Costs related to obtaining organs for transplantation: A systematic review.

INTRODUCTION: The number of transplants in the world is growing, although there is a demand that exceeds supply. It is worth mentioning that the costs for obtaining organs are considered high. However, few studies have been developed on analyzing the costs of obtaining organs and tissues for transplants in order to support the decision-making of managers and health professionals. OBJECTIVE: To summarize the studies related to the cost of obtaining organs for transplants from a deceased donor. METHOD: A systematic literature review was conducted in the following databases: PubMed, Cochrane Library CINAHAL, Virtual Health Library (BVS), SCOPUS, Web of Science and EMBASE, using the following descriptors: Costs and cost analysis; Donor Selection; Tissue and Organ Procurement; Tissue and Organ Harvesting; and Tissue Donors, in studies published until April 2021. The risk of bias assessment was performed using the Joanna Briggs Institute's Checklist for Economic Assessments. It was not possible to perform a meta-analysis due to the heterogeneity of the studies. RESULTS: A total of 1731 studies were identified, of which 11 were analyzed. The cost of kidneys in US dollars (USD) ranged between USD $1672 and USD $25,058. Obtaining a liver ranged from USD $586 to USD $44,478. Heart procurement ranged from USD $633 to USD $24,264. The combined heart-lung transplant ranged from USD $860 to USD $23,203. Obtaining the pancreas ranged from USD $413 to USD $29,708. CONCLUSIONS: Cost of obtaining organs for transplants from a deceased donor is substantial and varies widely across different studies. The overall cost of failures to obtain organs is currently unknown. Understanding organ procurement expenses can help clarify areas in which organ and tissue procurement can improve in cost and efficiency.

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018.

PURPOSE: To analyze the clinical-epidemiological characteristics and mortality in patients with sickle-cell anemia (SCA). PATIENTS AND METHODS: A cohort study with retrospective data, conducted in two reference hospitals for SCA treatment from January 1980 to December 2018, recorded in two reference services. With a 5% significance level, the Chi-Square and Student's t-tests were employed in the inferential statistical analysis. RESULTS: A total of 128 patients with SCA were studied. Diagnosis up to the fifth day of life was made in 10 patients. There were 19 deaths, of which 12 (63.2%) were female, and the average age at death was 27.05 (± 14.78) years. The leading causes of death were septic shock and cardiogenic shock. The use of invasive medical devices was considered a risk factor for death (RR=2.63; 95% CI=1.16-5.96; p=0.018), and monitoring time up to 20 years presented a 31% reduction in the risk of dying (RR=0.31; 95% CI=0.12-0.82; p=0.011) when compared to the monitoring of more than 20 years. CONCLUSION: These findings are to be considered in the treatment of patients with SCA, mainly regarding early diagnosis and access to the treatment immediately afterward, since they are fundamental in improving survival and reducing severe complications.

Effects of oxidative stress on liver, brain and spinal cord of rats using L-NAME and treated with hydroxyurea. A model of sickle cell complication.

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.

Quality of life in adults with sickle cell disease: an integrative review of the literature.

OBJECTIVE: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. METHOD: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. RESULTS: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. CONCLUSION: Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.

Caracterização epidemiológica e causas da não doação por potenciais doadores de órgãos em morte encefálica / Epidemiological characterization and causes of non-donation by potential brain-dead organ donors / Caracterización epidemiológica y causas de la no donación de potenciales donantes de órganos en muerte cerebral

Objetivo: analisar as características epidemiológicas e causas da não efetivação do processo de doação de órgãos e tecidos de potenciais doadores em morte encefálica. Método: estudo transversal, com dados secundários do ano de 2019, obtidos de formulários de notificação de morte encefálica de pacientes com idade igual e superior a 18 anos, analisados descritiva e inferencialmente, após aprovação pelo comitê de ética. Resultados: notificadas 231 mortes encefálicas, com maior incidência de pessoas do sexo masculino, idade média de 48 anos e notificações dos hospitais públicos. Em Campo Grande, Mato grosso do Sul, observou-se maior número de notificações e o menor tempo entre a notificação e o primeiro exame clínico (RR: 4,01; IC 2,17-7,41; p<0,001). Entre os casos de não doação, 75,8% ocorreram por contraindicação médica e recusa familiar. Conclusão: houve predomínio de adultos jovens, não doadores de órgãos, cuja negativa da família deu-se pela vontade de manter o corpo íntegro.(AU)

Objective: to analyze the epidemiological characteristics and causes of non-completion of the organ and tissue donation process from potential brain-dead donors. Method: cross-sectional study, with secondary data from 2019, obtained from brain death notification forms of patients aged 18 years and over, analyzed descriptively and inferentially, after approval by the ethics committee. Results: 231 brain deaths were reported, with a higher incidence of males, average age of 48 years and notifications from public hospitals. In Campo Grande, Mato grosso do Sul, Brazil, there was a greater number of notifications and a shorter time between notification and the first clinical examination (RR: 4.01; CI 2.17-7.41; p<0.001). Among cases of non-donation, 75.8% occurred due to medical contraindication and family refusal. Conclusion: there was a predominance of young adults, non-organ donors, whose family's refusal was due to the desire to keep the body intact(AU)

Objetivo: analizar las características epidemiológicas y las causas de la no finalización del proceso de donación de órganos y tejidos de potenciales donantes en muerte cerebral. Método: estudio transversal, con datos secundarios del año 2019, obtenidos de formularios de notificación de muerte encefálica de pacientes de 18 años y más, analizados de manera descriptiva e inferencial, previa aprobación del comité de ética. Resultados: Se reportaron 231 muertes encefálicas, con mayor incidencia de hombres, edad promedio de 48 años y notificaciones de los hospitales públicos. En Campo Grande, Mato grosso do Sul, hubo mayor número de notificaciones y menor tiempo entre la notificación y el primer examen clínico (RR: 4,01; IC 2,17-7,41; p<0,001). Entre los casos de no donación, el 75,8% se produjo por contraindicación médica y negativa familiar. Conclusión: hubo predominio de adultos jóvenes, no donantes de órganos, cuya negativa familiar se debió al deseo de mantener el cuerpo íntegro(AU)

Estudos econômicos completos sobre tratamentos da anemia falciforme / Estudios económicos completos sobre tratamientos para la anemia falciforme / Comprehensive economic studies on sickle cell anemia treatments

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.

[Situation of endemic pemphigus foliaceus in Mato Grosso do Sul, Brazil, 1990-1999]. / Situação do pênfigo foliáceo endêmico em Mato Grosso do Sul, Brasil, 1990-1999.

307 cases were examined to characterize epidemiological aspects of endemic pemphigus foliaceus. The disease was predominantly identified in rural workers between 10 and 42 years old. Incidence was higher in the December, January, February, March and May (average: 30.7 cases/year). Our findings identified a corridor, coinciding with the state watershed.

Fatores de risco para mortalidade em pacientes com doença falciforme: uma revisão integrativa / Factores de riesgo para la mortalidad en pacientes con enfermedad falciforme: una revisión integradora / Risk factors for mortality in patients with sickle cell disease: an integrative review

RESUMO Objetivo Sumarizar fatores de risco e indicadores de mortalidade em pacientes com doença falciforme. Método Revisão integrativa em periódicos indexados nas bases de dados CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO e Web of Science. A questão norteadora foi elaborada por meio da estratégia Population, variable, outcome (PVO). A busca ocorreu no portal de periódicos da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior entre outubro e novembro de 2018. Resultados Dos 19 artigos, 18 eram coorte e um ensaio clínico randomizado. A amostra foi constituída, em sua maioria, pelo sexo feminino e genótipo HbSS. Se repetiram mais a taxa de mortalidade cumulativa e a curva de mortalidade global. Sete estudos identificaram fatores de risco com associação estatisticamente significativa para morte. Os mais frequentes foram o baixo nível de hemoglobina, variáveis hepáticas (enzimas fosfatase alcalina e transaminase glutâmico oxalacética) e cardiovasculares (velocidade de regurgitação da válvula tricúspide ≥ 2,5m/s). Conclusão e implicações para a prática Indicadores de mortalidade constituem ferramentas de manejo de pacientes com doença falciforme e prevenção de riscos e complicações. Há necessidade de estudos sobre os fatores relacionados à mortalidade desses pacientes. A prevenção do óbito, certamente, promoverá uma melhoria na qualidade de vida e na sobrevida dessa população.

RESUMEN Objetivo Resumir los factores de riesgo y los indicadores de mortalidad en pacientes con enfermedad de células falciformes. Método revisión integradora de publicaciones en las bases de datos CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO y Web of Science. La pregunta guía basada en Population, variable, outcome (PVO) conduciu la búsqueda en el Portal de revistas de la Coordinación de Mejoramiento de Personal de Nivel Superior, entre octubre-noviembre de 2018, con los descriptores anemia, sickle cell "and" mortality "and" survival y sus sinónimos. Resultados De 18/19 artículos son cohortes y un ensayo controlado aleatorio. La muestra consistió en mujeres y genotipo HbSS. La tasa de mortalidad acumulada y la curva de mortalidad general fueron más repetidas. Siete estudios identificaron factores de riesgo con asociación estadísticamente significativa con la muerte. Los más frecuentes fueron el bajo nivel de hemoglobina, variables hepáticas (fosfatasa alcalina y enzimas glutámicas transaminasas oxalacéticas) y variables cardiovasculares (velocidad de regurgitación de la válvula tricúspide ≥ 2.5m/s). Conclusión e implicaciones para la práctica Los indicadores de mortalidad son herramientas de manejo de los pacientes con esta enfermedad, la prevención de factores de riesgo y complicaciones. Hace necesidad de estudios sobre los factores relacionados con la mortalidad. La prevención de las muertes mejorará la calidad de vida y la supervivencia.

ABSTRACT Objective To summarize the risk factors and mortality indicators in sickle cell disease patients. Method Integrative review searched publications in journals in CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO, and Web of Science databases. The guiding question was elaborated through the Population, Variable, Outcome (PVO) strategy and the search was from October-to-November 2018, at the Coordination of Higher Level Personnel Improvement Periodicals' Gate. The keywords anemia, sickle cell "and" mortality "and" survival and their synonyms were used. Results From 18/19 articles were cohort and one randomized controlled trial. The sample consisted mostly of females and HbSS genotype. The cumulative mortality rate and the overall mortality curve were the most repeated. Seven studies identified risk factors with a statistically significant association with death. The most frequent were low hemoglobin level, liver variables (alkaline phosphatase and oxalacetic glutamic transaminase enzymes) and cardiovascular variables (tricuspid valve regurgitation speed ≥ 2.5m/s). Conclusion and implications for practice Mortality indicators are tools for better management of sickle cell disease's patient, prevention of risks and complications. There is a need for further studies on the factors related to mortality of these patients. Preventing the causes that lead to death will certainly improve the quality of life and survival of this population.

Experiência da família no manejo da criança com anemia falciforme: implicações para o cuidado / Families' experience in managing children with sickle cell anemia: implications for care / Experiencia de la familia en el manejo del niño con anemia falciforme: implicaciones para la atención

Objetivo: compreender a experiência da família no manejo da criança com anemia falciforme. Método: estudo qualitativo desenvolvido com 14 famílias de crianças com anemia falciforme, mediante entrevistas semiestruturadas abordando o manejo, as experiências e os enfrentamentos vivenciados do diagnóstico ao adoecimento. Utilizou-se como técnica a narrativa e como referencial teórico o Interacionismo Simbólico. Resultados: a experiência da família revela que ela permanece em constante vigilância e proteção, tendo de enfrentar desafios a partir do diagnóstico da anemia falciforme na criança. O desconhecimento dos profissionais sobre a doença, as lacunas na rede de atenção à saúde e a falta de informação sobre a doença marcam o itinerário, impondo a redefinição na dinâmica familiar. Conclusão: serviços de saúde e profissionais devem ser capacitados para atender as necessidades e demandas da criança com anemia falciforme e de sua família, bem como fornecer informações que as fortaleça.

Objective: to understand families' experience in managing children with sickle cell anemia. Method: this qualitative study of 14 families of children with sickle cell anemia was conducted by semi-structured interviews addressing the families' management, experiences and coping from diagnosis to illness. The narrative technique was used and Symbolic Interactionism gave the theoretical framework. Results: the families' experiences revealed that they are constantly vigilant and protective, and have to face challenges from the moment their children are diagnosed with sickle cell anemia. Their progress was hindered by health personnel's lack of knowledge about the disease, lacunae in the health care system, and a lack of information about the disease, which imposed a reworking of family dynamics. Conclusion: health services and personnel must be trained to meet the needs and demands of children with sickle cell anemia and their families, and to provide information to strengthen them.

Objetivo: comprender la experiencia de las familias en el manejo de niños con anemia de células falciformes. Método: este estudio cualitativo de 14 familias de niños con anemia falciforme se realizó mediante entrevistas semiestructuradas que abordaron el manejo, las experiencias y el afrontamiento de las familias desde el diagnóstico hasta la enfermedad. Se utilizó la técnica narrativa y el Interaccionismo Simbólico dio el marco teórico. Resultados: las experiencias de las familias revelaron que están constantemente vigilantes y protectoras, y deben enfrentar desafíos desde el momento en que a sus hijos se les diagnostica anemia falciforme. Su avance se vio obstaculizado por el desconocimiento del personal de salud sobre la enfermedad, las lagunas en el sistema de salud y la falta de información sobre la enfermedad, lo que impuso una reelaboración de la dinámica familiar. Conclusión: los servicios y el personal de salud deben estar capacitados para atender las necesidades y demandas de los niños con anemia falciforme y sus familias, y brindar información para fortalecerlos.

Survival and mortality among users and non-users of hydroxyurea with sickle cell disease.

OBJECTIVE: to estimate survival, mortality and cause of death among users or not of hydroxyurea with sickle cell disease. METHOD: cohort study with retrospective data collection, from 1980 to 2010 of patients receiving inpatient treatment in two Brazilian public hospitals. The survival probability was determined using the Kaplan-Meier estimator, survival calculations (SPSS version 10.0), comparison between survival curves, using the log rank method. The level of significance was p=0.05. RESULTS: of 63 patients, 87% had sickle cell anemia, with 39 using hydroxyurea, with a mean time of use of the drug of 20.0±10.0 years and a mean dose of 17.37±5.4 to 20.94±7.2 mg/kg/day, raising the fetal hemoglobin. In the comparison between those using hydroxyurea and those not, the survival curve was greater among the users (p=0.014). A total of 10 deaths occurred, with a mean age of 28.1 years old, and with Acute Respiratory Failure as the main cause. CONCLUSION: the survival curve is greater among the users of hydroxyurea. The results indicate the importance of the nurse incorporating therapeutic advances of hydroxyurea in her care actions.

[Nursing care for patients with sickle cell disease, in the light of Roy's model]. / Assistência de enfermagem a portadores de anemia falciforme, à luz do referencial de Roy.

This study aimed at applying the concepts from Roy's Adaptation Model in order to identify the behaviors (adaptive and ineffective) of patients with sickle cell disease as well as the focal, contextual and residual stimuli that are responsible for such behaviors. Data collection was conducted in the Hemoglobinopathy Outpatient Unit of the University Hospital at the University of São Paulo at Ribeirão Preto School of Medicine. Nine subjects (8 females and 1 male) were investigated. With respect to the physiological aspect, the ineffective behavior of low oxygenation and its outcomes (compromised physical development, sexual retardation hemolysis jaundice, respiratory alterations) were evidenced. Regarding the psychosocial aspects, self-image showed a reduction in self-esteem as the most distinguished behavior; role performance was characterized by occupation change. Considering interdependence, the mother was reported as the most frequent significant "other". Vaso-occlusion was predominantly identified as the causing stimulus for ineffective behaviors. It was verified that the affected physiological function alters other adaptive aspects.

Prevalence of latent tuberculosis and treatment adherence among patients with chronic kidney disease in Campo Grande, State of Mato Grosso do Sul.

INTRODUCTION: The primary strategy for tuberculosis control involves identifying individuals with latent tuberculosis. This study aimed to estimate the prevalence of latent tuberculosis in chronic kidney disease (CKD) patients who were undergoing hemodialysis in Campo Grande, State of Mato Grosso do Sul, Brazil, to characterize the sociodemographic and clinical profiles of patients with latent tuberculosis, to verify the association between sociodemographic and clinical characteristics and the occurrence of latent tuberculosis, and to monitor patient adherence to latent tuberculosis treatment. METHODS: This epidemiological study involved 418 CKD patients who were undergoing hemodialysis and who underwent a tuberculin skin test. RESULTS: The prevalence of latent tuberculosis was 10.3%. The mean patient age was 53.43±14.97 years, and the patients were predominantly men (63.9%). The population was primarily Caucasian (58.6%); half (50%) were married, and 49.8% had incomplete primary educations. Previous contact with tuberculosis patients was reported by 80% of the participants. Treatment adherence was 97.7%. CONCLUSIONS: We conclude that the prevalence of latent tuberculosis in our study population was low. Previous contact with patients with active tuberculosis increased the occurrence of latent infection. Although treatment adherence was high in this study, it is crucial to monitor tuberculosis treatment administered to patients in health services to maintain this high rate.

Scope and efficiency of the newborn screening program in identifying hemoglobin S.

BACKGROUND: In 2001, the Brazilian Ministry of Health added hemoglobinopathies to the National Neonatal Screening Program to be implemented in three steps. In order to meet the proposed goals, it is crucial to establish periodic assessments of this program with the aim of monitoring its implementation. OBJECTIVE: To assess the scope and the efficiency of the stages of the National Newborn Screening Program in identifying hemoglobin S. METHODS: A cross-sectional study was developed with the results of the heel prick test using the high performance liquid chromatography method for babies born in Mato Grosso do Sul from 2006 to 2010. The following variables were investigated: year, number of live births, total screening, coverage ratio, prevalence, time between the child birth and the blood collection; age at diagnosis; age at the time of the first consultation; and time between the diagnosis and the first appointment. RESULTS: Over the five years of the study, the mean coverage rate was 91.77%.The prevalences of hemoglobin FAS and hemoglobin FS were 1.65% and 0.011%, respectively. Blood samples from 43.48% of children were collected from the second to the seventh day. The age at diagnosis was within the first 28 days in 87.80% of the screened children. The lowest mean indices for the first consultation and the time between the diagnosis and the first appointment (58.8 and 46.4 days, respectively) occurred in 2010. CONCLUSIONS: The scope of the National Neonatal Screening Program for hemoglobinopathies is good, with a large number of individuals being tested. Efficiency is a suitable indicator to assess the program steps. Three points are recommended: the training of the individuals involved, studies to assess the assistance provided to the affected child, and genetic counseling to the mothers.

Psychological suffering of patients transplanted with hematopoietic stem cells / Sofrimento psicológico de pacientes transplantados com células-tronco hematopoéticas

Hematopoietic stem cell transplantation (HSCT) affects serious risks for the patient, including death. For this reason, it is considered a treatment that can cure or can present morbidities and lead to death. In this context, patients experience the first psychological conflicts before this transplant. To describe the psychological suffering developed by patients transplanted with hematopoietic stem cells from a referral service in the state of Rio Grande do Norte. This is a cross-sectional study with a quantitative, descriptive, hospital-based approach developed with patients submitted to HSCT at a referral service in the state of Rio Grande do Norte. Data were collected between March and September of 2016, through the evaluation of the medical records of 43 patients who underwent HSCT and developed some type of psychological distress. Among 43 patients with psychological disorders, 51.16% were female, 62.79% developed anxiety 32.56% developed insomnia and 20.93% developed depression. Of these, 27.91% had a main diagnosis indicating the transplantation of Multiple Myeloma (MM), and 58.14 received autologous transplantation. It was observed that the patient had psychological suffering from the diagnosis until the end. The health professional praxis also must go beyond the resolution of physical human responses and, especially in these cases, it should aim at the early identification of signs and symptoms of psychological distress, being possible to achieve the real health needs and treat them with effectiveness.

O Transplante de Células-Tronco Hematopoéticas (TCTH) acarreta sérios riscos para o paciente, inclusive o de morte. Por esse motivo é considerado um tratamento que pode curar ou que pode apresentar morbidades e levar ao óbito. Diante desse contexto, os pacientes vivenciam os primeiros conflitos psicológicos que antecedem o transplante em si. Descrever o sofrimento psicológico desenvolvido por pacientes transplantados com células-tronco hematopoéticas de um serviço de referência do estado do Rio Grande do Norte. Trata de um estudo do tipo transversal, com abordagem quantitativa, descritiva, de base hospitalar, desenvolvida com pacientes submetidos ao TCTH em um serviço de referência do estado do Rio Grande do Norte. Os dados foram coletados entre os meses de março e setembro de 2016, mediante avaliação dos prontuários de 43 pacientes que realizaram o TCTH e desenvolveram algum tipo de sofrimento psicológico. Dentre os 43 pacientes que apresentaram distúrbios psicológicos, 51,16% eram do sexo feminino, 62,79% desenvolveram ansiedade, 32,56% insônia e 20,93% depressão. Destes, 27,91% tinham como diagnóstico principal que indicou o transplante o Mieloma Múltiplo (MM), 58,14 receberam transplante autólogo. Observou-se que o sofrimento psicológico acompanhou o paciente desde o diagnóstico até o desfecho. Ademais, a práxis do profissional de saúde deve ir para além da resolução de respostas humanas físicas e, especialmente para estes casos, deve visar a identificação precoce dos sinais e sintomas de sofrimento psicológico para que seja possível alcançar as reais necessidades de saúde e tratá-las com efetividade.

Prevalência da doença falciforme em adultos com diagnóstico tardio / Prevalencia de la anemia falciforme en adultos con diagnóstico tardío / Prevalence of sickle cell disease in adults with delayed diagnosis

Resumo Objetivo: Estimar a prevalência da doença falciforme em adultos com diagnóstico tardio, em tratamento nos ambulatórios de hematologia na rede de saúde do Estado do Mato Grosso do Sul de 2013 a 2017; descrever as características sociodemográficas; verificar associações entre os genótipos em relação a idade atual, os genótipos e a idade ao diagnóstico. Métodos: Estudo transversal, retrospectivo, com dados coletados em dois hospitais de ensino. As variáveis investigadas foram: ano do atendimento, genótipo, sexo, data de nascimento, idade ao diagnóstico, naturalidade e procedência. A prevalência foi estimada por ponto (%) e intervalo de confiança de 95%. Resultados: A prevalência foi 3,9%, com 103 adultos com doença falciforme, sendo 60 do sexo feminino e 43 do masculino. Predominou o genótipo HbSS, seguido pelo HbSC. A mediana de idade foi de 35 para os HbSS e 31 para os HbSC. A mediana de idade ao diagnóstico foi cinco anos para os HbSS e 21 para HbSC. Não houve associação entre idade (anos) dos pacientes e genótipo (teste Qui-quadrado p=0,601) e nem entre genótipo e faixa etária (teste Qui-quadrado p= 0,318). Conclusão: O genótipo mais frequente foi o HbSS, seguido pelo HbSC. O diagnóstico dos pacientes com SC foi mais tardio do que naqueles com genótipo SS. As variáveis sociodemográficas e o diagnóstico tardio alertam para a necessidade de fortalecimento de ações na rede de saúde, que interferem sensivelmente na morbimortalidade de adultos com Doença Falciforme.

Resumen Objetivo: Estimar la prevalencia de la anemia falciforme en adultos con diagnóstico tardío, en tratamiento ambulatorio de hematología de la red de salud del estado de Mato Grosso do Sul de 2013 a 2017; describir las características sociodemográficas; verificar asociaciones entre los genotipos con relación a la edad actual, los genotipos y la edad de diagnóstico. Métodos: Estudio transversal, retrospectivo, con datos recopilados en dos hospitales universitarios. Las variables investigadas fueron: año de atención, genotipo, sexo, fecha de nacimiento, edad de diagnóstico, naturalidad y procedencia. La prevalencia fue estimada por punto (%) e intervalo de confianza de 95%. Resultados: La prevalencia fue 3,9%, con 103 adultos con anemia falciforme, 60 de sexo femenino y 43 masculino. Predominó el genotipo HbSS, seguido de HbSC. La mediana de edad fue 35 años en los HbSS y 31 en los HbSC. La mediana de edad de diagnóstico fue 5 años en los HbSS y 21 en los HbSC. No hubo relación entre edad (años) de los pacientes y genotipo (prueba ⊠2 de Pearson p=0,601) y tampoco entre genotipo y grupo de edad (prueba ⊠2 de Pearson p=0,318). Conclusión: El genotipo más frecuente fue el HbSS, seguido del HbSC. El diagnóstico de los pacientes con SC fue más tardío que el de los de genotipo SS: Las variables sociodemográficas y el diagnóstico tardío advierten sobre la necesidad de fortalecer acciones en la red de salud, que interfieren sensiblemente en la morbimortalidad de adultos con anemia falciforme.

Abstract Objective: To estimate the prevalence of sickle cell disease in adults with delayed diagnosis, receiving treatment at hematology outpatient clinics in the health network of the state of Mato Grosso do Sul, between 2013 and 2017; to describe sociodemographic characteristics; to verify associations between genotypes in relation to current age, the genotypes, and age at diagnosis. Methods: A retrospective, cross-sectional study with data collected at two teaching hospitals. The variables investigated were: year of treatment, genotype, sex, date of birth, age at diagnosis, and city in which they lived. Prevalence was estimated per point, using a 95% confidence interval. Results: The prevalence was 3.9% in 103 adults with sickle cell disease: 60 female and 43 male. The HbSS genotype was predominant, followed by HbSC. The median age was 35 for HbSS, and 31 for HbSC. Median age at diagnosis was five years for HbSS, and 21 for HbSC. No association was found between age (years) of patients and genotype (chi-square test p=0.601), or between genotype and age group (chi-square test p= 0,318). Conclusion: The most frequent genotype was HbSS, followed by HbSC. The diagnosis of patients with hemoglobin SC occurred later in life than those with the hemoglobin SS genotype. Sociodemographic variables and delayed diagnosis warns for the need to strengthen actions in the health network, which interfere significantly in the morbidity and mortality of adults with sickle cell disease.

Clinical and epidemiological profile of children and adolescents submitted to the hematopoietic cell transplantation / Perfil clínico e epidemiológico de crianças e adolescentes submetidos ao transplante de células-tronco hematopoéticas

The Hematopoietic stem cell transplantation (HSCT) is used in children as a definitive treatment for various oncological, immune deficiencies, hemoglobinopathy, and malignancies diseases that involve the hematological system, congenital metabolism disorders, among others. To characterize the clinical and epidemiological profile of children and adolescents submitted to HSCT at a referral service in the state of Rio Grande do Norte. This is a quantitative, retrospective, observational, descriptive and analytical quantitative approach approaching the medical records of children and adolescents submitted to HSCT in a referral hospital service for this type of transplantation in the state of Rio Grande do Sul North (RN). The final sample consisted of 35 records patients aged between 2 and 18 years old who underwent HSCT from February 2008 to December 2015 and who presented the data necessary for the study. The records analyzed showed a littlemajority of male patients (51.42%) and 60.00% of these men were students and 71.42% lived in the state of the Rio Grande do Norte. According to the clinical characteristics, 34.3% of the patients had Acute Lymphoblastic Leukemia and 25.71% had Acute Myeloid Leukemia as the main diagnosis. Gastrointestinal toxicities were the most frequent (97.1%) and all patients received antineoplastic/chemotherapeutic and antiemetic treatment. The allogeneic HSCT was the most frequently performed (57.14%) and the most used source of Hematopoietic progenitor cells (HPC) was the peripheral blood (54.29%) and 5.71% of these patients developed the Graft versus Host Disease (GVHD), of which one was affected by acute GVHD and another by chronic GVHD. Septsis was the most frequent cause of death (60%). The profile of the clinical variables presented by the children and adolescents of this study shows that the most prevalent diagnosis was ALL, the most frequent toxicities were gastrointestinal, cardiac, respiratory and hematological, the most common HSCT was allogeneic peripheral blood and the greatest cause of mortality was sepsis. These data are similar to studies conducted in North America, Europe and Asia.

O Transplante de Células-Tronco Hematopoéticas (TCTH) é utilizado em crianças como um tratamento definitivo para várias doenças oncológicas, imunodeficiências, hemoglobinopatias, malignidades que envolvem o sistema hematológico, distúrbios de metabolismo congênito, entre outros. Caracterizar o perfil clínico e epidemiológico de crianças e adolescentes submetidos ao TCTH em um serviço de referência do estado do Rio Grande do Norte. Trata de um estudo epidemiológico de abordagem quantitativa, do tipo coorte retrospectiva, observacional, descritivo e analítico onde foram abordados os prontuários de crianças eadolescentes submetidos ao TCTH em um serviço hospitalar de referência para esse tipo de transplante no estado do Rio Grande do Norte (RN). A amostra final foi composta por 35 prontuários de pacientes com idade entre dois e 18 anos que realizaram o TCTH no período de fevereiro de 2008 a dezembro de 2015 e que apresentavam os dados necessários ao estudo. Do total de prontuários analisados houve discreta maioria de pacientes do sexo masculino (51,42%). Destes, 60,00% eram estudantes e 71,42% residiam no estado do RN. De acordo com as características clínicas, 34,3% apresentaram como diagnóstico principal a Leucemia Linfoblástica Aguda e 25,71% a Leucemia Mieloide Aguda. As toxicidades gastrointestinais foram as que mais ocorreram (97,1%) e todos receberam tratamento com antineoplásicos/quimioterápicos e antieméticos. O TCTH alogênico foi o mais frequentemente realizado (57,14%) e a fonte de CPH mais utilizada foi o sangue periférico (54,29%) e 5,71% desenvolveram a Doença do Enxerto Contra Hospedeiro (DECH), dos quais um foi acometido por DECH aguda e outro pela forma crônica. A causa de morte com maior frequência foi a sepse (60%). O perfil das variáveis clínicas apresentadas pelas crianças e adolescents desta pesquisa mostram que o diagnóstico mais prevalente foi o de LLA, as toxicidades que mais ocorreram foram as gastrointestinais, cardíacas, respiratórias e hematológicas, o TCTH mais realizado foi o alogênico de sangue periférico e a maior causa de mortalidade foi a sepse. Tais dados se assemelham aos estudos realizados na América do Norte,Europa e Ásia.

Quality of life in adults with sickle cell disease: an integrative review of the literature / Cualidad de vida en adultos con enfermedad falciforme: revisión integradora de la literatura / Qualidade de vida em adultos com doença falciforme: revisão integrativa da literatura

ABSTRACT Objective: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. Method: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. Results: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. Conclusion: Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.

RESUMEN Objetivo: Identificar las evidencias disponibles en la literatura sobre cualidad de vida relacionada a la salud en adultos con enfermedad falciforme. Método: revisión integradora en las bases de datos MEDLINE,CUMED,LILACS y SciELO, de artículos provenientes de estudios desarrollados en esta temática, publicados entre 2005 y 2015, en inglés, portugués o español. Resultados: fueron incluidos 22 artículos, siendo utilizadas seis escalas para evaluar las puntuaciones de cualidad de vida relacionada a la salud: tres genéricas y tres específicas. Ninguna escala específica para adultos con enfermedad falciforme fue adaptada para el portugués brasileño hasta el momento. Pacientes acometidos por crisis dolorosas frecuentes, con baja adhesión al tratamiento, presentaron comprometimiento de la cualidad de vida. Conclusión: Los estudios seleccionados evidenciaron que los pacientes con enfermedad falciforme tienen peores puntuaciones que la población en general. Esos indicadores deberían instrumentalizar el enfermero en la proposición de intervenciones y estrategias asistenciales y socioeducativas, con vistas a mejorar la cualidad de vida de esta clientela.

RESUMO Objetivo: Identificar as evidências disponíveis na literatura sobre qualidade de vida relacionada à saúde em adultos com doença falciforme. Método: revisão integrativa nas bases de dados MEDLINE, CUMED, LILACS e SciELO, de artigos provenientes de estudos desenvolvidos nesta temática, publicados entre 2005 a 2015, em inglês, português ou espanhol. Resultados: foram incluídos 22 artigos, sendo utilizadas seis escalas para avaliar os escores de qualidade de vida relacionada à saúde: três genéricas e três específicas. Nenhuma escala específica para adultos com doença falciforme foi adaptada para o português brasileiro até o momento. Pacientes acometidos por crises dolorosas frequentes, com baixa adesão ao tratamento, apresentaram comprometimento da qualidade de vida. Conclusão: Os estudos selecionados evidenciaram que os pacientes com doença falciforme têm piores escores do que a população em geral. Esses indicadores deveriam instrumentalizar o enfermeiro na proposição de intervenções e estratégias assistenciais e socioeducativas, com vistas a melhorar a qualidade de vida desta clientela.

Fatores associados à qualidade de vida de pessoas com feridas complexas crônicas / Los factores asociados con la calidad de vida de personas con heridas crónicas complejo / Factors associated with quality of life of people with chronic complex wounds

Objective: to analyze the quality of life of people with chronic complex wounds. Method: Data were collected through a questionnaire containing the WHOQOL-Bref instrument of the World Health Organization and questions about sociodemographic and clinical variables. Statistical analysis methods used were: linear correlation of Pearson; Analysis of Variance and Tukey with reliability of 95% and p <0.05. Results: We evaluated 53 patients with complex wounds. 28 (52.83%) of these were female with an average age of 62.17 years and with low education. In the evaluation of the quality of life, the pain was correlated with the physical domain (p = 0.030) and psychological (p = 0.051); while the area was correlated with age (p = 0.051) and pain correlated with the time of injury (p = 0.001). Conclusion: Knowning the clinical characteristics and the quality of life enables better understanding of the complaints and better healthcare planning, which contributs to improved quality of life.

Objetivo: analisar a qualidade de vida de pessoas com feridas complexas crônicas. Método: Os dados foram coletados por meio de questionário contendo o instrumento WHOQOL-Bref da Organização Mundial de Saúde e questões sobre variáveis sociodemográficos e clínicas. Para análise estatística foram utilizados os métodos: Correlação Linear de Pearson; Análise de Variância e Tukey com confiabilidade de 95% e p<0,05. Resultados: Foram avaliados 53 pacientes com feridas complexas. Desses, 28 (52,83%) são do sexo feminino com média de idade de 62,17 anos e com baixa escolaridade. Na avaliação da qualidade de vida, a dor se correlacionou com o domínio físico (p=0,030) e psicológico (p=0,051); enquanto que a área se correlacionou com a idade (p=0,051), e a dor se correlacionou com o tempo de lesão (p=0,001). Conclusão: Conhecer as características clínicas e a qualidade de vida possibilita melhor compreensão das queixas e melhor planejamento assistencial contribuindo para melhoria da qualidade de vida.

Objetivo: analizar la calidad de vida de las personas con heridas crónicas complejas. Métodos: Los datos fueron recolectados a través de un cuestionario que contiene el instrumento WHOQOL-BREF de la Organización Mundial de la Salud y las preguntas acerca de las variables sociodemográficas y clínicas. Se utilizaron métodos de análisis estadístico: correlación lineal de Pearson; El análisis de varianza y Tukey con una fiabilidad del 95% y p <0,05. Resultados: Se evaluaron 53 pacientes con heridas complejas. De éstos, 28 (52,83%) eran mujeres con una edad media de 62,17 años y con bajo nivel de educación. En la evaluación de la calidad de vida el dolor se correlacionó con el dominio físico (p = 0,030) y psicológico (p = 0,051); mientras que el área se correlacionó con la edad (p = 0,051) y el dolor correlacionado con el momento de la lesión (p = 0,001). Conclusión: Conocer las características clínicas y la calidad de vida permite una mejor comprensión de las quejas y una mejor planificación de la salud, lo que contribuye a una mejor calidad de vida.

[Insufficient loss of weight and/or absence of DM2 remission after Roux-en-Y gastric bypass: factors that may influence the unsatisfactory results]. / Perda insuficiente de peso e ou ausência de remissão da diabete melito tipo 2 após a derivação gástrica em Y-de-Roux: fatores que podem influenciar os resultados insatisfatórios.

BACKGROUND: Roux-en-Y gastric by-pass is considered one of the most effective treatments for maintaining long-term weight loss. However, it is associated to failures manifested by the inability to maintain weight loss, weight gain or poor glycemic control. OBJECTIVE: Study the possible factors that influence weight loss failure and/or DM2 remission. METHODS: Case-control study of 159 patients submitted to gastric by-pass two or more years postoperatively. Twenty-four individuals with unsatisfactory weight loss and/or DM2 remission were selected as cases and 24 with satisfactory weight loss and/or absence of DM2 remission as controls, matched for age and postoperative time. RESULTS: Of the 24 cases and controls evaluated, the percentage weight gain was 19.32% and 8.68%, percentage DM2 remission 26.6% and 87.5% and percentage DM2 recurrence 6.6% and 0.0%, respectively. Cases and controls exhibited mean maximum preoperative BMI of 53.50±12.24 kg/m2 and 48.77±5.19 kg/m2, respectively. These values were statistically significant in terms of poor weight management or failed surgery. CONCLUSION: Patients with elevated initial maximum BMI (≥ 50kg/m2) displayed higher weight loss failure rates. Food intolerance and socioeconomic differences are considered factors in weight gain.

Profile of the obese patients submitted to Roux-en-Y gastric bypass whithout diabetes melittus type 2 remission and/or insufficient weight loss.

BACKGROUND: The literature reports that gastrojejunal derivation with Roux-en-Y gastric bypass is highly efficient in controlling weight and resolving; but studies have shown worsened glycemic control in a considerable number of patients and associated factors that have not been fully elucidated. AIM: To analyze the profile of patients submitted to gastric bypass that did not achieve satisfactory weight loss or complete diabetes remission. METHODS: Case-control study of 32 patients submitted to gastric bypass with at least two years postoperative time, unsatisfactory results in terms of weight loss or absence of complete diabetes remission. The control group was composed of another 32 patients submitted to the same operation at the same facility, matched for age and postoperative time. A structured questionnaire was applied and clinical and laboratory data were analyzed. RESULTS: Among the cases and controls, BMI was 38.9 kg/m² and 29.5 kg/m2, excess weight loss was 56.1% and 77.2%, % excess weight regain of initial excess weight loss, was 20.2% and 7.7%, respectively. Family history of type 2 diabetes mellitus, hypertension and food intolerance showed a significant relationship between cases and controls. CONCLUSION: Food intolerance and family history of hypertension and diabetes were associated to lower loss and weight regain or less likelihood of complete diabetes remission after gastric bypass.