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Rev. méd. Maule ; 36(2): 69-73, dic. 2021.
Article in Spanish | LILACS | ID: biblio-1378547


Sickle cell anemia or sickle cell disease is an autosomal recessive disease, caused by a mutation in the hemoglobin gene, where glutamic acid is substituted for valine at position 6 of the beta chain of hemoglobin, resulting in hemoglobin S The diagnosis is made with electrophoresis. The clinical manifestations are varied, the most frequent being the vaso-occlusive crisis, which can increase in pregnancy, during which sickle cell disease also increases the risk of maternal-fetal complications, caused by pre-eclampsia infections, intrauterine growth restriction, and premature delivery. and miscarriage. The usual treatment for the management of seizures is hydroxyurea, a drug that is teratogenic, so its use is contraindicated during pregnancy. Other treatment alternatives are red blood cell transfusion and red blood cell exchange. Next, the first case of red blood cell exchange or exchange transfusion in a pregnant patient with sickle cell anemia at the Hospital Regional de Talca is presented.

Humans , Male , Female , Pregnancy , Infant, Newborn , Adult , Pregnancy Complications, Hematologic/therapy , Exchange Transfusion, Whole Blood , Erythrocyte Transfusion/methods , Anemia, Sickle Cell/therapy , Pregnancy Complications, Hematologic/prevention & control , Pregnancy Outcome , Hydroxyurea/administration & dosage , Anemia, Sickle Cell/prevention & control
Hematol., Transfus. Cell Ther. (Impr.) ; 43(4): 468-475, Oct.-Dec. 2021. tab, graf
Article in English | LILACS | ID: biblio-1350824


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.

Humans , Male , Female , Adolescent , Adult , Middle Aged , Aged , Hydroxyurea , Anemia, Sickle Cell , Tumor Necrosis Factor-alpha , Oxidative Stress , Cytotoxicity, Immunologic , Dosage , Inflammation , Malondialdehyde , Neutrophils
Prensa méd. argent ; 107(3): 157-161, 20210000. tab
Article in English | LILACS, BINACIS | ID: biblio-1359922


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

Humans , Fetal Hemoglobin , Case-Control Studies , Clinical Laboratory Techniques , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/pathology , Antimetabolites
Adv Rheumatol ; 61: 11, 2021. tab
Article in English | LILACS | ID: biblio-1152745


Abstract Background: Sickle cell disease (SCD) is an autosomal recessive genetic disease in which a mutation occurs in the β-globin chain gene, resulting in abnormal hemoglobin levels. In an environment with reduced oxygen concentration, red blood cells change their conformation, resulting in chronic hemolysis and consequent anemia and vaso-occlusive crises with injuries to several organs, with a significant impairment of the osteoarticular system. This study aimed to verify the chronic osteoarticular alterations and their association with clinical and laboratory characteristics of patients with SCD with a more severe phenotype (SS and Sβ0), on a steady-state fasis. Methods: Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. Results: In total, 74.5% patients had hemoglobinopathy SS; 67.3% were female; and 78.2% were non-whites. The mean patient age was 30.5 years. Most patients (61.8%) reported up to three crises per year, with a predominance of high-intensity pain (65.5%). Radiographic alterations were present in 80% patients. A total of 140 lesions were identified, most which were located in the spine, femur, and shoulders. Most lesions were osteonecrosis and osteoarthritis and were statistically associated with the non-use of hydroxyurea. Conclusions: There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.(AU)

Humans , Osteoarthritis/etiology , Osteonecrosis/etiology , Bone Diseases, Metabolic/etiology , Hydroxyurea/administration & dosage , Anemia, Sickle Cell/physiopathology , Prognosis , Cross-Sectional Studies/instrumentation , Risk Factors , Hydroxyurea/adverse effects
Acta Paul. Enferm. (Online) ; 34: eAPE01641, 2021. tab, graf
Article in Portuguese | LILACS, BDENF | ID: biblio-1152651


Resumo Objetivo: Analisar os estudos econômicos completos com enfoque nos tratamentos da Anemia Falciforme. Métodos: Estudo de revisão integrativa de literatura desenvolvido mediante coleta de dados nas bases eletrônicas National Library of Medicine - Medline via PubMed; Elservier's Scopus; Current Index to Nursing and Allied Health Literature; Science Direct e Web of Science com descritores indexados no Medical Subject Headings. Os estudos foram selecionados pelo teste de relevância e analisados de acordo com a classificação das análises econômicas em saúde e o sistema de Classificação da qualidade das evidências e a força das recomendações. Resultados: Fizeram parte desta revisão 09 artigos, dos quais sete recuperados na base Elservier's Scopus e dois na Medline via PubMed. Todos estudos completos com enfoque nas perspectivas do uso da Hidroxiureia e da transfusão sanguínea no tratamento da Anemia Falciforme. Conclusão: Não foram identificados estudos realizados no Brasil com este tipo de análise para Anemia Falciforme. Há muito a ser feito mundialmente para avaliação das tecnologias vigentes, reavaliação das utilizadas atualmente e implementação de diagnóstico e tratamento contínuo, com um sistema que garanta uma rede de atenção ativa e eficiente aos pacientes.

Resumen Objetivo: Analizar los estudios económicos completos con enfoque en tratamientos para la anemia falciforme Métodos: Estudio de revisión integradora de la literatura desarrollado mediante la recolección de datos en las bases electrónicas National Library of Medicine - Medline vía PubMed; Elservier's Scopus; Current Index to Nursing and Allied Health Literature; Science Direct y Web of Science con descriptores indexados en Medical Subject Headings. Los estudios fueron seleccionados mediante la prueba de relevancia y analizados de acuerdo con la clasificación de análisis económicos en salud y con el sistema de clasificación de la calidad de las evidencias y la fuerza de las recomendaciones. Resultados: Nueve artículos formaron parte de esta revisión, de los cuales siete fueron encontrados en la base Elservier's Scopus y dos en Medline vía PubMed. Todos son estudios completos con enfoque en las perspectivas del uso de hidroxiurea y transfusión sanguínea para el tratamiento de anemia falciforme. Conclusión: No se identificaron estudios realizados en Brasil con este tipo de análisis de anemia falciforme. Hay mucho por hacer a nivel mundial para evaluar las tecnologías vigentes, revaluar las que se utilizan en la actualidad e implementar el diagnóstico y tratamiento continuo, con un sistema que garantice una red de atención activa y eficiente para los pacientes.

Abstract Objective: To analyze complete economic studies focusing on sickle cell anemia treatments. Methods: Integrative literature review developed by collecting data in the electronic databases National Library of Medicine - Medline via PubMed; Elsevier's Scopus; Current Index to Nursing and Allied Health Literature; Science Direct and Web of Science with descriptors indexed in Medical Subject Headings. The studies were selected by the relevance test and analyzed according to the classification of economic analyses in health and the classification system of evidence quality and strength of recommendations. Results: Nine articles were part of this review, seven of which were retrieved from Elsevier's Scopus and two from Medline via PubMed. All articles reported on studies focusing on the perspectives of using hydroxyurea and blood transfusion in the treatment of sickle cell anemia. Conclusion: No studies were identified that were conducted in Brazil and involved this type of analysis for sickle cell anemia. Much remains to be done worldwide to assess existing technologies, reassess the technologies currently used and implement continuous diagnosis and treatment, by means of a system that guarantees an active and efficient care network for the patients.

Health Care Economics and Organizations , Blood Transfusion , Databases, Bibliographic , Cost-Benefit Analysis , Costs and Cost Analysis , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/drug therapy , Anemia, Sickle Cell/therapy
Rev. Paul. Pediatr. (Ed. Port., Online) ; 39: e2019365, 2021. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1155471


ABSTRACT Objective: To describe two cases of unusual variants of sickle cell disease. Case description: We present two cases of sickle cell disease variants (haemoglobinopathies), from unrelated families, in the state of Balochistan (Pakistan). One was diagnosed with sickle cell disease in the haemoglobin electrophoresis, whereas the other was diagnosed with sickle cell SE disease. Both were diagnosed based on the presentation of osteomyelitis. Comments: Haemoglobin SD disease (Hb SD) and haemoglobin SE disease (Hb SE) are rare haemoglobinopathies in the world. The lack of available literature suggests that both are variants of sickle cell disease (SCD), with heterogeneous nature. The prevalence of sickle cell disease with compound heterozygotes was found at a variable frequency in the population of the Asian Southeast. The frequency of osteomyelitis in SCD is 12 to 18%, but its occurrence among variant haemoglobinopathies is little reported. Both reported cases presented with osteomyelitis as a characteristic of the disease presentation.

RESUMO Objetivo: Descrever dois casos de variantes raras da hemoglobinopatia falciforme. Descrição do caso: Apresentamos aqui dois casos de hemoglobinopatias variantes das células falciformes, de famílias não relacionadas, no estado do Baluchistão (Paquistão), sendo um diagnosticado como doença da hemoglobina SD na eletroforese de hemoglobina, enquanto o outro com doença da hemoglobina SE. Ambos foram diagnosticados a partir da apresentação de osteomielite. Comentários: Hemoglobina SD (Hb SD) e hemoglobina SE (Hb SE) são hemoglobinopatias raras no mundo. A escassez de literatura disponível sugere que ambas são variantes da doença falciforme (DF) com natureza heterogênea. A prevalência de hemoglobinopatia falciforme com heterozigosidade composta foi encontrada com frequência variável na população do sudeste asiático. A frequência de osteomielite na DF é de 12 a 18%, mas sua ocorrência entre as hemoglobinopatias falciformes variantes é pouco relatada. Os dois casos reportados apresentaram osteomielite como característica de apresentação da doença.

Humans , Male , Female , Child , Osteomyelitis/diagnosis , Blood Protein Electrophoresis/methods , Hemoglobinopathies/genetics , Anemia, Sickle Cell/diagnosis , Anemia, Sickle Cell/genetics , Osteomyelitis/etiology , Osteomyelitis/drug therapy , Pakistan/ethnology , Magnetic Resonance Imaging/methods , Radiography/methods , Mass Screening/standards , Mass Screening/ethics , Prevalence , Administration, Oral , Treatment Outcome , Administration, Intravenous , Hemoglobinopathies/diagnosis , Hemoglobinopathies/blood , Heterozygote , Hydroxyurea/administration & dosage , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Anti-Bacterial Agents/administration & dosage , Anti-Bacterial Agents/therapeutic use , Antisickling Agents/administration & dosage , Antisickling Agents/therapeutic use
Esc. Anna Nery Rev. Enferm ; 25(1): e20190337, 2021.
Article in Portuguese | LILACS, BDENF | ID: biblio-1124790


RESUMO Objetivo analisar a internalização do cuidado com o corpo pelo escolar com a doença falciforme com base na teoria do desenvolvimento de Vigotski e no conceito de cuidado de Collière. Método Estudo qualitativo com 15 escolares que convivem com a doença falciforme, acompanhados em ambulatório na cidade de Vitoria - ES. A técnica utilizada foi a entrevista individual e a análise temática. A hidratação corporal, o brincar, a prevenção e manejo da crise falcêmica, a alimentação e as roupas foram as unidades temáticas que emergiram. Resultados Os participantes referiram ingerir variados tipos de líquidos. As brincadeiras foram predominantemente ativas. Os medicamentos foram de reparação e manutenção da saúde. Não se evidenciou consumo de alimentos saudáveis. Observou-se a utilização de roupas adequadas ao frio. A dor foi um signo da internalização do cuidado e do conhecimento para brincadeiras. A diminuição de líquidos e roupas inadequadas desencadearam a crise falcêmica. Considerações Finais Evidenciaram-se a internalização do conhecimento e dos cuidados mediados pela dor e o despreparo dos professores pela falta de conhecimento. Implicações para a prática este estudo poderá subsidiar a melhor articulação entre profissional de saúde, criança e escola.

RESUMEN Objetivo analizar la internalización de la atención por parte del escolar con la enfermedad de células falciformes basado en la teoría del desarrollo de Vigotski y el concepto de atención de Collière. Método Estudio cualitativo con 15 escolares que viven con la enfermedad de células falciformes, monitoreados en una clínica ambulatoria en la ciudad de Vitoria - ES. La técnica fue la entrevista individual y el análisis temático. La hidratación corporal, el juego, la prevención y el manejo de la crisis falcémica, la alimentación y la ropa fueron las unidades temáticas que emergieron. Resultados Los participantes informaron de la ingestión de varios tipos de líquidos. Los juegos fueron predominantemente activos. Los medicamentos fueron de reparación y mantenimiento de la salud. No se ha demostrado el consumo de alimentos saludables. Se observó el uso de ropa adecuada para el frío. El dolor fue un signo de la internalización de la atención y el conocimiento para los juegos. La disminución de líquidos y la ropa inadecuada desencadenaron la crisis falcémica. Consideraciones finales Se señalaron la internalización del conocimiento y la atención mediados por el dolor y la falta de preparación de los maestros debido a la falta de conocimiento. Implicaciones para la práctica este estudio podrá subsidiar la mejor articulación entre los profesionales de la salud, los niños y la escuela.

ABSTRACT Objective to analyze the internalization of body care by the schoolchildren with sickle cell disease based on Vigotski's development theory and Collière's concept of care. Method Qualitative study with 15 schoolchildren living with sickle cell disease, followed in an outpatient clinic in the city of Vitoria - ES. The technique was the individual interview and thematic analysis. Body hydration, playing, prevention and management of the sickle cell crisis, food and clothing were the thematic units that emerged. Results Participants reported ingesting various types of liquids. The games were predominantly active. The medications were repair and maintenance of health. It was observed no consumption of healthy foods. The use of clothes suitable for the cold was evidenced. Pain was a sign of the internalization of care and knowledge for games. The decrease in liquids and inadequate clothing triggered the sickle cell crisis. Final Considerations The internalization of knowledge and care mediated by pain and the unpreparedness of teachers due to lack of knowledge were highlighted. Implications for practice this study can support the best articulation between health professionals, children and school.

Humans , Male , Female , Child , Self Care , Anemia, Sickle Cell/prevention & control , Pain/drug therapy , Play and Playthings , Dehydration , Qualitative Research , Drinking , Eating , Folic Acid/therapeutic use , Hydroxyurea/therapeutic use , Analgesics/therapeutic use
Rev. bras. ciênc. vet ; 27(3): 115-119, jul./set. 2020. il.
Article in Portuguese | LILACS, VETINDEX | ID: biblio-1370655


O objetivo deste relato é apresentar o caso clínico de uma cadela, sem raça definida, com cinco anos de idade, diagnosticada com leucemia mieloide crônica (LMC). As leucemias são neoplasias malignas que se originam de células precursoras da medula óssea e as consequências podem ser trombocitopenia, anemia, leucocitose persistente e presença de células neoplásicas no sangue. O tratamento de escolha envolve o uso de inibidores de tirosina quinase, porém este não pode ser usado neste caso. Dessa forma a cadela recebeu diferentes protocolos quimioterápicos que incluíram inicialmente hidroxiureia, citarabina, doxorrubicina e prednisona. Devido a remissão parcial dos sinais clínicos e a resposta terapêutica pouco duradoura a essas medicações o protocolo foi alterado para quimioterapia metronômica com clorambucil. O uso desses quimioterápicos não foram eficazes em reduzir a leucocitose e controlar a anemia e trombocitopenia da paciente, devido a ocorrência do surgimento de células imaturas no sangue e resistência aos quimioterápicos. Na ausência da crise e da possibilidade do uso dos inibidores de tirosina quinase, a hidroxiureia permanece sendo o quimioterápico de eleição. O animal apresentou sobrevida de 210 dias, devido a leucocitose e anemia severas pouco responsivas ao protocolo terapêutico utilizado e o surgimento no hemograma de precursores neutrofilicos que ocorreu 46 dias após ao início do tratamento com hidroxiureia.

The aim of this report is to present the clinical case of a five-year-old mixed breed female dog diagnosed with chronic myeloid leukemia (CML). Leukemias are malignant neoplasms that originate from bone marrow precursor cells and the consequences can be thrombocytopenia, anemia, persistent leukocytosis and the presence of neoplastic cells in the blood. The treatment of choice involves the use of tyrosine kinase inhibitors, but it cannot be used in this case. Thus, the dog received different chemotherapy protocols that initially included hydroxyurea, cytarabine, doxorubicin and prednisone. Due to the partial remission of clinical signs and the short-term therapeutic response to these medications, the protocol was changed to metronomic chemotherapy with chlorambucil. The use of these chemotherapeutic agents was not effective in reducing leukocytosis and controlling the patient's anemia and thrombocytopenia, due to the occurrence of immature cells in the blood and resistance to chemotherapeutic agents. In the absence of the crisis and the possibility of using tyrosine kinase inhibitors, hydroxyurea remains the chemotherapy of choice. The animal had a 210-day survival, due to severe leukocytosis and anemia, which were not responsive to the therapeutic protocol used and the appearance in the blood count of neutrophilic precursors that occurred 46 days after the beginning of hydroxyurea treatment.

Animals , Dogs , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/veterinary , Drug Resistance, Neoplasm/drug effects , Dogs/immunology , Drug Therapy/veterinary , Hydroxyurea/therapeutic use , Antineoplastic Agents/therapeutic use , Survival , Anemia/veterinary , Leukocytosis/veterinary
Brasília; s.n; 30 jun. 2020.
Non-conventional in Portuguese | LILACS, BRISA, Inca, PIE | ID: biblio-1117603


O Informe Diário de Evidências é uma produção do Ministério da Saúde que tem como objetivo acompanhar diariamente as publicações científicas sobre tratamento farmacológico e vacinas para a COVID-19. Dessa forma, são realizadas buscas estruturadas em bases de dados biomédicas, referente ao dia anterior desse informe. Não são incluídos estudos pré-clínicos (in vitro, in vivo, in silico). A frequência dos estudos é demonstrada de acordo com a sua classificação metodológica (revisões sistemáticas, ensaios clínicos randomizados, coortes, entre outros). Para cada estudo é apresentado um resumo com avaliação da qualidade metodológica. Essa avaliação tem por finalidade identificar o grau de certeza/confiança ou o risco de viés de cada estudo. Para tal, são utilizadas ferramentas já validadas e consagradas na literatura científica, na área de saúde baseada em evidências. Cabe ressaltar que o documento tem caráter informativo e não representa uma recomendação oficial do Ministério da Saúde sobre a temática. Foram encontrados 14 artigos e 31 protocolos.

Humans , Pneumonia, Viral/drug therapy , Coronavirus Infections/drug therapy , Betacoronavirus/drug effects , Technology Assessment, Biomedical , Vitamin D/therapeutic use , Ivermectin/therapeutic use , Immunoglobulins/therapeutic use , Prednisone/therapeutic use , BCG Vaccine/therapeutic use , Influenza Vaccines/therapeutic use , Azithromycin/therapeutic use , Antirheumatic Agents/therapeutic use , Ritonavir/therapeutic use , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Lopinavir/therapeutic use , Janus Kinase Inhibitors/therapeutic use , Glucocorticoids/therapeutic use , Hydroxychloroquine/therapeutic use , Hydroxyurea/therapeutic use , Immunosuppressive Agents/therapeutic use
Brasília, DF; Ministério da Saúde; 1; 2020. 51 p. ilus.
Monography in Portuguese | LILACS, ColecionaSUS, Inca, PIE | ID: biblio-1099590


Essa síntese teve como objetivo levantar opções para prevenção de complicações da doença falciforme, e informar sobre avanços na implementação da política no país. Seguem as opções descritas na síntese que já fazem parte das recomendações do Ministério da Saúde: Promover a antibioticoterapia profilática e a vacinação anti-pneumocócica; Promover o uso de hidroxiureia para prevenção e tratamento de complicações da doença falciforme; Promover o uso de ultrassonografia Doppler Transcraniano (DTC) e transfusão sanguínea para a prevenção primária de acidente vascular cerebral (AVC); Promover a educação em saúde para as pessoas com doença falciforme e seus cuidadores sobre medidas para reduzir as complicações

Humans , Antibiotic Prophylaxis , Pneumococcal Vaccines , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/prevention & control , Anemia, Sickle Cell/therapy , Blood Transfusion , Health Education , Ultrasonography, Doppler, Transcranial
Acta cir. bras ; 35(3): e202000301, 2020. tab, graf
Article in English | LILACS | ID: biblio-1130626


Abstract Purpose: To analyze the serum levels of nitric oxide and correlate them with the levels of thiobarbituric acid reactive substances (TBARS) in liver, brain and spinal cord of animals using L-NAME and treated with hydroxyurea. Methods: Eighteen male albino Wistar rats were divided into three groups. NG-nitro-L-arginine methyl ester (L-NAME) was intraperitoneally administered to induce oxidative stress. TBARS and plasma nitric oxide levels were analyzed in all groups. Histopathology of the liver and vascular tissue was performed. Results: Statistically significant differences were seen in liver, brain and spinal cord TBARS levels. Conclusions: Following the use of L-NAME, hepatic tissue increased the number of Kupffer cells as oxidative stress and inflammatory response increased. The use of L-NAME caused an increase in lipid peroxidation products and, consequently, in oxidative stress in animals. Hydroxyurea doses of 35 mg / kg / day reduced TBARS values in liver, brain and spinal cord.

Animals , Male , Rats , Spinal Cord/metabolism , Brain/metabolism , Oxidative Stress/physiology , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/drug therapy , Liver/metabolism , Rats, Wistar , NG-Nitroarginine Methyl Ester , Disease Models, Animal , Anemia, Sickle Cell/physiopathology , Anemia, Sickle Cell/metabolism
Ann. afr. méd. (En ligne) ; 13(4): 3849-3860, 2020. tab
Article in French | AIM | ID: biblio-1259095


Contexte et objectifs. L'hydroxyurée est connu efficace dans la prise en charge des crises drépanocytaires. L'objectif de la présente étude était d'établir la disponibilité et d'évaluer le cout moyen de ce médicament en République Démocratique du Congo (RDC). Méthodes. Une étude transversale descriptive a été réalisée dans 188 pharmacies de deux villes (une grande, Lubumbashi versus une enclavée, Mbuji Mayi en RDC, entre les 1er avril et 1er septembre 2017. Seules des questions fermées ont été posées, avec des choix soit dichotomiques soit multiples. Résultats. L'hydroxyurée n'était disponible que dans 22% des pharmacies participantes (41/188) et beaucoup plus fréquemment dans celles d'une grande ville que dans celles d'une ville reculée (34/96 contre 7/92). La plupart des patients ont présenté une ordonnance médicale (36/41 soit 88% d'entre eux) pour obtenir le médicament. Le prix de l'hydroxyurée variait entre 10 et 35 $, avec un prix moyen de 15 $ pour une boîte de 25 gélules. Ce prix est onéreux pour le pouvoir d'achat de la majorité des patients drépanocytaires. Conclusions. Cette étude montre que l'hydroxyurée est à la fois peu disponible dans les pharmacies en RDC et peu accessible financièrement pour beaucoup de patients drépanocytaires

Anemia, Sickle Cell/therapy , Democratic Republic of the Congo , Disease Management , Health Care Costs , Hydroxyurea
Journal of Experimental Hematology ; (6): 1424-1428, 2020.
Article in Chinese | WPRIM | ID: wpr-827100


β-thalassaemias are inherited hemoglobin disorders caused by defects in the β-globin gene. In recent years, researches have re-mentioned the therapeutic significance of drug-induced fetal hemoglobin (HbF), which can reduce the imbalance of α and β chains and improve the severity of anemia by increasing the expression of γ chain. Drug trials, such as hydroxyurea, thalidomide and desitabine have shown elevated hemoglobin, decreased blood transfusion dependence, and reduced symptoms other than anemia after treatment. In addition, in vitro experiments suggested that HbF can also induce by other drugs, which providing important clues for safe and effective HbF inducers. Therefore, this article reviews the current research progress so as to expect beneficial to clinical treatment.

Blood Transfusion , Fetal Hemoglobin , Humans , Hydroxyurea , beta-Globins , beta-Thalassemia
Hematol., Transfus. Cell Ther. (Impr.) ; 41(2): 125-128, Apr.-June 2019. tab
Article in English | LILACS | ID: biblio-1012180


ABSTRACT We analyzed the management and outcomes of pregnancies of patients with chronic myeloid leukemia at a single center over fifteen years. Among the 203 CML female patients, there were ten pregnancies in seven women, all of them not planned. In three cases, the chronic myeloid leukemia diagnosis was made during pregnancy. Five patients received tyrosine kinase inhibitors in the first weeks of pregnancy and the drug was interrupted until delivery. One patient lost complete cytogenetic response, and two patients lost the hematological response. A patient with a stable major molecular response had two successful pregnancies without loss of response. There were four premature births. There were no maternal adverse events, fetal malformation or death. All patients received Interferon-alpha during gestation, and two received hydroxyurea for a short period. Leukapheresis was performed in two patients for hyperleukocytosis control. One patient with sickle cell disease died from disease progression six months after delivery. Conclusions: The tyrosine kinase inhibitors ministration should be interrupted during pregnancy. Patients should be advised to achieve a stable and deep molecular response if they plan to conceive, to avoid the risk of disease progression.

Humans , Female , Pregnancy , Pregnancy , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Interferon-alpha , Imatinib Mesylate , Dasatinib , Hydroxyurea
Hematol., Transfus. Cell Ther. (Impr.) ; 41(2): 114-118, Apr.-June 2019. tab, graf, ilus
Article in English | LILACS | ID: biblio-1012185


ABSTRACT Introduction and objective: In this study, we evaluated the influence of the transcript type on hematological and clinical parameters, as well as the event-free survival of 50 patients in the Chronic myeloid leukemia chronic phase. Methods: We reviewed the medical records of 55 patients with Chronic myeloid leukemia. The eligibility criteria were based on the availability of hematological and clinical baseline data in the medical records. Data on BCR-ABL transcripts were obtained from medical records. Results: Eighteen patients (36%) had the b2a2 transcript, 24 (48%) had b3a2 and 8 (16%) had b2a2/b3a2. The median platelet count for transcripts b2a2, b3a2 and b2a2/b3a2 was 320.65 × 103/L, 396 × 103/L, and 327.05 × 103/L, respectively (p = 0.896). We could not find any differences in relation to the other hematological parameters, when compared to the transcript type. Comparison between spleen and liver size and type of transcript did not differ inside the groups (p = 0.395 and p = 0.647, respectively) and the association between risk scores and transcript type did not show statistical significance (p > 0.05). The 21-month probability for event-free survival was 21%, 48% and 66% for the transcripts b2a2, b3a2 and b2a2/b3a2 respectively (p = 0.226) Conclusion: We conclude that the expression BCR-ABL transcripts have no influence on hematological, clinical and event-free survival parameters of patients in the Chronic myeloid leukemia chronic phase.

Humans , Prognosis , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Fusion Proteins, bcr-abl , Hydroxyurea/therapeutic use
Einstein (Säo Paulo) ; 17(4): eAO4742, 2019. tab, graf
Article in English | LILACS | ID: biblio-1019812


ABSTRACT Objective To evaluate the induction of DNA damage in peripheral blood mononuclear cells of patients with sickle cell disease, SS and SC genotypes, treated with hydroxyurea. Methods The study subjects were divided into two groups: one group of 22 patients with sickle cell disease, SS and SC genotypes, treated with hydroxyurea, and a Control Group composed of 24 patients with sickle cell disease who were not treated with hydroxyurea. Peripheral blood samples were submitted to peripheral blood mononuclear cell isolation to assess genotoxicity by the cytokinesis-block micronucleus cytome assay, in which DNA damage biomarkers - micronuclei, nucleoplasmic bridges and nuclear buds - were counted. Results Patients with sickle cell disease treated with hydroxyurea had a mean age of 25.4 years, whereas patients with sickle cell disease not treated with hydroxyurea had a mean age of 17.6 years. The mean dose of hydroxyurea used by the patients was 12.8mg/kg/day, for a mean period of 44 months. The mean micronucleus frequency per 1,000 cells of 8.591±1.568 was observed in the Hydroxyurea Group and 10.040±1.003 in the Control Group. The mean frequency of nucleoplasmic bridges per 1,000 cells and nuclear buds per 1,000 cells for the hydroxyurea and Control Groups were 0.4545±0.1707 versus 0.5833±0.2078, and 0.8182±0.2430 versus 0.9583±0.1853, respectively. There was no statistically significant difference between groups. Conclusion In the study population, patients with sickle cell disease treated with the standard dose of hydroxyurea treatment did not show evidence of DNA damage induction.

RESUMO Objetivo Avaliar o efeito da indução de danos ao DNA em células monocelulares do sangue periférico de pacientes com doença falciforme, genótipos SS e SC, tratados com hidroxiureia. Métodos Os sujeitos da pesquisa foram divididos em dois grupos: um de 22 pacientes com doença falciforme genótipos SS e SC tratados com hidroxiureia, e o outro controle, composto por 24 pacientes com doença falciforme que não eram tratados com o fármaco. As amostras de sangue periférico foram submetidas ao isolamento de células mononucleares do sangue periférico para avaliação da genotoxicidade pelo ensaio de micronúcleo citoma com bloqueio da citocinese, tendo sido quantificados os biomarcadores de danos ao DNA - micronúcleos, pontes nucleoplasmáticas e brotamento nuclear. Resultados Os pacientes com doença falciforme tratados com hidroxiureia apresentaram média de idade de 25,4 anos, enquanto aqueles com doença falciforme não tratados com hidroxiureia tiveram média de idade de 17,6 anos. A dose média de hidroxiureia utilizada pelos pacientes foi de 12,8mg/kg/dia, por período médio de 44 meses. A frequência média de micronúcleos por 1.000 células de 8,591±1,568 foi observada no Grupo Hidroxiureia e de 10,040±1,003 no Grupo Controle. Adicionalmente, a frequência média de pontes nucleoplasmáticas por 1.000 células e brotamento nuclear por 1.000 células para o Grupo Hidroxiureia e Controle foi de 0,4545±0,1707 versus 0,5833±0,2078, e de 0,8182±0,2430 versus 0,9583±0,1853, respectivamente. Não houve diferença estatisticamente significativa entre os grupos. Conclusão Na população estudada de pacientes com doença falciforme com tratamento em dose padrão de hidroxiureia, não houve evidência de indução de danos ao DNA.

Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Young Adult , DNA Damage/drug effects , Nucleic Acid Synthesis Inhibitors/pharmacology , Hydroxyurea/pharmacology , Anemia, Sickle Cell/genetics , DNA Damage/genetics , Micronucleus Tests , Nucleic Acid Synthesis Inhibitors/adverse effects , Nucleic Acid Synthesis Inhibitors/therapeutic use , Cytokinesis , Hydroxyurea/adverse effects , Hydroxyurea/therapeutic use , Anemia, Sickle Cell/drug therapy , Middle Aged , Mutagenicity Tests , Mutation/drug effects
Article in Chinese | WPRIM | ID: wpr-771926


OBJECTIVE@#To explore the symptomatic burden of patients with essential thrombocythemia (ET) and its relation with clinical characteristics including the mutation status, therapeutic protocols and sex.@*METHODS@#Total of 173 Chinese ET patients were selected and grouped on the basis of disease characteristics (mutation status, therapeutic pro to- cols, and sex).@*RESULTS@#All the groups showed low-to-high symptom burden, with the highest in the Hu (hydroxyurea)-group (total symptom score [TSS], 14.7; range, 7.6-14.7). In the JAK2V617F-positive, Hu-treated, and female groups TSS and independent symptom scores were higher than those in the control group. The CALR-positive and IFN-α-treated groups had lower overall and individual scores as compared with groups lacking the corresponding characteristics. As the number of characteristics (JAK2V617F-positive, Hu-treated, and female) increases, the severity of symptoms gradually increased.@*CONCLUSION@#The different characteristics have various effects on symptom burden in ET patients. The accumulation of certain characteristics will lead to more severe symptom burden, thus the patient's symptom burden should be considered comprehensively when making up the treatment schemes and prognosis.

Asians , Calreticulin , Female , Humans , Hydroxyurea , Janus Kinase 2 , Mutation , Thrombocythemia, Essential
J. pediatr. (Rio J.) ; 94(6): 666-672, Nov.-Dec. 2018. tab, graf
Article in English | LILACS | ID: biblio-976014


Abstract Objectives: Hemoglobin SC is the second most common variant of sickle-cell disease worldwide, after hemoglobin SS. The objectives of the study were to describe the clinical and laboratory characteristics of hemoglobin SC disease in children from a newborn screening program and treated at a blood center. Methodology: This study assessed a cohort of 461 infants born between 01/01/1999 and 12/31/2012 and followed-up until 12/31/2014. Clinical events were expressed as rates for 100 patient-years, with 95% confidence intervals. Kaplan-Meier survival curves were created. Results: The median age of patients was 9.2 years; 47.5% were female. Mean values of blood tests were: hemoglobin, 10.5 g/dL; reticulocytes, 3.4%; white blood cells, 11.24 × 109/L; platelets, 337.1 × 109/L; and fetal hemoglobin, 6.3%. Clinical events: acute splenic sequestration in 14.8%, blood transfusion 23.4%, overt stroke in 0.2%. The incidence of painful vaso-occlusive episodes was 51 (48.9-53.4) per 100 patient-years and that of infections, 62.2 episodes (59.8-64.8) per 100 patient-years. Transcranial Doppler ultrasonography (n = 71) was normal given the current reference values for SS patients. Hydroxyurea was given to ten children, all of whom improvement of painful crises. Retinopathy was observed in 20.3% of 59 children who underwent ophthalmoscopy. Avascular necrosis was detected in seven of 12 patients evaluated, predominantly in the left femur. Echocardiogram compatible with pulmonary hypertension was recorded in 4.6% of 130 children, with an estimated average systolic pulmonary artery pressure of 33.5 mmHg. The mortality rate from all causes was 4.3%. Conclusions: Clinical severity is variable in SC hemoglobinopathy. Several children have severe manifestations similar to those with SS disease.

Resumo Objetivos: A hemoglobinopatia SC é a segunda variante mais comum da doença falciforme no mundo, após a hemoglobinopatia SS. Os objetivos do estudo foram descrever as características clínicas e laboratoriais da hemoglobinopatia SC em recém-nascidos diagnosticados por programa de triagem neonatal e encaminhados para acompanhamento em hemocentro. Metodologia: Coorte de 461 recém-nascidos SC nascidos entre 01/01/1999 e 31/12/2012 e seguidos até 31/12/2014. A incidência de eventos clínicos foi expressa por taxas relativas a 100 pacientes-ano, com limites de confiança a 95%. Curvas de sobrevida foram construídas segundo Kaplan-Meier. Resultados: Mediana de idade, 9,2 anos; 47,5%, feminino. Médias dos valores hematológicos: hemoglobina 10,5 g/dL; reticulócitos 3,4%; leucometria 11,24 x 109/L; plaquetometria 337,1x109/L; hemoglobina fetal 6,3%. Eventos clínicos: sequestro esplênico agudo em 14,8%, hemotransfusão 23,4%, AVC isquêmico 0,2%. A incidência de episódios vaso-oclusivos dolorosos foi de 51 (48,9-53,4) por 100 pacientes-ano; a de infecções, 62,2 episódios (59,8-64,8) por 100 pacientes-ano. Doppler transcraniano (n = 71) foi normal, se usados os valores de referência de crianças SS. Dez pacientes usaram hidroxiureia, todos com melhoria das crises dolorosas. Retinopatia foi observada em 20,3% das 59 crianças que fizeram fundoscopia. Necrose avascular foi detectada em 7 de 12 pacientes avaliados, com predomínio no fêmur esquerdo. Ecocardiograma compatível com hipertensão pulmonar foi registrado em 4,6% de 130 crianças, com média estimada de 33,5 mm Hg de pressão arterial pulmonar. A taxa de mortalidade por todas as causas foi de 4,3%. Conclusões: A hemoglobinopatia SC tem gravidade variável; várias crianças apresentam manifestações clínicas intensas, semelhantes às da hemoglobinopatia SS.

Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Hemoglobin SC Disease/blood , Hemoglobin SC Disease/epidemiology , Splenic Diseases/pathology , Splenic Diseases/epidemiology , Time Factors , Brazil/epidemiology , Incidence , Retrospective Studies , Age Factors , Neonatal Screening , Ultrasonography, Doppler, Transcranial , Kaplan-Meier Estimate , Hemoglobin SC Disease/pathology , Hemoglobin SC Disease/drug therapy , Hydroxyurea/therapeutic use , Antisickling Agents/therapeutic use