Reliability and validity of the Patient Generated Index (PGI) in patients with chronic kidney disease (CKD) on dialysis, a new approach to quality of life.

PURPOSE: To evaluate the test-retest reliability and validity of the Patient Generated Index (PGI) in individuals with Chronic Kidney Disease (CDK) undergoing hemodialysis. METHODS: Through a non-experimental study with repeated measures, PGI was applied twice to assess internal consistency and test-retest reliability. Correlations with the Kidney Disease Quality of Life Short Form (KDQOL-SF), the Human Activity Profile (HAP) questionnaire, the Social Participation Scale, and the Glittre ADL Test were used. RESULTS: 91 individuals with CKD were evaluated. There was high reliability for the PGI (ICC= 0.97) PGI correlated with KQDOL - SF in Functional Capacity r = 0.38 (p < 0.001), Emotional Well-Being r = 0.31 (p = 0.003), Social Aspect r = 0.22 (p = 0.036), Emotional Function r = 0.22 (p = 0.038) and Effect of Kidney Disease r = 0.21 (p = 0.042), and Physical scores r = 0.24 (p = 0.021)), Mental r = 0.21 (p = 0.05) and General r = 0.22 (p = 0.037) summarized. There was a significant correlation between PGI and HAP r = 0.40 (p < 0.001) and the Social Participation Scale r = -0.36 (p < 0.001). There was no correlation between the PGI and Glittre ADL scores r = 0.12 (p = 0.247). CONCLUSION: In adults receiving hemodialysis, the PGI proved to be an accurate and reliable instrument for the assessment of the quality of life from the perspective of the patient.IMPLICATIONS FOR REHABILITATIONAlthough hemodialysis treatment is associated with increased survival and symptom control, there is a significant change in the patient's lifestyle.In order to provide a more focused view of the individual, the Patient Generated Index (PGI) was created to evaluate the quality of life.PGI is reliable and correlates with KQDOL - SF and the Social Participation Scale in this population.

Determining factors of functioning in hemodialysis patients using the international classification of functioning, disability and health.

BACKGROUND: Hemodialysis (HD) treatment affects functioning, physical activity level, clinical biomarkers, and body composition. However, the association between these variables with functioning, considering International Classification of Functioning, Disability and Health (ICF) domains remains unclear. Thus, the aim of this study was to investigate the possible association between physical activity, biomarkers, and body composition with functioning in HD patients in reference to the ICF. METHODS: Eighty HD patients performed different tests grouped according to ICF domain: Body structure and function - handgrip strength (HS), 5-repetition sit-to-stand test, and 60-s sit-to-stand test (5-STS, 60-STS, respectively); Activity - short physical performance battery (SPPB); and Participation - participation scale questionnaire. Physical activity [Human Activity Profile questionnaire (HAP)], body composition (Dual-energy X-ray absorptiometry), Parathormone (PTH), and alkaline phosphatase were analyzed as possible variables associated with ICF domains. Data analyses were performed using simple and multiple regression models adjusted for age, duration of HD, and diuresis volume. RESULTS: In the body structure and function domain, appendicular lean mass, PTH level, and age were associated with HS (R2 = 0.558); HAP and PTH were associated with 5-STS (R2 = 0.263); and HAP, PTH, duration of HD, and age were associated with 60-STS (R2 = 0.337). In the activity domain, HAP, PTH, alkaline phosphatase, duration of HD, age, and body fat were associated with SPPB (R2 = 0.689). Finally, only HAP was associated with the participation scale (R2 = 0.067). CONCLUSION: Physical activity and PTH levels are determinant protagonists of functioning in all ICF domains in hemodialysis patients.

The Glittre activities of daily living as a potential test for functional evaluation of patients on hemodialysis: a validation study.

PURPOSE: To investigate the validity and within-day reliability of the Glittre activities of daily living test (Glittre ADL-test) in patients on hemodialysis. METHODS: Ninety-one patients on hemodialysis were evaluated using Glittre ADL-test, functional tests, health-related quality of life, human activity profile, and social participation. Convergent validity was analyzed by the correlation analysis. The Glittre ADL-test results were compared among different levels of the functional tests, human activity profile, and participation (known-groups validity). Within-day reliability was examined by Intra-class Correlation Coefficient (ICC). RESULTS: There was a significant moderate to strong correlation between Glittre ADL-test and functional tests (r = 0.62 to 0.66 and -0.70 to -0.44). A significant weak correlation was found between Glittre ADL-test, health-related quality of life (r = 0.28 to 0.32), and human activity profile (r=-0.33). There was no significant correlation with participation. Except for participation level, the Glittre ADL-test results were able to identify individuals with low performance in all evaluated tests and were able to differentiate among inactive and active individuals by human activity profile (p < 0.05). Within-day reliability was evaluated in 42 patients. The ICC was 0.97. CONCLUSION: The Glittre ADL-test is a reliable test associated with functional parameters and physical aspects related to health-related quality of life, with potential value in the functional evaluation of patients on hemodialysis.Implications for rehabilitationFunctional impairment is a common clinical finding and is an important predictor of poor prognosis in patients on hemodialysis.The Glittre ADL-test has excellent within-day reliability and is a valid instrument for functional assessment in patients on hemodialysis.Activity limitations of patients on hemodialysis can be assessed by Glittre ADL-test.

Maximal inspiratory pressure is associated with health-related quality of life and is a reliable method for evaluation of patients on hemodialysis.

OBJECTIVE: To evaluate the association between Maximal Inspiratory Pressure (MIP) and health-related quality of life (HRQoL) and to verify the reliability of the MIP in patients on hemodialysis. METHODS: In a repeated-measures design, patients on hemodialysis performed MIP and specific HRQoL questionnaire (trial 1). The MIP was repeated after 6 to 8 weeks (trial 2) and reliability was assessed using Intra-class Correlation Coefficient. Standard Error of Measurement and Minimal Detectable Change scores were calculated. RESULTS: Sixty-one individuals (68.9% men) were evaluated in trial 1. MIP was associated with specific domains "Symptoms" (r = 0.45; R2 adjusted = 0.192) and the kidney disease component summary (r = 0.38; R2 adjusted = 0.138). Regarding generic domains, the MIP was associated with "Physical Functioning" (r = 0.57; R2 adjusted = 0.375) and Physical component summary (r = 0.47; R2 adjusted = 0.258). Thirty-three patients were randomly selected to perform a second MIP test (trial 2). The Intra-class Correlation Coefficient was 0.94 (95%CI 0.88-0.97). By Bland-Altman analysis, the bias was 3.2 cmH2O, which represents a difference of 3.7%. The Standard Error of Measurement and Minimal Detectable Change for MIP were 5.9 cmH2O and 13.8 cmH2O, respectively. CONCLUSION: The MIP is a reliable test, associated with physical domains of HRQoL in patients on hemodialysis. Thus, it is a useful method for respiratory evaluation in this population.

An international, multicenter, retrospective study on the positive impact of cutaneous involvement on the clinical outcome of adult T-cell leukemia/lymphoma.

Adult T-cell leukemia/lymphoma (ATLL) is a largely incurable disease. Cutaneous involvement is common and could be first symptom of the disease. We analyzed 169 patients with ATLL of whom 63 had cutaneous involvement. Cutaneous involvement was found in 48, 27, 17, and 60% of acute, lymphomatous, chronic and smoldering ATLL cases, respectively. Eight cases had primary cutaneous tumoral variant. Erythroderma (24%) and plaques (22%) were the most frequent skin lesions. The presence of cutaneous involvement was associated with better overall survival compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37-0.82], p < 0.01; 1-year OS 53 vs. 27%, respectively, p = 0.012). Combination zidovudine and interferon-alpha (AZT-IFN) yielded high response rates (overall response, OR = 100%, n = 8; complete response 62.5%) compared to chemotherapy (OR = 33.3%, n = 12/36). In conclusion, cutaneous involvement was associated with better survival in Latin American patients with ATLL. AZT-IFN demonstrated encouraging responses in ATLL patients with cutaneous involvement.

Modos imaginativos e colaborativos de fazer pesquisa: dispositivos e disposições com cuidado / Imaginative and cooperative ways of doing research: dispositives and dispositions with care

Resumo Este artigo é direcionado para aspectos metodológicos implicados em uma pesquisa sobre respostas indígenas à covid-19 realizada por uma rede de pesquisadoras indígenas e não indígenas em diferentes estados brasileiros. Pretendemos compartilhar experiências e reflexões sobre limites e potencialidades de uma pesquisa realizada na pandemia e com a pandemia, já que o adoecimento experimentado nos corpos e na vida coletiva das pesquisadoras foi um agente incontornável em percursos metodológicos e analíticos, em diálogo com debates feministas sobre a tópica do cuidado.

Abstract This article is focused on methodological aspects impplied in a research on the responses of indigenous peoples to COVID-19 conducted by a network of indigenous and non-indigenous women researchers in different Brazilian states. We seek to share experiences and reflections on the limits and potentialities of a research carried out in the pandemic and with the pandemic, since the sickeness felt in the bodies and collective life of the researchers was an unavoidable agent in the methodological and analytical parcourses, in dialogue with feminist debates on the theme of care.

Percepção da qualidade de vida e autoestima após intervenção odontológica e transplante renal: uma série de casos / Quality of life perception and self-esteem after dental intervention and kidney transplantation: a case series

Contexto: a insuficiência renal crônica dá-se quando os rins deixam de desenvolver suas multitarefas com eficácia, levando o paciente à hemodiálise ou transplante renal e a um progressivo desgaste emocionalObjetivo:avaliar percepção da qualidade de vida relacionada à saúde bucal e autoestima de pacientes submetidos à hemodiálise, após intervenções odontológicas e transplantes renais. Materiais e Métodos: Oitenta e dois pacientes responderam aos questionários SF-36, OHIP-14 e Escala de Autoestima de Rosenberg. Destes, 76 passaram por intervenções odontológicas e responderam aos questionários novamente. Após 3 anos, apenas 33 deram continuidade ao estudo. Doze foram transplantados e 10 responderam aos questionários ao final. Resultados:Apenas a autoestima mostrou melhora, com alterações estatisticamente significativas. Conclusão:Tratamento odontológico e transplante renal melhoraram a autoestima dos pacientes. Houve uma tendência de melhora no aspecto social.

Background: Chronic kidney failure occurs when the kidneys fail to perform their multitasking effectively, leading the patient to hemodialysis or kidney transplantation and progressive emotional exhaustionObjective:to evaluate the perception of quality of life related to oral health and self-esteem of patients undergoing hemodialysis, after dental interventions and kidney transplants. Materials and Methods:Eighty-two patients answered the SF-36, OHIP-14 and Rosenberg Self-Esteem Scale questionnaires. Of these, 76 underwent dental interventions and answered the questionnaires again. After 3 years, only 33 continued the study. Twelve were transplanted and 10 completed the questionnaires. Results:Only self-esteem showed improvement, with statistically significant changes. Conclusion:Dental treatment and kidney transplantation improved patients' self-esteem. There was a trend towards improvement in the social aspect.

Real-World Data on Adult T-Cell Leukemia/Lymphoma in Latin America: A Study From the Grupo de Estudio Latinoamericano de Linfoproliferativos.

PURPOSE: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease caused by the human T-cell leukemia virus type 1. Real-world data of ATLL in Latin America are lacking. PATIENTS AND METHODS: We analyzed patients with ATLL (acute, lymphomatous, chronic, and smoldering) encountered in 11 Latin American countries between 1995 and 2019. Treatment response was assessed according to the 2009 consensus report. Survival curves were estimated using the Kaplan-Meier method and log-rank test. RESULTS: We identified 253 patients; 226 (lymphomatous: n = 122, acute: n = 73, chronic: n = 26, and smoldering: n = 5) had sufficient data for analysis (median age 57 years). Most patients with ATLL were from Peru (63%), Chile (17%), Argentina (8%), and Colombia (7%). Hypercalcemia was positively associated with acute type (57% v lymphomatous 27%, P = .014). The median survival times (months) were 4.3, 7.9, 21.1, and not reached for acute, lymphomatous, chronic, and smoldering forms, with 4-year survival rates of 8%, 22%, 40%, and 80%, respectively. First-line zidovudine (AZT)-interferon alfa (IFN) resulted in an overall response rate of 63% (complete response [CR] 24%) for acute. First-line chemotherapy yielded an overall response rate of 41% (CR 29%) for lymphomatous. CR rate was 42% for etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone versus 12% for cyclophosphamide, vincristine, doxorubicin, and prednisone-like regimen (P < .001). Progression-free survival at 1 year for acute type patients treated with AZT-IFN was 67%, whereas 2-year progression-free survival in lymphomatous type patients who achieved CR after chemotherapy was 77%. CONCLUSION: This study confirms Latin American ATLL presents at a younger age and has a high incidence of lymphomatous type, low incidence of indolent subtypes, and worse survival rates as compared with Japanese patients. In aggressive ATLL, chemotherapy remains the preferred choice for lymphomatous favoring etoposide-based regimen (etoposide, cyclophosphamide, vincristine, doxorubicin, and prednisone), whereas AZT-IFN remains a good first-line option for acute subtype.

ALK-negative anaplastic large cell lymphoma: features and outcomes of 235 patients from the International T-Cell Project.

Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma (ALK- ALCL) is an aggressive neoplasm of T-cell/null-cell lineage. The T-Cell Project is a global prospective cohort study that consecutively enrolled patients newly diagnosed with peripheral T-cell lymphoma, registered through a centralized computer database between September 2006 and February 2018. Of 1553 validated cases from 74 sites in 13 countries worldwide, 235 were reported as ALK- ALCL. The median age at diagnosis was 54 years (range, 18-89 years), with a male predominance (62%). Stage III to IV disease was identified in 71% of patients, bulky disease and bone marrow involvement were uncommon, and 66% of patients presented with a low (0-1) International Prognostic Index score. Of all treated patients, 85% received multiagent initial chemotherapy, and 8% were consolidated with autologous hematopoietic cell transplantation. The initial overall and complete response rates were 77% and 63%, respectively. After a median follow-up of 52 months (95% confidence interval [CI], 41-63), the median progression-free survival (PFS) and overall survival (OS) were 41 months (95% CI, 17-62) and 55 months (95% CI, 36-75), respectively. The 3- and 5-year PFS rates were 52% and 43%, and the 3- and 5-year OS rates were 60% and 49%. Treatments containing both anthracycline and etoposide were associated with superior OS (P = .05) but not PFS (P = .18). In this large prospective cohort study, outcomes comparable to those previously reported in the retrospective International Peripheral T-Cell Lymphoma Project were observed. The study underscores the need for introducing novel platforms for ALK- ALCL and establishes a benchmark for future clinical trials. This trial was registered at www.clinicaltrials.gov as #NCT01142674.

Allogeneic stem cell transplantation improves survival in relapsed Hodgkin lymphoma patients achieving complete remission after salvage treatment.

Allogeneic stem cell transplant (alloSCT) is a current treatment option for patients with refractory/relapsed classic Hodgkin lymphoma (CHL), including those who have failed an autologous transplantation. We performed a retrospective multicenter analysis of 113 patients (median age 28 years; range 14-56; 54% males) with refractory/relapsed (R/R) CHL who had undergone alloSCT in Argentina. Kaplan-Meier was used to estimate overall (OS) and progression-free survival (PFS). Relapse rate (RR) and non-relapse mortality (NRM) were estimated with cumulative incidence analysis. Disease status at transplant was complete remission (CR) in 39%, partial remission (PR) in 44%, and stable/progressed disease (S/PD) in 17% of the patients. Donor type was matched related (MRD) in 60%, unrelated (URD) in 19%, and haploidentical (HID) in 21% of the patients. OS and PFS at 2 years were 43% and 27%, respectively, for all the cohort. In the univariate analysis, patients in CR showed better OS (p ≤ 0.001) and PFS (p ≤ 0.001), and lower NRM (p = 0.04). HID had better PFS (p = 0.04) and lower RR (p = 0.02). In the multivariate analysis, CR showed a significant impact on OS and PFS, and HID on PFS. AlloSCT is a feasible procedure in patients with CHL. Those in CR at the time of the transplant had better outcomes. Haploidentical transplantation is associated with better PFS in these patients with poor prognosis.

Características clínicas de clientes com hipertensão arterial e diabetes mellitus / Clinical characteristics of users with arterial hypertension and diabetes mellitus

Objetivo: descrever as características clínicas da clientela com hipertensão arterial sistêmica e diabetes mellitus associado acompanhada pelo sistema Hiperdia em uma Estratégia Saúde da Família, relacionando com variáveis sociodemográficas. Método: trata-se um estudo quantitativo, transversal, com 20 pacientes que responderam a um questionário estruturado. Utilizou-se o programa Microsoft Office Excel 2007® para a análise dos dados. Resultados: verificou-se a predominância do sexo feminino, idosas, brancas, casadas e com baixa escolaridade. Observou-se alta prevalência de inatividade física, histórico familiar de hipertensão ou diabetes, baixa adesão a ações educativas, com tempo de diagnostico maior que cinco anos, distúrbio do sono e a realização de apenas duas refeições diárias. Conclusão: conclui-se que a alta prevalência da ocorrência simultânea de fatores de risco como inativadade física, histórico hereditário, distúrbio do sono e baixo número de alimentações diárias evidencia vazios socioassistencais. Avança-se, além disso, por este estudo, na perspectiva multidimensional da atenção à saúde, pois engloba variáveis para além da doença.(AU)

Objetivo: to describe the clinical characteristics of users with systemic arterial hypertension and associated diabetes mellitus assisted by the Hiperdia system in a Family Health Strategy, relating to sociodemographic variables. Method: this is a quantitative, cross-sectional study, with 20 patients who answered a structured questionnaire. The Microsoft Office Excel 2007® program was used for data analysis. Results: there was predominance of females, elderly, white, married and with low education. There was a high prevalence of physical inactivity, a family history of hypertension or diabetes, low adherence to educational activities, with a diagnosis time greater than five years, sleep disturbance and the accomplishment of only two meals a day. Conclusion: it is concluded that the high prevalence of the simultaneous occurrence of risk factors such as physical inactivity, hereditary history, sleep disturbance and low number of daily meals shows social assistance voids. Furthermore, this study advances the multidimensional perspective of health care, as it encompasses variables beyond the disease.(AU)

Objetivo: describir las características clínicas de la clientela con hipertensión arterial sistémica y diabetes mellitus asociado acompañada del sistema Hiperdia en una Estrategia de Salud Familiar, en relación con variables sociodemográficas. Método: este es un estudio cuantitativo, transversal, con 20 pacientes que respondieron un cuestionario estructurado. El programa Microsoft Office Excel 2007® se utilizó para el análisis de datos. Resultados: predominó el sexo femenino, ancianas, blancas, casadas y con baja educación. Hubo una alta prevalencia de inactividad física, antecedentes familiares de hipertensión o diabetes, baja adherencia a actividades educativas, con un tiempo de diagnóstico superior a cinco años, trastornos del sueño y solo dos comidas al día. Conclusión: se concluye que la alta prevalencia de la ocurrencia simultánea de factores de riesgo como inactividad física, antecedentes hereditarios, trastornos del sueño y bajo número de comidas diarias muestra vacíos en la asistencia social. Además, este estudio avanza la perspectiva multidimensional de la atención médica, ya que abarca variables además de la enfermedad.(AU)

The physical education teachers and the passion for teaching activity in higher education / Os professores de educação física e a paixão pela atividade docente no ensino superior

ABSTRACT The aim of the study was to analyze the passion for teacher career and the level of satisfaction with life of Physical Education professionals who work at higher education considering the professional development cycles. The sample were 133 teachers who work in state universities of Paraná (Brazil). The instruments used for the research were the Passion Scale, The Satisfaction with life Scale and a socio demographic questionnaire. For data analysis were used the chi-square test and the logistic regressions binary and multinomial, considering a significance level of 95% (p<0,05). The results showed that most of the teachers were passionate by profession. It was verified that teachers who worked since 20 years or more had more level of harmonious passion (p=0,022). Moreover, was evidenced strong correlation between the Passion Criteria and the Harmonious Passion (r=0,626). In conclusion, the longer the teacher stays in the career more is the harmonious passion for work.

RESUMO O objetivo do estudo foi de analisar a paixão pela carreira docente e o nível de satisfação de vida dos profissionais de Educação Física que atuam no ensino superior considerando os ciclos de desenvolvimento profissional. A amostra foi constituída por 133 professores atuantes em universidades do estado do Paraná (Brasil). Os instrumentos utilizados na pesquisa foram a Escala da Paixão, a Escala de Satisfação com a Vida e o questionário sociodemográfico. Para a análise de dados empregou-se os testes qui-quadrado e regressões logísticas binária e multinomial, considerando o nível de significância de 95% (p<0,05). Os resultados revelaram que a maioria do professores são apaixonados pela profissão. Verificou-se que os professores que atuavam a 20 anos ou mais possuíam maior indice de paixão harmoniosa (p=0,022). Além disso, observou-se forte correlação entre o Critério da Paixão e a Paixão Harmoniosa (r=0,626). Por fim, conclui-se que quanto mais tempo o professor permanece na carreira maior é a paixão harmoniosa pelo trabalho.

Allogeneic hematopoietic stem cell transplantation in adults with myelodysplastic syndrome: Experience of the Argentinean Group of Bone Marrow Transplantation (GATMO).

INTRODUCTION: Allogeneic hematopoietic stem cell transplantation (AHSCT) is a curative approach for patients with myelodysplastic syndrome (MDS). METHODS: In this multicenter retrospective study, we analyzed the outcome of adult patients with MDS who underwent AHSCT in Argentina and evaluated the prognostic factors associated with progression-free survival (PFS), overall survival (OS), cumulative incidence (CI) of relapse, and non-relapse mortality (NRM). RESULTS: We analyzed data from 87 adults (median age: 43 years, range 18-66) who underwent SCT after myeloablative (n = 60) or non-myeloablative conditioning (n = 27), and from related (n = 62) or unrelated (n = 25) donors. For all patients, unadjusted 4-year PFS and OS were 37% and 38%, respectively; no significant differences were found between recipients of related or unrelated donors. One-year CI of relapse and NRM were 21% and 20%, respectively. In the multivariate analysis, intermediate disease risk index (DRI) and acute graft versus host disease AGVHD of all grades (I-IV) were independent variables associated with better PFS and lower relapse CI; only intermediate DRI was associated with better OS. CONCLUSIONS: AHSCT is a feasible procedure in Argentina, with more than 30% of the patients achieving long-term survival. Recipients with unrelated donors had at least similar outcome than those with related donors. DRI may be useful to identify patients at higher risk of relapse after transplantation.

O Parque do Ingá como espaço público de lazer: uma análise da percepção do ambiente / The Ingá park as leisure public space: an analysis of the perception of the environment

Esta pesquisa objetivou analisar o Parque do Ingá, no município de Maringá-PR, como espaço público de lazer, com vistas à identificação dos aspectos concernentes às dimensões física e sociocultural. A amostra foi constituída por 55 usuários do parque. Para a coleta de dados utilizamos o instrumento escala de autopercepção do ambiente para a realização de atividades físicas. Os resultados indicaram que, para os usuários do parque, os fatores político-normativos foram considerados como os mais inibidores (escore final de 2,8) e os socioculturais aparecem no topo da escala como contributo para o estímulo (escore final de 4,1). Como conclusão do estudo percebe-se a necessidade de ações planejadas que possam intervir positivamente para os aspectos geográficos, tecnológico-arquitetônico, político- normativos, culturais e sociais e que atendam os interesses da comunidade frequentadora do parque.

This study aimed to analyze the Ingá Park, in Maringá-PR, as a leisure public space, in order to identify issues related to physical and sociocultural dimensions. The sample consisted of 55 users of the park. To collect the data we used the environment self-perceived scale for performing physical activities. The results indicated that, for users of the park, the political-normative factors were considered as more inhibitors (final score of 2.8) and the sociocultural factors appear at the top of the scale as a contribution to the stimulation (final score of 4.1). As a conclusion of the study realizes the need for planned actions that can intervene positively for the geographical, technological-architectural, political-normative, cultural and social issues and that meet the interests of the park goers.

Allogeneic hematopoietic stem cell transplantation in pediatric myelodysplastic syndromes: a multicenter experience from Argentina.

BACKGROUND: Allogeneic hematopoietic stem cell transplantation (AHSCT) represents the only curative treatment for the majority of pediatric patients with Myelodysplastic Syndrome (MDS). We aimed to evaluate overall survival (OS), disease-free survival (DFS), non-relapse mortality (NRM) and relapse incidence in children who underwent AHSCT for MDS in six institutions from Argentina. PROCEDURE: A retrospective analysis of 54 AHSCT was carried out in 52 patients (mean age: 9 years; range: 2-19; 35 males). RESULTS: MDS subtypes were refractory cytopenia of childhood (RCC) (n: 26, 50%), refractory anemia with excess blasts (RAEB) (n: 9, 18%), RAEB in transformation (RAEB-T) (n: 8, 15%) and juvenile myelomonocytic leukemia (JMML) (n: 9, 17%). At time of transplant, seven (13%) patients transformed to acute myeloid leukemia (AML) and two patients with RCC to RAEB. Donors were related in 32 cases (59%) and the stem cells source was: bone marrow (63%), peripheral blood (26%), and umbilical cord blood (11%). Five-year DFS and OS were 50% and 55% respectively; and for patients with JMML, 57% and 67% respectively. Cumulative incidence of NRM and relapse were 27% and 21% respectively. In the multivariate analysis, umbilical cord blood (HR 4.07; P = 0.025) and age ≥ 9 years at transplantation (HR 3.28; P = 0.017) were associated with lower OS; age and graft-versus-host disease (GVHD) had a higher NRM. CONCLUSIONS: In our series, more than half of the patients achieved long term OS with AHSCT. Less toxic conditioning regimens or more intensive GVHD prophylaxis could lead to better results in some children.

Equipe de apoio e a construção coletiva do trabalho em Saúde Mental junto à Estratégia de Saúde da Família: espaço de discussão e de cuidado / Support team and collective elaboration of the Mental Healthcare work within the Family Health Program: space of discussion and care

A grande demanda por consulta psiquiátrica e psicoterapia e a necessidade de otimizar os recursos humanos em Saúde Mental têm conduzido à organização de novos modos de ação e suporte técnico aos profissionais de unidades com Estratégia de Saúde da Família. Uma Unidade Básica de Saúde de uma região da cidade de São Paulo iniciou a prática denominada Apoio Matricial, que consiste no suporte à identificação, tratamento, e proposta de solução dos problemas de saúde mental em conjunto com as equipes de Saúde da Família. O presente trabalho tem por objetivo relatar a experiência focada nesse processo de trabalho, tendo como horizonte ético os princípios do Sistema Único de Saúde e concepções técnicas e científicas mais humanizadas com vistas ao atendimento integral ao paciente e a sua inserção no meio social. Trata-se de um estudo de natureza qualitativa que utilizou a observação participante como meio para coletar os dados e analisar a situação. Consoante com a Reforma Psiquiátrica no Brasil, busca-se uma visão complementar e menos fragmentada do atendimento em Saúde Mental. A experiência mostrou que por meio do apoio matricial pode ser alcançada a necessária (re)organização dos serviços para acolher a demanda e efetivar a atenção em Saúde Mental de qualidade. Esse modo de operar, ao integrar Equipes de Saúde da Família e Unidade Básica Tradicional, permitiu a construção compartilhada do cuidado em saúde.

Estilo de liderança no contexto de treinadores de natação do Paraná / Leadership style in the context swimming coaches in the state of Paraná

Este estudo de cunho descritivo com caráter transversal teve como objetivo investigar o estilo de liderança dos treinadores de natação do Paraná. Os sujeitos da pesquisa foram 33 atletas (67,3%) do gênero masculino, 16 atletas (32,7%) do gênero feminino e 6 técnicos. A coleta de dados ocorreu durante a realização da fase final dos Jogos Abertos do Paraná 2010. O instrumento de medida utilizado foi a Escala de Liderança no Desporto (ELD). Para análise dos dados, utilizaram-se o teste de Shapiro Wilk, Mann-Whitney, a Manova e Post Hoc de Scheffe (p<0,05). Os resultados evidenciaram que na comparação entre a percepção e a preferência dos atletas houve diferença significativa nas dimensões treino-instrução (p=0,001), reforço (p=0,008) e estilo democrático (p=0,020); atletas do gênero masculino têm maior preferência pelo estilo autocrático do que atletas do gênero feminino (p=0,031); não houve diferença significativa entre as dimensões: percepção dos atletas e a autopercepção dos treinadores. Conclui-se: os treinadores paranaenses da modalidade de natação apresentam o estilo democrático, utilizando-se do reforço e do treino instrução como recursos técnico-táticos para os seus treinamentos; na equipe campeã, o treinador apresentou perfil semelhante entre o atual e o preferido dos atletas que, segundo a literatura, seria o ideal para alcançar o sucesso esportivo.

This descriptive cross-sectional study aimed to investigate the leadership style of swimming coaches from the state of Paraná, Brazil. The trial subjects were 33 male athletes (67.3%), 16 female athletes (32.7%), and six coaches. Data collection occurred during the final phase of the Parana 2010 Open Games. The measuring instrument used was the Leadership Scale for Sports (LSS). For data analysis we used the Shapiro Wilk test, Mann-Whitney, the Manova, and Post Hoc of Scheffe (p <0.05). The results showed that comparing between the perception and preference of athletes there were significant differences in dimension of training instruction (p = 0.001), reinforcement (p = 0.008), and democratic style (p = 0.020), male athletes have a greater preference for autocratic behavior than female athletes (p = 0.031), there was no significant difference between the dimensions: perception of athletes and self-perception of coaches. We reached the conclusion that the swimming coaches from the state of Paraná have the democratic style, using reinforcement and training-instruction as tactical and technical resources for their coaching; the champion team coach had a profile similar to what is currently preferred by athletes which, according to literature, would be the ideal one to achieve success in sports.

Hepatotoxicidade e síndrome DRESS induzida por anticonvulsivantes: relato de dois casos e revisão da literatura / Hepatotocicity and anticonvulsant induced DRESS syndrome: report of two cases and literature review

O termo DRESS (Drug Rash with Eosinophilia and Systemic Symptoms - erupção a medicamento com eosinofilia e sintomas sistêmicos) é uma reação de hipersensibilidade com características sistêmicas, sendo o acometimento hepático manifestação visceral mais comum. Objetivo: descrever dois casos de síndrome DRESS e hepatotoxicidade por carbamazepina e fenitoína, e fazer uma breve revisão da literatura. Relato de Casos: dois pacientes usuários de fármacos anticonvulsivantes apresentaram reações adversas com comprometimento do estado geral, erupção cutânea, eosinofilia periférica, aumento das aminotransferases e enzimas canaliculares. Após serem firmados os diagnósticos de síndrome DRESS e hepatotoxicidade, foram suspensos os anticonvulsivantes e introduzida corticoterapia sistêmica. Os pacientes evoluíram com importante melhora do quadro clínico-laboratorial. Conclusão: o reconhecimento precoce da DRESS é essencial pois o atraso no diagnóstico e na suspensão do medicamento desencadeante pode resultar no aumento da mortalidade.

The term DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) is a hypersensitivity reaction to drugs with systemic features, and liver involvement is its most common visceral manifestation. Objective: describe two cases of DRESS syndrome and hepatotoxicity by carbamazepine and phenytoin and make a brief literature review. Case reports: two patients taking anticonvulsants had adverse reactions with malaise, cutaneous rash, peripheral eosinophilia, increasing of canalicular and liver enzymes. After DRESS syndrome and hepatotoxicity diagnose, the anticonvulsants were discontinued and oral corticosteroids were introduced. The patients had significant clinical and laboratory improvement. Conclusion: early detection of DRESS is essential because delay in both diagnosis and discontinuation of the triggering medication can result in increasing mortality.

Partial and total monosomal karyotypes in myelodysplastic syndromes: comparative prognostic relevance among 421 patients.

Myelodysplastic syndromes (MDS) include a group of heterogeneous hematological disorders with a variable risk of leukemic evolution and short survival. Around 40-50% of patients show abnormal karyotypes that are mostly characterized by monosomies or deletions. Cytogenetic findings are an independent prognostic factor and the International prognostic scoring system (IPSS) differentiates three cytogenetic categories, despite the Intermediate one being heterogeneous. The aim of this study, including 421 Argentinean patients with primary MDS, is to characterize the cytogenetic profile, to test its prognostic value and to compare partial and monosomal karyotypes against other cytogenetic findings. An abnormal karyotype (median survival: 26 months) was observed in 176 patients. The presence of complex karyotypes, number of alterations, and the IPSS cytogenetic groups showed significant differences for predicting outcome. Behavior of patients with isolated deletions (median survival: 49 months) did not differ from those with normal karyotype (56 months, P = 0.654) or Good prognostic findings (43 months, P = 0.371). However, a worse prognosis was observed when another alteration was added (31 months, P = 0.043). Karyotypes with autosomal monosomies (median survival: 16 months) had a prognostic impact similar to other Poor cytogenetic findings (17 months, P = 0.626). In our population classified according to French-American-British (FAB) or World Health Organization (WHO), this new categorization of cytogenetic abnormalities, recognizing three different risk groups, showed an independent prognostic impact and a better discriminating power than the IPSS categories. It can be concluded that all isolate deletions (excluding 7q-) are good prognostic findings and all monosomies (excluding Y chromosome loss) are bad indicators.

Risk-adapted therapy with three or six cycles of doxorubicin/bleomycin/vinblastine/dacarbazine plus involved-field radiation therapy in Hodgkin lymphoma, based on prognosis at diagnosis and early response: results from the GATLA study.

BACKGROUND: Doxorubicin/bleomycin/vinblastine/dacarbazine (ABVD) plus involved-field radiation therapy (IFRT) is the gold-standard treatment for early and advanced stages of Hodgkin lymphoma (HL). We evaluated the outcomes of patients according to prognosis at diagnosis and over time to determine who achieved complete remission (CR). PATIENTS AND METHODS: Treatment-naive patients under the age of 75 years at all stages of HL were eligible. The favorable group (FG) contained patients with stage IA-IIIA disease without bulky areas who achieved CR after the third cycle of ABVD. They received only IFRT at 25 Gy. Patients in the unfavorable group (UG) exhibited stages IIIB and IV HL. The UG also included all patients with bulky disease and the subset of the FG without CR after 3 cycles of ABVD, ie, slow responders (FGSR). The UG received 6 cycles of ABVD plus IFRT at 30 Gy to bulky areas at diagnosis or to those areas remaining positive after the third cycle of ABVD. RESULTS: In total, 584 patients were evaluable: 285 of them belonged to the FG, and 299 to the UG. Rates of CR were 98% and 85% for the FG and the UG, respectively (P < .001). Sixty patients in the FG received 6 cycles of ABVD because they had not achieved CR after 3 cycles (ie, the FGSR subgroup). The 5-year event-free survival rate was 89% for the FG, 66% for the FGSR, and 72% for the UG (P < .001). The overall survival at 5 years was significantly better for the FG (98%) than for the FGSR (87%) and the UG (88%; P < .001). CONCLUSION: Patients from the FG demonstrated excellent outcomes compared with those from the FGSR and UG, despite receiving less chemotherapy and fewer doses of IFRT.