación básica, el 52,9% con ingresos de 2 a 3 sueldos mínimos, el 70,6% eran eutróficas, el 82,4% hacían uso de suplementos vitamínicos. Las mujeres presentaron un total de 29 úlceras, el 52,9% tenían úlcera única, el 41,4% ubicadas en la zona maleolar; el 55,17% con existencia entre 2 y 36 meses, todas clasificadas en estadio 3. El tratamiento se dio con vendajes y bota de Unna. Conclusión: las mujeres recibieron cuidados de enfermeros especialistas y la mayoría obtuvo la curación con hasta ocho meses de seguimiento. Fueron sometidas a la consulta de enfermería, comprendiendo la evaluación clínica, el tratamiento tópico y la aplicación de terapia de compresión.


Subject(s)
Health Profile , Anemia, Sickle Cell , Leg Ulcer
7.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(3): 263-267, July-Sept. 2021. tab
Article in English | LILACS | ID: biblio-1346272

ABSTRACT

Abstract Introduction: Knowledge on the characteristics of neuropathic pain in people with sickle cell disease (SCD) may help to provide more effective treatment procedures. Objective: To describe the characteristics of neuropathic pain in patients with sickle cell disease and identify the impact on their quality of life. Method: A cross-sectional study (CAAE 57274516.8.0000.5544) was conducted at a reference center in Salvador, Bahia, Brazil. The instruments used were the Brief Pain Inventory (BPI), the Douleur Neuropatique Questionnaire (DN-4), the Anxiety and Depression Hospital scale (ADH) and the abbreviated version of the World Health Organization of Quality of Life questionnaire (WHOQOL-brief). The Mann-Whitney test was used to evaluate the association between the scores (5% alpha). Results: A total of 100 adults with SCD participated in the study, 69.7% of whom had neuropathic pain. Anxiety was present in 99% of the sample and depression, in 100%. Patients with neuropathic pain had worse scores in all domains of quality of life (p < 0.05), but no association was found with pain intensity. Conclusion: Neuropathic pain was more frequent than nociceptive pain in adults with SCD and generated worse scores in all domains of quality of life. Anxiety and depression were present in patients with both types of pain.


Subject(s)
Humans , Male , Female , Quality of Life , Anemia, Sickle Cell , Neuralgia , Anxiety , Depression
8.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(3): 243-248, July-Sept. 2021. tab, graf
Article in English | LILACS | ID: biblio-1346265

ABSTRACT

Abstract Introduction: Sickle cell anemia (SCA) is a Mendelian disorder with a heterogeneous clinical course. The reasons for this phenotypic diversity are not entirely established, but it is known that high fetal hemoglobin levels lead to a milder course of the disease. Additionally, genetic variants in the intergenic region HBS1L-MYB promote high levels of fetal hemoglobin into adulthood. Objective: In the present study, we investigated the HMIP1 C-839A (rs9376092) polymorphism, located at the HBS1L-MYB intergenic region block 1, in SCA patients. Method: We analyzed 299 SCA patients followed in two reference centers in Brazil. The HMIP1 C-839A (rs9376092) genotypes were determined by allele specific polymerase chain reactions. Clinical and laboratory data were obtained from patient interviews and medical records. Results: The median fetal hemoglobin levels were higher in patients with the HMIP1 C-839A (rs9376092) AA genotype (CC = 6.4%, CA = 5.6% and AA = 8.6%), but this difference did not reach significance (p = 0.194). No association between HMIP1 C-839A (rs9376092) genotypes and other clinical and laboratorial features was detected (p > 0.05). Conclusion: In summary, our data could not support the previously related association between the HMIP1 C-893A (rs9376092) polymorphism and differential fetal hemoglobin levels.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Fetal Hemoglobin , Anemia, Sickle Cell , Polymorphism, Genetic
9.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(3): 268-279, July-Sept. 2021. tab, graf, ilus
Article in English | LILACS | ID: biblio-1346257

ABSTRACT

ABSTRACT Objective: To investigate the effectiveness of a home-based therapeutic exercise program on lower back pain and functionality of SCD patients. Setting: A Hematology and Transfusion Medicine Center, University of Campinas (HEMOCENTRO-UNICAMP). Methods: This was a prospective study, with a three-month follow-up of SCD patients with lower back pain. The lumbar spine functionality was evaluated by questionnaires, trunk flexion and extension analyses by fiber-optic-electrogoniometry and measurements of muscle strength of trunk flexor and extensors. The Intervention Group (IG) comprised 18 volunteers, median age 44y (28-58) and the control group (CG) comprised 15 volunteers, median age 42y (19-58), who did not perform exercises. The protocol consisted of daily home-based exercises with two evaluations: at the beginning and end of a three-month program. In order to compare the groups at baseline, the Fishers´ exact test and Mann-Whitney test were used for categorical and numeric variables, respectively. The Wilcoxon test was used for related samples comparing numeric measures of each group over time with a 5% (p < 0.05) significance level. Results: After the intervention, patients demonstrated a significant improvement, according to the Visual-Analog-Scale (VAS; p = 0.01), Rolland Morris Disability questionnaire (RMDQ; p < 0.01) and trunk flexion and extension muscle strength (p < 0.01). No significant differences were found for the Start-Back-Screening-Tool-Brazil (SBST) and in measures of trunk flexion and extension range-of-motion (RoM). Conclusion: Results suggest that daily home-based exercises for a three-month period ameliorate pain and improve disability related to lower back pain and muscle strength.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Exercise , Back Pain , Anemia, Sickle Cell
10.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(3): 324-331, July-Sept. 2021. tab, ilus
Article in English | LILACS | ID: biblio-1346253

ABSTRACT

Introduction and objective: Sickle cell anemia (SCA) is a genetic condition that alters the conformation of deoxygenated red blood cells, which results in their stiffening and the occurrence of vaso-occlusive crises, endothelium damage, organ dysfunction and systemic complications. Additionally, SCA limits the participation of individuals in physical and social activities. As we consider that physical exercise promotes the recovery of functional capacity and cardiorespiratory conditioning, we aim to verify the patterns of prescription, the effects and safety of exercise for individuals with SCA. Methodology: We systematically reviewed the published literature focusing on clinical trials that correlated physical exercise with SCA patients and cross-sectional studies that applied the stress test. The data research was based on the PRISMA recommendations and the following databases were used: Medline by PubMed, Cochrane, PEDro, Scielo. Results: Six studies which were based on the evaluation of 212 patients aged between 13 and 40 years, were selected from 122 identified studies. Those studies associated the individual effort tolerance improvement, its inflammatory profile adjustment and the absence of alteration in the autonomic nervous system activity to physical exercise or stress test. Conclusion: Low-to-moderate intensity physical exercise increased the SCA individual tolerance without causing vaso-occlusive crises, nor changes in the hemorheological and inflammatory profiles.


Subject(s)
Humans , Adolescent , Adult , Exercise , Anemia, Sickle Cell , Hematology , Inflammation
12.
Rev. cuba. med ; 60(3): e1641, 2021.
Article in Spanish | LILACS, CUMED | ID: biblio-1347518

ABSTRACT

Introducción: La anemia drepanocítica es una enfermedad pleiotrópica sistémica. Con su padecimiento, casi todos los órganos se afectan. Las formas de presentación de esta enfermedad varían entre los pacientes. Objetivo: Describir las características principales de las complicaciones y comorbilidades más frecuentes en la anemia drepanocítica. Método: Se realizó una revisión de la literatura, de artículos publicados en los últimos 10 años sobre complicaciones y comorbilidades más frecuentes en la anemia drepanocítica. Conclusión: El conocimiento y diferenciación de las complicaciones y comorbilidades en la anemia drepanocítica permite administrar las terapias adecuadas(AU)


Introduction: Sickle cell anemia is a systemic pleiotropic disease. Almost all organs are affected with his condition. The forms of presentation of this disease vary. Objective: To describe the main characteristics of the most frequent complications and comorbidities in sickle cell anemia. Method: A literature review of articles published in the last 10 years on the most frequent complications and comorbidities in sickle cell anemia was carried out. Conclusion: The knowledge and differentiation of complications and comorbidities in sickle cell anemia allows the administration of appropriate therapiesAU)


Subject(s)
Humans , Comorbidity , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy
13.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(2): 156-164, Apr.-June 2021. tab, graf, ilus
Article in English | LILACS | ID: biblio-1286679

ABSTRACT

ABSTRACT Introduction Sickle cell disease (SCD) is a monogenic disease and it is estimated that 300,000 infants are born annually with it. Most treatments available are only palliative, whereas the allogeneic hematopoietic stem cell transplantation offers the only potential cure for SCD. Objective Generation of human autologous cells, when coupled with induced pluripotent stem cell (iPSC) technology, is a promising approach for developing study models. In this study, we provide a simple and efficient model for generating hematopoietic cells using iPSCs derived from a sickle cell anemia patient and an inexpensive in-house-prepared medium. Method This study used iPSCs previously generated from peripheral blood mononuclear cells (PBMCs) from a patient with sickle cell anemia (iPSC_scd). Hematopoietic and erythroid differentiation was performed in two steps. Firstly, with the induction of hematopoietic differentiation through embryoid body formation, we evaluated the efficiency of two serum-free media; and secondly, the induction of hematopoietic stem/progenitor cells to erythroid progenitor cells was performed. Results The patient-specific cell line generated CD34+/CD45+ and CD45+/CD43+ hematopoietic stem/progenitor cells and erythroid progenitors, comprising CD36+, CD71+ and CD235a+ populations, as well as the formation of hematopoietic colonies, including erythroid colonies, in culture in a semi-solid medium. Conclusion In conjunction, our results described a simple serum-free platform to differentiate human the iPSCs into hematopoietic progenitor cells. This platform is an emerging application of iPSCs in vitro disease modeling, which can significantly improve the search for new pharmacological drugs for sickle cell disease.


Subject(s)
Hematopoietic Stem Cells , Induced Pluripotent Stem Cells , Anemia, Sickle Cell/therapy , Erythroid Precursor Cells
14.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(2): 165-170, Apr.-June 2021. tab
Article in English | LILACS | ID: biblio-1286677

ABSTRACT

ABSTRACT Introduction Mutations affecting genes involved in oxidative and signaling pathways may be associated with kidney disease in sickle cell anemia. We determined the allele and genotype frequencies of some polymorphisms in the promoter regions of the Heme Oxygenase-1 (HMOX1) [rs2071746 (A > T) and (GT)n repeats, short (S) and long (L) alleles] and Bone Morphogenetic Protein Receptor type-1B (BMPR1B) [rs17022863 (A > G), rs4331783 (A > G) and rs1470409 (A > G)] genes in 75 adult patients with sickle cell anemia and 160 healthy controls and investigated whether these polymorphisms may influence the estimated glomerular filtration rate for the patients. Methods The single nucleotide polymorphisms were genotyped using the TaqMan assays, the HMOX1(GT)n repeats were determined by polymerase chain reaction fragment size analysis and the estimated glomerular filtration rate was calculated by the Modification of Diet in Renal Disease formula. Results Regarding the HMOX1rs2071746, the estimated glomerular filtration rate median was significantly higher in TT patients (p = 0.019), including when TT was compared with AT + AA (p = 0.009); for the (GT)n repeats, the estimated glomerular filtration rate medians of SS, SL and LL significantly differed (p = 0.009), being the LL estimated glomerular filtration rate median significantly higher, when compared with the LS + SS (p = 0.005). These results suggest that both the homozygotes, TT for rs2071746 and LL for (GT)n repeats, lead to a higher risk of developing renal complications. Concerning the BMPR1B, the frequencies of GG for rs17022863 and AA for rs4331783 were significantly higher in patients than in controls (p = 0.002 and p = 0.008, respectively), however no association with estimated glomerular filtration rate was found. Conclusion These results contribute to a better understanding of the genetic factors related to the development of nephropathy in sickle cell anemia patients.


Subject(s)
Humans , Male , Female , Polymorphism, Genetic , Oxidative Stress , Heme Oxygenase-1 , Glomerular Filtration Rate , Anemia, Sickle Cell
16.
Prensa méd. argent ; 107(3): 157-161, 20210000. tab
Article in English | LILACS, BINACIS | ID: biblio-1359922

ABSTRACT

Antecedentes: el fármaco antimetabolito aumenta el nivel de hemoglobina fetal y reduce la frecuencia de crisis en pacientes con anemia de células falciformes. Objetivo: Evaluar el efecto de los antimetabolitos (hidroxiurea) en casos con crisis falciforme frecuente de anemia de células falciformes y talasemia no dependiente de transfusiones en el hospital de formación de Karbala desde abril de 2016 hasta diciembre de 2020. Pacientes y métodos: de 81 pacientes realizados en este estudio de casos y controles, cuarenta recibieron hidroxiurea y los otros cuarenta y un pacientes no. Se realizaron monitoreos cada dos semanas en los primeros tres meses mediante el envío para análisis (Hb, WBC, recuento de plaquetas y urea en sangre y creatinina sérica) LA PRENSA MÉDICA ARGENTINA Antimetabolite drug in patients with sickle cell diseases in hematological center of kerbalaa training hospital 161 V.107/Nº 3 además de la evaluación de los efectos secundarios de los medicamentos. Los cuarenta y un pacientes restantes que rechazaron la terapia con medicamentos los consideramos un grupo de control. Resultado: el grupo de casos que recibió hidroxilurea tuvo crisis principalmente después de 12 semanas desde la última crisis, mientras que el grupo de control tuvo crisis principalmente cada 3 a 7 semanas con un valor P=0,0001. No hubo efectos secundarios en el 77,5% de los casos que recibieron hidroxiurea. El 22,5% restante de los casos tuvo efectos secundarios menores o inespecíficos. Conclusión: En pacientes con drepanocitosis que sufrieron episodios recurrentes de crisis, la terapia con Hidroxiurea disminuye significativamente la frecuencia de la crisis dolorosa, con un bajo nivel de efectos secundarios en comparación con el grupo control.


Background: the antimetabolite drug increase fetal hemoglobin level and reduce the frequency of crisis in sickle cell disease patients. Aim: To evaluate the effect of antimetabolites (hydroxyurea) in cases with frequent sickling crisis of sickle cell disease and non-transfusion dependent thalassemia in Karbala training hospital from APRIL 2016 till December 2020. Patient and methods: from eighty-one patients conducted in this case control study, forty were received hydroxyurea and the other forty-one patients were not. Monitoring every two weeks in the first three months by sending for investigations (Hb, WBC, platelet count and blood urea and serum creatinine) in addition to assessment of drug side effects. The remaining forty-one patients who refused drug therapy we consider them as a control group. Result: the case group who received hydroxylurea had crisis mostly after 12 weeks from last crisis, whereas the control group had crisis mostly each 3 to 7 weeks in P value 0.0001. There was no side effect in 77.5% of cases received hydroxyurea.The remaining 22.5% of cases had less or nonspecific side effects. Conclusion: In patient with sickle cell diseases who suffered from recurrent episodes of crisis, Hydroxyurea therapy significantly decreases the frequency of the painful crisis, with low level of side effects in comparison with control group


Subject(s)
Humans , Fetal Hemoglobin , Case-Control Studies , Clinical Laboratory Techniques , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/pathology , Antimetabolites
17.
Rev. Pesqui. Fisioter ; 11(2): 384-392, Maio 2021. ilus, tab
Article in English, Portuguese | LILACS | ID: biblio-1253542

ABSTRACT

INTRODUÇÃO: O sintoma mais comum na Doença Falciforme (DF) é a dor de alta intensidade que se manifesta desde a infância. Para ser controlada, é necessária a determinação do seu tipo. OBJETIVO: Delinear o perfil da dor em crianças e adolescentes com doença falciforme e analisar o impacto do tipo desta na qualidade de vida. METODOLOGIA: Estudo transversal realizado com crianças e adolescentes com idade entre 8 e 17 anos e diagnóstico de DF HbSS e HbSC (critérios da OMS), em unidades de referência (Salvador, Bahia, Brasil). Foram aplicados a escala infantil de avaliação de dor Adolescent Pediatric Tool (APPT) e o questionário de qualidade de vida PedsQL módulo DF. Foram testadas associações e correlações entre os itens dos instrumentos (Alfa 5%, Beta 20%). CAAE 57274516.8.0000.5544 e 09163419.3.0000.5544. RESULTADOS: Duas amostras, uma com 46 e outra com 44 participantes, com cerca de 60% do tipo HbSS, confirmaram presença de alta intensidade da dor (de 5,0±0,00 a 9,94±0,23 pontos na escala de intensidade de dor) por todo o corpo e uso sistemático de analgésicos (95,5%). Quanto maior a intensidade da dor, principalmente no caso da dor neuropática, maior o total de descritores e o uso de termos sensoriais, afetivos e temporais (p < 0,05), com correlações fortes (r ≥ 0,84; p<0,05) entre intensidade e descritores sensoriais e avaliativos. CONCLUSÃO: A dor em crianças e adolescentes com DF apresenta alta intensidade, com os piores prejuízos para os que referem dor com características neuropáticas.


INTRODUCTION: The most common symptom in sickle cell disease (SCD) is high-intensity pain that manifests in childhood. To be controlled, it is necessary to determine its type. OBJECTIVE: To outline the profile of pain in children and adolescents with sickle cell disease and analyze its impact on quality of life. METHODS: Cross-sectional study was con-ducted with children and adolescents aged 8 to 17 years and diagnosed with DF HbSS and HbSC (WHO criteria) in reference units (Salvador, Bahia, Brazil). The Adolescent Pediatric Tool (APPT) children's pain assessment scale and the PedsQL module DF quality of life questionnaire were applied. Associations and correlations between the items of the instruments were tested (Alpha 5%, Beta 20%). CAAE 57274516.8.0000.5544 and 09163419.3.0000.5544. RESULTS: Two samples, one with 46 and the other with 44 partici-pants, with about 60% of the HbSS type, confirmed the presence of high pain intensity (from 5.0 ± 0.00 to 9.94 ± 0.23 points on the scale pain intensity) throughout the body and systematic use of analgesics (95.5%). The greater the intensity of pain, especially in the case of neuropathic pain, the greater the total number of descriptors and the use of sensory, affective, and temporal terms (p <0.05), with strong correlations (r ≥0.84; p <0,05) between intensity and sensory and evalua-tive descriptors. CONCLUSION: Pain in children and adolescents with SCD is high intensity, with the worst losses for those who report pain with neuropathic characteristics.


Subject(s)
Anemia, Sickle Cell , Pain , Child
18.
Rev. Ciênc. Méd. Biol. (Impr.) ; 20(1): 125-130, maio 5, 2021. fig, tab
Article in Portuguese | LILACS | ID: biblio-1355066

ABSTRACT

Introdução: diante das preocupações causadas pela pandemia do novo Corona vírus e doença relacionada (COVID-19), seja para profissionais de saúde ou da sociedade em geral, estudos relacionados aos riscos para indivíduos com doença falciforme (DF) são ainda escassos. A doença falciforme possui um estado de hipercoagulabilidade, inclusive em pacientes estáveis, sendo assim, a infecção causada pela COVID-19 possivelmente agrava as manifestações fisiopatológicas desses pacientes, podendo ter consequências fatais. Objetivo: identificar eventos tromboembólicos causados pela COVID-19 que são frequentes na DF e podem trazer complicações para esses indivíduos, com consequente aumento do risco de mortalidade. Metodologia: revisão integrativa, onde foram identificados inicialmente 278 estudos, dos quais 264 foram excluídos, sendo então mantidos 14 artigos, os quais foram categorizados conforme o desenho estabelecido para cada um destes estudos, considerando como critérios de qualidade os itens definidos na escala PEDro, associados a outros parâmetros sugestivos de artigos de boa qualidade, como o Índice H, fator de impacto, representatividade dos autores na área do estudo e instituições as quais estes autores estão vinculados. Resultados: com base nas informações descritas, identificou-se o risco aumentado de indivíduos portadores de doença falciforme para o desenvolvimento de eventos tromboembólicos graves diante da exposição à COVID-19. Conclusão: o indivíduo portador de Doença Falciforme diante da infecção pela COVID-19 tem maior risco e suscetibilidade de desenvolvimento de complicações tromboembólicas, consequentemente aumentando suas chances de mortalidade. É de grande importância a realização de estudos clínicos sobre os efeitos tromboembólicos da COVID-19 em pacientes com DF devendo ser incentivados.


Introduction: in view of the concerns caused by the new Coronavirus pandemic and its disease (COVID-19), whether for health professionals or society in general, studies related to the risks for diseases with Sickle Cell Disease (SCD) are still scarce. Sickle cell disease has a state of hypercoagulability, even in stable patients, thus, an infection caused by COVID-19, possibly worsens the pathophysiological manifestations of these patients, which can have fatal consequences. Objective: to identify thromboembolic events caused by COVID-19, which are frequent in the SCD and can cause complications for these individuals, with a consequent increase in the risk of mortality. Methodology: integrative review, in which 278 studies were identified, of which 264 were excluded, and 14 articles were then kept, and they were categorized according to the design established for each of these studies, considering as quality criteria the items written in PEDro scale, associated with other criteria suggestive of good quality articles, such as H Index, impact factor, representativeness of authors in the study area and institutions, such as which these authors are linked to. Results: based on the information described, an increased risk of patients with sickle cell disease for the development of serious thromboembolic events exposed to exposure to COVID-19 was identified. Conclusion: people diagnosed with Sickle Cell Disease if infected with COVID-19 have a higher risk and susceptibility to the development of thromboembolic complications, consequently enhancing their chances of mortality. Clinical studies on thromboembolic effects of COVID-19 in patients with DF are of great importance and must be encouraged.


Subject(s)
Humans , Thromboembolism , COVID-19 , Anemia, Sickle Cell , Review
20.
Hematol., Transfus. Cell Ther. (Impr.) ; 43(1): 87-100, Jan.-Mar. 2021. tab, ilus
Article in English | LILACS | ID: biblio-1154295

ABSTRACT

ABSTRACT Introduction: It is important to know if patients with hemoglobinopathy could be more susceptible to COVID-19. Objective: Analyze SARS-CoV-2 infection in pediatric patients with hemoglobinopathy. Methods: Using the online platforms LILACS, PUBMED and EMBASE, on 17- JUL-2020 a search was made for the terms COVID-19 and SARS-CoV-2 associated with "sickle cell", "thalassemia" and "hemoglobinopathy". Results: There were 623 pediatric and adult patients with sickle cell disease (SCD) or beta thalassemia (BT) and COVID-19. Total mortality rate was 6.42%. No pediatric patient with BT has been described. So, our analysis focused on children and adolescents with SCD: there were 121 pediatric patients, one adolescent died, prophylactic anticoagulation was prescribed to six patients, 11.76% needed intensive care unit, blood transfusion was prescribed in 29.70%. Vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) were the main clinical manifestations in SCD. Discussion: Pediatric patients with SCD and COVID-19 have a low mortality rate when compared to adults, although is higher than the global pediatric population with COVID-19 (0−0.67%). The comorbidities associated with age and the long-term complications inherent to hemoglobinopathies may contribute to the increased mortality outside the pediatric age group. In SCD the clinical manifestations, both in children and adults, are VOC and ACS, and there was increase in blood requirement. Pediatric SCD patients with COVID-19 need more intensive care unit than the global pediatric population (3.30%). Conclusion: Despite pediatric population with SCD needs more intensive care, the outcome after infection by COVID-19 is favorable.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Aged , Thalassemia , Child , Coronavirus , SARS-CoV-2 , COVID-19 , Hemoglobinopathies , Anemia, Sickle Cell
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