Conhecimento de graduandos sobre doação de medula óssea / Knowledge of undergraduates about bone marrow donation

Caracterizar o conhecimento dos graduandos de uma instituição de ensino superior acerca do processo de doação de medula óssea. Método: Trata-se de um estudo descritivo com abordagem quantitativa. Foram entrevistados 266 graduandos, de ambos os sexos, entre 17 e 21 anos de idade. Foi utilizado um questionário estruturado, contendo perguntas sobre o conhecimento a respeito do processo de doação de medula óssea. Resultados: A maioria dos participantes não conhece o processo de cadastro e doação de medula óssea, tendo como a falta de informação a principal causa para a desinformação a respeito do tema abordado, consequentemente resultando em pouca demanda para que mais pessoas sejam cadastradas no REDOME. Conclusão: os estudantes do ensino superior desconhecem os processos que envolvem desde ao cadastro até a doação de medula óssea, devido à desinformação e pouca divulgação sobre a temática. (AU)

To characterize the knowledge of undergraduates from a higher education institution about the bone marrow donation process. Method: This is a descriptive study with a quantitative approach. 266 undergraduates were interviewed, of both sexes, between 17 and 21 years old. A structured questionnaire was used, containing questions about their knowledge about the bone marrow donation process. Results: Most participants do not know the bone marrow registration and donation process, with lack of information being the main cause for misinformation about the topic addressed, consequently resulting in little demand for more people to be registered in REDOME. Conclusion: the higher education students are unaware of the processes that involve from registration to bone marrow donation, due to misinformation and little dissemination on the subject. (AU)

Caracterizar el conocimiento de estudiantes de grado de una institución de educación superior sobre el proceso de donación de médula ósea. Método: Se trata de un estudio descriptivo con abordaje cuantitativo. Se entrevistaron 266 estudiantes universitarios, de ambos sexos, entre 17 y 21 años. Se utilizó un cuestionario estructurado que contenía preguntas sobre el conocimiento sobre el proceso de donación de médula ósea. Resultados: La mayoría de los participantes desconocen el proceso de registro y donación de médula ósea, siendo la falta de información la principal causa de la desinformación sobre el tema abordado, por lo que se genera poca demanda para que más personas se registren en REDOME. Conclusión: los estudiantes de educación superior desconocen los procesos que involucran desde el registro hasta la donación de médula ósea, debido a la desinformación y poca difusión sobre el tema. (AU)

Common mental disorders in hematopoietic stem cell transplant patients: a scoping review / Trastornos mentales comunes en pacientes con trasplante de células madre hematopoyéticas: revisión del alcance / Transtornos mentais comuns em pacientes transplantados com células-tronco hematopoéticas: revisão de escopo

ABSTRACT Objective: to map common recurrent mental disorders in patients undergoing hematopoietic stem cell transplantation. Methods: this is a scoping review carried out in January 2022 in electronic databases and repositories of dissertations and thesis. Studies that answered the research question, met the objective of the study and were available in full electronically, in any language, were included. Results: the sample consisted of 28 studies, 14 of which were published in the United States of America. The common mental disorders found were depressive, anxiety, post-traumatic stress and mood disorders. Twenty symptoms were mentioned, among the most prevalent are fatigue and sleep disorders/insomnia. Conclusions: the difficulty and importance of carrying out the differential diagnosis of these disorders were highlighted, since their symptoms can be confused with other health problems and have a strong potential to interfere with patients' evolution.

RESUMEN Objetivo: mapear los trastornos mentales recurrentes comunes en pacientes sometidos a trasplante de células madre hematopoyéticas. Métodos: se trata de una revisión de alcance realizada en enero de 2022 en bases de datos electrónicas y repositorios de disertaciones y tesis. Se incluyeron publicaciones que respondieron a la pregunta de investigación, cumplieron con el objetivo del estudio y estaban disponibles en su totalidad en formato electrónico, en cualquier idioma. Resultados: la muestra estuvo compuesta por 28 estudios, 14 de los cuales fueron publicados en los Estados Unidos de América. Los trastornos mentales comunes encontrados fueron depresión, ansiedad, estrés postraumático y trastornos del estado de ánimo. Se mencionaron 20 síntomas, entre los más prevalentes se encuentran fatiga y trastornos del sueño/insomnio. Conclusiones: se destacó la dificultad e importancia de realizar el diagnóstico diferencial de estos trastornos, ya que sus síntomas pueden confundirse con otros problemas de salud y tienen un fuerte potencial de interferir en la evolución del paciente.

RESUMO Objetivo: mapear os transtornos mentais comuns recorrentes em pacientes submetidos ao transplante de células-tronco hematopoéticas. Métodos: trata-se de revisão de escopo realizada em janeiro de 2022 em bases de dados eletrônicas e repositórios de dissertações e tese. Foram incluídas publicações que respondessem à questão de pesquisa, atendessem ao objetivo do estudo e que estivessem disponíveis na íntegra em meio eletrônico, em qualquer idioma. Resultados: a amostra foi composta por 28 estudos, dos quais 14 foram publicados nos Estados Unidos da América. Os transtornos mentais comuns encontrados foram os transtornos depressivos, de ansiedade, estresse pós-traumático e de humor. Foram citados 20 sintomas, entre os mais prevalentes estão a fadiga e distúrbios do sono/insônia. Conclusões: evidenciaram-se a dificuldade e a importância de realizar o diagnóstico diferencial desses transtornos, uma vez que seus sintomas podem ser confundidos com outros problemas de saúde e têm forte potencial para interferir na evolução do paciente.

Insuficiencia cardiaca secundaria a amiloidosis AL: diagnóstico y tratamiento / Heart failure secondary to amyloidosis AL: diagnosis and treatment

Resumen La amiloidosis por depósito de cadenas livianas de inmunoglobulinas (AL) es una enfermedad poco frecuen te y subdiagnosticada. El mejor tratamiento disponible al momento es el trasplante autólogo de médula ósea (TMO). El compromiso cardíaco es el principal determi nante pronóstico en esta patología y en ocasiones un impedimento para recibir el TMO. Se presenta el caso de un varón de 44 años que consultó por signos y síntomas de insuficiencia cardiaca (IC) con biomarcadores cardia cos elevados. Se realizó un ecocardiograma transtorácico donde se objetivó aumento de espesores parietales con hipoquinesia global y fracción de eyección deteriorada en grado leve (50%). El paciente se internó en unidad coronaria para balance negativo y para estudio etiológico del cuadro. Ante la sospecha de enfermedad infiltrativa, se solicitaron un centellograma óseo con pirofosfato y cadenas livianas libres en suero. El centellograma óseo resultó no sugestivo para amiloidosis por transtiretina y las cadenas livianas libres mostraron una relación me nor a 0.26 con predominio lambda. Se realizó una biopsia de encía que confirmó el diagnóstico de amiloidosis AL. Posterior al diagnóstico comenzó tratamiento qui mioterápico específico con Ciclofosfamida, Bortezomib y Dexametasona (esquema CYBORD) y Daratumumab. Evolucionó con IC refractaria por lo que ingresó a lista de trasplante cardiaco, recibiendo el mismo al poco tiempo con buena evolución. Esto permitió reiniciar el esquema quimioterápico y en segundo término finalmente recibir el TMO, con buena evolución.

Abstract Light chain amyloidosis (AL) is a rare and underdi agnosed disease. The best treatment available is au tologous bone marrow transplantation (BMT). Cardiac involvement is the main prognostic determinant in this pathology and sometimes an impediment to re ceive BMT. We present a clinical case of a 44-year-old who consulted for signs and symptoms of heart failure (HF) with elevated cardiac biomarkers. A transthoracic echocardiogram showed increased wall thickness with global hypokinesia and mildly impaired ejection fraction (50%). The patient was admitted to the coronary unit for treatment with diuretics and for etiological study of the condition. In view of the suspicion of infiltrative disease, a bone scintigraphy with pyrophosphate and free light chains in serum were requested. The bone scintigraphy was not suggestive of transthyretin amyloidosis and the free light chains showed a ratio of less than 0.26 with lambda predominance. A gum biopsy was per formed and confirmed the diagnosis of AL amyloidosis. After diagnosis, specific chemotherapy treatment with Cyclophosphamide, Bortezomib and Dexamethasone (CYBORD scheme) and Daratumumab was started. He evolved with refractory HF so it was decided to admit him to the cardiac transplantation list, receiving the same soon after, with good evolution. This allowed the patient to restart the chemotherapy regimen and finally receive BMT, with good evolution.

Calidad de vida y toxicidad financiera de los receptores de trasplantes de células madre hematopoyéticas en COVID-19 / Quality of life and financial toxicity of hematopoietic stem cell transplant recipients in COVID-19 / Qualidade de vida e toxicidade financeira dos transplantados de células-tronco hematopoéticas na COVID-19

Objetivo: evaluar y correlacionar la calidad de vida y la toxicidad financiera de pacientes adultos sometidos a trasplante de células madre hematopoyéticas durante el período de la pandemia de COVID-19. Método: estudio observacional, analítico, realizado con 35 pacientes en un hospital de referencia para trasplante en Latinoamérica. Para la recolección de datos, se utilizaron los cuestionarios Functional Assessment Cancer Therapy Bone Marrow Transplantation y el COmprehensive Score for financial Toxicity. Para el análisis de los datos se utilizaron las pruebas de correlación de Spearman y Mann-Whitney. Resultados: la calidad de vida general durante la COVID-19 mostró un puntaje bajo (67,09/108) con mayor deterioro en el bienestar funcional (14,47/28), bienestar social (16,76/28) y preocupaciones adicionales (23,41/40). Los promedios del grupo alogénico fueron inferiores a los del grupo autólogo en todos los dominios, presentando diferencia significativa en relación a preocupaciones adicionales (p=0,01) y en el índice de evaluación del tratamiento (p=0,04). Se consideró que la toxicidad financiera tenía un impacto leve (22.11/44). Se observó una relación, aunque no significativa, entre la calidad de vida y la toxicidad financiera (p=0,051). Conclusión: la calidad de vida de la muestra fue baja; existe una correlación entre la calidad de vida y la toxicidad financiera, aunque no significativa. Cuanto mayor es la toxicidad financiera, menor es la calidad de vida.

Objective: to evaluate and correlate the quality of life and financial toxicity of adult patients undergoing hematopoietic stem cell transplantation during the COVID-19 pandemic. Method: observational, analytical study, carried out with 35 patients in a reference hospital for transplantation in Latin America. For data collection, the Functional Assessment Cancer Therapy Bone Marrow Transplantation and COmprehensive Score for Financial Toxicity questionnaires were used. Spearman and Mann-Whitney correlation tests were used for data analysis. Results: general quality of life during COVID-19 had a low score (67.09/108) with greater impairment in functional well-being (14.47/28), social well-being (16.76/28) and additional concerns (23.41/40). The means of the allogeneic group were lower than those of the autologous group in all domains, showing a significant difference in relation to additional concerns (p=0.01) and in the treatment evaluation index (p=0.04). Financial toxicity was considered to have a slight impact (22.11/44). There was a relationship, albeit not significant, between quality of life and financial toxicity (p=0.051). Conclusion: the quality of life of the sample was low; there is a correlation between quality of life and financial toxicity, although not significant. The higher the financial toxicity, the lower the quality of life.

Objetivo: avaliar e correlacionar a qualidade de vida e a toxicidade financeira dos pacientes adultos submetidos ao transplante de células-tronco hematopoéticas no período da pandemia de COVID-19. Método: estudo observacional, analítico, realizado com 35 pacientes em um hospital de referência para o transplante na América Latina. Para coleta de dados, utilizaram-se os questionários Functional Assessment Cancer Therapy Bone Marrow Transplantation e COmprehensive Score for financial Toxicity. Na análise dos dados empregaram-se os testes de correlação de Spearman e Mann-Whitney. Resultados: a qualidade de vida geral, durante a COVID-19, apresentou baixo escore (67,09/108), com maior comprometimento nas funções bem-estar funcional (14,47/28), social (16,76/28) e preocupações adicionais (23,41/40). As médias do grupo alogênico foram inferiores às do autólogo em todos os domínios, apresentando diferença significativa em relação às preocupações adicionais (p=0,01) e ao índice de avaliação do tratamento (p=0,04). A toxicidade financeira foi considerada de impacto leve (22,11/44). Observou-se relação, ainda que não significativa, entre a qualidade de vida e a toxicidade financeira (p=0,051). Conclusão: a qualidade de vida da amostra foi baixa, logo há uma correlação entre qualidade de vida e a toxicidade financeira, embora não significativa. Quanto maior a toxicidade financeira, menor a qualidade de vida.

Doença granulomatosa crônica: características clínicas, seguimento e terapêutica de cinco pacientes pediátricos / Chronic granulomatous disease: clinical characteristics, follow-up, and therapy of 5 pediatric patients

Introdução: A doença granulomatosa crônica (DGC) é caracterizada por um defeito na capacidade microbicida das células fagocíticas (monócitos e neutrófilos), com alta mortalidade se não diagnosticada precocemente. Os pacientes apresentam infecções recorrentes ou graves, suscetibilidade a granulomas em órgãos profundos, doenças autoimunes e doença inflamatória intestinal. Objetivo e Método: Relato de aspectos clínicos e do tratamento de cinco pacientes com doença granulomatosa crônica. Resultados: Cinco pacientes, três meninos, medianas de idade no início dos sintomas e diagnóstico de 8 meses e 48 meses, respectivamente, foram estudados por um período de 10 anos. Pneumonia (5/5) e doença micobacteriana (3/5) foram as manifestações iniciais mais comuns. Alterações pulmonares foram observadas em todos os casos. Mutações nos genes CYBB e NCF1 foram identificadas em três casos. Antibioticoprofilaxia foi instituída em todos os pacientes e três foram submetidos ao transplante de células tronco-hematopoiéticas (TCH), aos 7, 18 e 19 anos e com sobrevida atual entre 4 a 5 anos. Conclusão: O monitoramento cuidadoso de infecções graves com tratamento imediato foi crucial para a sobrevivência. O TCH, mesmo ao final da adolescência, promoveu a cura da DGC em três pacientes.

Introduction: Chronic granulomatous disease (CGD) is characterized by a defective microbicidal capacity of phagocytic cells (monocytes and neutrophils) with high mortality if not early diagnosed. Patients have recurrent or severe infections and are susceptible to granulomas in visceral organs, autoimmune diseases, and inflammatory bowel diseases. Objective and Method: To report the clinical features and treatment of 5 patients with CGD. Results: Five patients, 3 boys, with median ages at symptom onset and diagnosis of 8 months and 48 months, respectively, were followed for 10 years. Pneumonia (5/5) and mycobacterial disease (3/5) were the most common initial manifestations. Pulmonary changes were observed in all cases. Mutations in the CYBB and NCF1 genes were identified in 3 cases. All patients received antibiotic prophylaxis. Three patients underwent a hematopoietic stem cell transplant (HSCT) at 7, 18, and 19 years, with current survival of 4 to 5 years. Conclusion: Careful monitoring for severe infection with prompt treatment was crucial for survival. Even though HSCT was performed in late adolescence, it promoted the cure of CGD in 3 patients.

Survival benefit of pediatric-based regimen for adults with acute lymphoblastic leukemia: A single-center retrospective cohort

ABSTRACT Introduction: Improving survival of Acute Lymphoblastic Leukemia (ALL) in adult patients has been a challenge. Despite intensive chemotherapy treatment, overall survival is poor. However, several studies demonstrate that young adult patients have better survival when treated with pediatric-based intensive regimens. Considering these results, We decided to treat newly diagnosed ALL patients according to age and risk factors. The goal of this study was to describe the results of this intensive chemotherapy treatment approach for ALL adult patients diagnosed at our institution. Methods: Fifty-eight ALL patients, diagnosed from 2004 to 2013, were included in the analysis. Patients were assigned to either the St. Jude Total Therapy XIIIB high-risk arm (St Jude) or the CALGB 8811 (CALGB). The Kaplan-Meier survival curve was used for the survival analyses and the Cox proportional hazard regression, for multivariable analysis. Results: The overall survival was 22.9% at 10 years. The St. Jude improved survival, compared to the CALGB (p = 0.007), with 32.6% vs. 7.4% survival rate at 10 years. However, no survival benefit was found for patients younger than 20 years old (p = 0.32). The multivariable analysis demonstrated that undetectable minimal residual disease (MRD) and hematopoietic stem cell transplantation (HSCT) had beneficial impact on survival (p = 0.0007 and p = 0.004, respectively). Conclusion: ALL is a disease of poor prognosis for adults. The joint effort to standardize treatment and seek solutions is the way to start improving this scenario.

One-year mortality of hematopoietic stem cell recipients admitted to an intensive care unit in a dedicated Brazilian cancer center: a retrospective cohort study

ABSTRACT BACKGROUND: Hematopoietic stem cell transplantation (HSCT) recipients requiring intensive care unit (ICU) admission early after transplantation have a poor prognosis. However, many studies have only focused on allogeneic HSCT recipients. OBJECTIVES: To describe the characteristics of HSCT recipients admitted to the ICU shortly after transplantation and assess differences in 1-year mortality between autologous and allogeneic HSCT recipients. DESIGN AND SETTING: A single-center retrospective cohort study in a cancer center in Brazil. METHODS: We included all consecutive patients who underwent HSCT less than a year before ICU admission between 2009 and 2018. We collected clinical and demographic data and assessed the 1-year mortality of all patients. The effect of allogeneic HSCT compared with autologous HSCT on 1-year mortality risk was evaluated in an unadjusted model and an adjusted Cox proportional hazard model for age and Sequential Organ Failure Assessment (SOFA) at admission. RESULTS: Of the 942 patients who underwent HSCT during the study period, 83 (8.8%) were included in the study (autologous HSCT = 57 [68.7%], allogeneic HSCT = 26 [31.3%]). At 1 year after ICU admission, 21 (36.8%) and 18 (69.2%) patients who underwent autologous and allogeneic HSCT, respectively, had died. Allogeneic HSCT was associated with increased 1-year mortality (unadjusted hazard ratio, HR = 2.79 [confidence interval, CI, 95%, 1.48-5.26]; adjusted HR = 2.62 [CI 95%, 1.29-5.31]). CONCLUSION: Allogeneic HSCT recipients admitted to the ICU had higher short- and long-term mortality rates than autologous HSCT recipients, even after adjusting for age and severity at ICU admission.

The effect of platelet infusion on the repair of bone marrow hematopoietic niche damage in mice induced by (60)Co radiation and hematopoietic reconstruction after bone marrow transplantation / 中华血液学杂志

Objective: To observe the effect of platelets on hematopoietic stem cell (HSCs) implantation in mice with radiation-induced bone marrow injury and bone marrow transplantation models. Methods: ①Male C57BL/6 mice were divided into a single irradiation group and a radiation infusion group after receiving (60)Co semimyeloablative irradiation for 18-10 weeks. The irradiation infusion group received 1×10(8) platelets expressing GFP fluorescent protein. ② The allogeneic bone marrow transplantation model was established. The experimental groups included the simple transplantation group (BMT) and the transplantation infusion group (BMT+PLT). The BMT group was infused through the tail vein only 5 × 10(6) bone marrow cells, the BMT+PLT group needs to be infused with bone marrow cells at the same time 1× 10(8) platelets. ③ Test indicators included peripheral blood cell and bone marrow cell counts, flow cytometry to detect the proportion of hematopoietic stem cell (HSC) and hematopoietic progenitor cells, bone marrow cell proliferation and apoptosis, and pathological observation of vascular niche damage and repair. Results: ①On the 3rd, 7th, 14(th), and 21st days after irradiation, the bone marrow cell count of the infusion group was higher than that in the single irradiation group (P<0.05), and the peripheral blood cell count was also higher. A statistically significant difference was found between the white blood cell count on the 21st day and the platelet count on the 7th day (P<0.05). In the observation cycle, the percentage of bone marrow cell proliferation in the infusion group was higher, while the percentage of apoptosis was lower. ② The results of bone tissue immunofluorescence after irradiation showed that the continuity of hematopoietic niche with red fluorescence was better in the irradiation infusion group. ③The chimerism percentage in the BMT+PLT group was always higher than that in the BMT group after transplantation.④ The BMT+PLT group had higher bone marrow cell count and percentage of bone marrow cell proliferation on the 7th and 28th day after transplantation than that in the BMT group, and the percentage of bone marrow cell apoptosis on the 14th day was lower than that in the BMT group (P<0.05). After the 14th day, the percentage of stem progenitor cells in the bone marrow cells of mice was higher than that in the BMT group (P<0.05). ⑤The immunohistochemical results of bone marrow tissue showed that the continuity of vascular endothelium in the BMT+PLT group was better than that in the BMT group. Conclusion: Platelet transfusion can alleviate the injury of vascular niche, promotes HSC homing, and is beneficial to hematopoietic reconstruction.

Molecular profile of patients with myelofibrosis: a 10-year experience

ABSTRACT Objective To analyze the karyotype test and myeloid panel with next-generation sequencing findings in patients with myelofibrosis, and to compare transplant characteristics in patients referred for bone marrow transplantation. Methods Retrospective, single-center study with patients diagnosed with myelofibrosis treated at Hospital Israelita Albert Einstein between 2010 and 2020. Results A total of 104 patients with myelofibrosis were examined. Patients who had not been submitted to tests in our service were excluded. The final sample comprised 69 patients. Of these 69, 56 were submitted to karyotyping and 22 to myeloid panel with next-generation sequencing. Karyotype was normal in 60% of the patients and altered in 40%. The prevalence of high-risk molecular mutations was higher in patients referred for bone marrow transplantation (100% versus 50%). The median follow-up of transplant patients was 2.4 years and the overall survival at 2 years was 80% (95%CI: 62-100%). Conclusion The molecular analysis enables estimating the patient's risk and thus instituting more aggressive treatment such as bone marrow transplant for patients at higher risk, being a relevant tool to guide therapy. Given the significance of molecular analysis for therapeutic decision-making in myelofibrosis, collection and disclosure of data on the prevalence of cytogenetic changes and findings of next-generation sequencing in affected patients is important.

Assessment of oral health condition in recipients of allogeneic hematopoietic cell transplantation

ABSTRACT Objective: The objective of this study was to evaluate the oral health condition and dental treatments performed in patients in pre-allogeneic HCT. Method: The records of patients treated during 2018 at a Brazilian HCT service were reviewed. The following oral health data were obtained: 1. Decayed, missing and filled teeth / correlated index for primary dentition (DMFT/dmft); 2. Quality of oral hygiene and 3. Dental pathologies: 3.1 Periodontal infectious focus, 3.2 Endodontic infectious focus and 3.3 Carie incidence. All dental procedures performed were surveyed. Results: Thirty-three patients were included, with a mean age of 28.42 (±16.37), 20 male (60%) and 13 female. The average DMFT/dmft found in this study was 10.24 (± 8.37), similar to the index found in the population in southeastern Brazil. The younger study population presented a DMFT/dmft considered high, when compared to the general population. A total of 27.2% of the patients had active caries lesions, 33.3%, foci of periodontal infection, 15.1%, endodontic infectious focus and 40%, poor oral hygiene. Almost half of the patients (48.4%) had to undergo dental intervention, 24.2% needing periodontal scaling, 21.2%, fillings and 12.1%, tooth extractions. Conclusion: We conclude that the studied population had an important incidence of dental pathologies and infectious conditions that could complicate throughout HCT, especially in younger patients, therefore presenting a high demand for dental treatment in the pre-HCT. Studies that assess the impact of dental conditioning on the outcomes of HCT with an emphasis on dental infectious complications, days of hospitalization and survival are necessary."

Impacto de la calidad de vida relacionada a la salud en pacientes pediátricos con trasplante de progenitores hematopoyéticos en una institución colombiana / Impact of health-related quality of life on pediatric patients who underwent hematopoietic stem cell transplantation in a Colombian institution

Introducción. El trasplante de células madre hematopoyéticas es la opción curativa para algunas enfermedades y está aumentando el tiempo de supervivencia de los pacientes. La calidad de vida relacionada con la salud en estos pacientes no se evalúa de manera sistemática. Objetivos. Describir la calidad de vida relacionada con la salud y las complicaciones en niños con trasplante de células madre hematopoyéticas. Materiales y métodos. Es un estudio transversal en pacientes pediátricos sobrevivientes al trasplante. Se midió la calidad de vida relacionada con la salud, utilizando el cuestionario KIDSCREEN-27 en pacientes entre 8 y 14 años y la SF-12™ (Short Form-12) en pacientes mayores de 14 años. El análisis estadístico se realizó en el software Stata 12. Utilizamos el modelo de Rasch, trasladando estimación de parámetros a valores t para obtener el resultado de los cuestionarios. Resultados. En total, 42 pacientes respondieron alguno de los cuestionarios. Los eventos adversos más frecuentes fueron "enfermedad crónica de injerto Vs. contra huésped" y "complicaciones endocrinas". De acuerdo con la normalidad de datos del KIDSCREEN-27, los puntajes de las dimensiones "ambiente escolar" y "soporte social y pares" fueron inferiores al percentil 50. En el cuestionario SF-12™, el grupo que utilizaba inmunosupresores tuvo un menor puntaje en el componente físico. Conclusiones. En general, los resultados del KIDSCREEN-27 sugieren un cierto déficit de calidad de vida en pacientes entre 8 y 14 años. Los cuestionarios mostraron confiabilidad en la muestra.

Introduction: Hematopoietic stem cell transplantation is the curative option for some diseases and is increasing patient survival. The health-related quality of life in these patients is not systematically evaluated. Objectives: The present study sought to describe the health-related quality of life and complications in children who underwent hematopoietic stem cell transplantation. Materials and methods: A cross-sectional study was conducted on pediatric transplanted survivors. Health-related quality of life was measured using the KIDSCREEN-27 scale and Short Form-12 (SF-12) in patients between 8 and 14 years of age and those over 14 years, respectively. Statistical analysis was performed using STATA 12 software. We used the Rasch model person parameter estimates translated into T-values to score the questionnaire. Results: A total of 42 children answered the questionnaires. The most frequent adverse events were chronic graft Vs. host disease and endocrine complications. According to European norm data in the KIDSCREEN-12 scale, scores for the school dimension and social and peer support were below the 50th. percentile. The group administered immunosuppressants had lower scores on the physical component of the SF-12™ scale. Conclusions: In general, the KIDSCREEN-27 does appear to suggest some quality-of-life deficit in younger children. The scales showed reliability in this population.

Transpedicular impaction and grafting of allogeneic bone containing enriched bone marrow in the treatment of stage III Kümmell′s disease / 中华创伤杂志

Objective:To compare the clinical effect of transpedicular impaction and grafting of allogeneic bone containing enriched bone marrow combined with posterior internal fixation and posterior subtotal vertebrectomy combined with posterior internal fixation in the treatment of stage III Kümmell′s disease.Methods:A retrospective cohort study was made on clinical data of 40 patients with stage III Kümmell′s disease admitted to Zhengzhou Orthopedic Hospital from June 2015 to December 2018. There were 10 males and 30 females, at age range of 57-79 years［(67.7±6.1)years］. A total of 19 patients were treated by transpedicular impaction and grafting of allogeneic bone containing enriched bone marrow combined with posterior internal fixation (impaction bone graft group), and 21 patients by posterior subtotal vertebrectomy combined with posterior internal fixation (subtotal vertebrectomy group). Operation time and intraoperative blood loss were compared between the two groups. Degree of pain, lumbar dysfunction and degree of kyphosis were evaluated by visual analogue scale (VAS), Japanese Orthopedic Association (JOA) score and kyphotic Cobb angle before operation, at 1 week after operation and at the last follow-up. Bone healing time was compared between the two groups. The complications of the two groups were observed.Results:All patients were followed up for 25-64 months［(40.6±10.4)months］. Operation time and intraoperative blood loss were (130.0±10.1)minutes and (284.5±43.5)ml in impaction bone graft group, lower than those in subtotal vertebrectomy group［(253.8±33.2)minutes, (889.1±95.7)ml］(both P<0.01). There were no significant differences in VAS, JOA score or kyphotic Cobb angle between the two groups before operation, at 1 week after operation and at the last follow-up (all P>0.05). Both VAS and JOA score showed significant differences within each group at any time point (all P<0.01). In both groups, the kyphotic Cobb angle reduced significantly at 1 week after operation when compared with that before operation (all P<0.01), and the angle showed a slight increase at the last follow-up, but remained significantly lower than that before operation (all P<0.01). There were no relapse of pain or aggravation of kyphosis. Bone healing time in impaction bone graft group［4.4(4.0, 5.0)months］was significantly shorter than that in subtotal vertebrectomy group［6.4(5.2, 8.1)months］( P<0.01). There were 2 patients with delayed healing of surgical incision in impaction bone graft group, with the complication rate of 11%. There were 2 patients with dural tear and 3 patients with delayed healing of surgical incision in subtotal vertebrectomy group, with the complication rate of 24%. The complication rate was not statistically significant between the two groups ( P>0.05). No loosening or breakage of internal fixation was observed during the follow-up. Conclusions:Transpedicular impaction and grafting of allogeneic bone containing enriched bone marrow combined with posterior internal fixation and posterior subtotal vertebrectomy combined with posterior internal fixation are effective in the treatment of stage III Kümmell disease. However, the former can shorten the operation time, reduce the intraoperative blood loss and accelerate the healing of injured vertebral bone, suggesting a relatively minimally invasive surgical method for reconstruction and maintenance of spinal biomechanical stability.

Effects of guided imagery relaxation in hematopoietic stem-cell transplantation patients: a quasi-experimental study / Efecto de la relajación con imagen guiada en trasplantados de células madre hematopoyéticas: estudio cuasi experimental / Efeito do relaxamento com imagem guiada em transplantados de células-tronco hematopoéticas: estudo quase experimental

ABSTRACT Objective: To analyze the effects of the technique of virtual reality guided imagery in the vital signs of hematopoietic stem-cell transplantation patients. Method: Quasi-experimental study with 35 participants who received an intervention using virtual reality guided imagery with progressive muscle relaxation, applied three times a week for four weeks in a referral hospital for transplants in the south of Brazil. Data collected included: temperature, arterial pressure, respiratory rate, heart rate, pain, and oxygen saturation, before and after each intervention. The comparisons were analyzed using Wilcoxon's test. Results: There was a clinical significance between the mean measurements before and after for respiratory rate (p=0.00) in all stages, and for the variables Heart rate, Temperature, and Oxygen saturation from the 1st to the 12th measurements (p=0.05). Conclusion: The intervention was low cost, easy to apply, and showed positive effects, presenting itself as an option for patient-focused care.

RESUMEN Objetivo: Analizar efectos de técnica de imagen guiada por realidad virtual en los signos vitales de trasplantados de células madre hematopoyéticas. Método: Estudio cuasi experimental con 35 participantes que recibieron intervención de imagen guiada por realidad virtual con relajación muscular progresivo, aplicada tres veces por semana durante cuatro semanas, en hospital de referencia en trasplante del Sur de Brasil. Los datos recolectados fueron: temperatura, presión arterial, respiración, frecuencia cardíaca, dolor y saturación de oxígeno, antes y después de cada intervención. Las comparaciones analizadas por test de Wilcoxon. Resultados: Hubo significación clínica entre las medianas de los contrastes de antes y después para Frecuencia respiratoria (p=0,00) en todas etapas; y en las variables Frecuencia cardíaca, Temperatura y Saturación de oxígeno, entre la 1ª hasta 12ª evaluación (p=0,05). Conclusión: Los efectos de la intervención mostrados favorables, bajo costo y fácil realización, siendo una opción de acción de cuidado centrado en el paciente.

RESUMO Objetivo: Analisar os efeitos da técnica de imagem guiada por realidade virtual nos sinais vitais de transplantados de células-tronco hematopoéticas. Método: Estudo quase experimental com 35 participantes que receberam intervenção de imagem guiada por realidade virtual com relaxamento muscular progressivo, aplicada três vezes por semana durante quatro semanas, em um hospital de referência em transplante no Sul do Brasil. Os dados coletados foram: temperatura, pressão arterial, respiração, frequência cardíaca, dor e saturação de oxigênio, antes e depois de cada intervenção. As comparações foram analisadas pelo teste de Wilcoxon. Resultados: Houve significância clínica entre as médias das aferições de antes e depois para Frequência respiratória (p=0,00) em todas as etapas; e nas variáveis Frequência cardíaca, Temperatura e Saturação de oxigênio, entre a 1ª até 12ª avaliação (p=0,05). Conclusão: Os efeitos da intervenção se mostraram favoráveis, de baixo custo e fácil realização, sendo uma opção de ação de cuidado centrado no paciente.

Análise dos fatores associados ao retransplante de células-tronco hematopoiéticas: estudo caso-controle / Analysis of factors associated with hematopoietic stem-cell retransplantation: a case-control study / Análisis de factores asociados al Retrasplante de Células Madre Hematopoyéticas: un estudio de caso-control

Resumo Objetivo: analisar os fatores associados ao insucesso do Transplante de Células-Tronco Hematopoiéticas (TCTH) em pacientes submetidos ao retransplante de Células-Tronco Hematopoiéticas (RCTH). Método: estudo quantitativo do tipo caso-controle para avaliar pacientes submetidos ao RCTH. Para tanto, utilizou-se amostra pareada de dois controles para cada caso (2:1). O grupo caso foi constituído pelos prontuários de saúde com todos os pacientes que foram submetidos ao RCTH (28) e o grupo controle (56) incluiu pacientes que receberam apenas um transplante. Três variáveis nortearam o pareamento: sexo, diagnóstico e tipo de transplante. Resultados: vinte e quatro (85,71%) pacientes do grupo caso receberam retransplante devido a recidiva da doença e quatro (14.29%) devido a falha do enxerto. Uma diferença estatística foi encontrada na análise entre os pacientes que não usaram o ácido ursodesoxicólico, analgésicos opioides ou imunossupressores. A necessidade de um RCTH entre aqueles que usaram estes medicamentos de forma inapropriada foi 16,12, 12,79 e 4,5 vezes maior, respectivamente, do que entre os que as usaram corretamente. Conclusão: houve uma diferença relacionada ao motivo que levou ao retransplante e os indivíduos analisados. A conclusão é que a razão preditiva para retransplante nesta amostra foi a recidiva da doença.

Abstract Objective: to analyze the factors associated with the failure of Hematopoietic Stem Cell Transplantation (HSCT) in patients undergoing Hematopoietic Stem Cell Retransplantation (HSCR). Method: this study implemented a quantitative approach and was a case-control type which addressed patients undergoing HSCR. To do so, a paired sample of two controls was used for each case (2:1). The case group consisted of the medical records of all patients who underwent HSCR (28) and the control group (56) of those who underwent only one transplant. Three variables guided the pairing: gender, diagnosis and type of transplant. Results: a total of 24 (85.71%) patients in the case group were re-transplanted due to disease relapse and four (14.29%) due to graft failure. There was a statistical difference in the analysis between patients who did not use ursodeoxycholic acid, opioid analgesics and immunosuppressants. The need for HSCR among those who used these medications inappropriately was 16.12, 12.79 and 4.5 times more likely, respectively, than those who used them correctly. Conclusion: there was a difference regarding the reasons which led to the retransplantation and the analyzed subjects, and this study concluded that the predictive reason for retransplantation in the studied sample was disease relapse.

Resumen Objetivo: analizar los factores asociados con el fracaso del Trasplante de Células Madre Hematopoyéticas (TCMH) en pacientes sometidos al Retrasplante de Células Madre Hematopoyéticas (RCMH). Método: estudio cuantitativo de tipo caso-control que abordó pacientes sometidos al RCMH. Para esto, se utilizó una muestra pareada de dos controles para cada caso (2:1). El grupo caso estuvo formado por los registros médicos de todos los pacientes que fueron sometidos al RCMH (28) y el grupo control (56) por los que fueron sometidos a un solo trasplante. Tres variables guiaron el emparejamiento: género, diagnóstico y tipo de trasplante. Resultados: un total de 24 (85.71%) pacientes en el grupo caso fueron retransplantados debido a la recaída de la enfermedad y 4 (14.29%) por el fracaso del injerto. Hubo una diferencia estadística en el análisis entre los pacientes que no usaron ácido ursodesoxicólico, analgésicos opioides e inmunosupresores. La necesidad de RCMH entre los que usaron estos medicamentos de manera inapropiada se encontraba 16,12 - 12,79 y 4,5 veces más probable, respectivamente, que aquellos que los usaron correctamente. Conclusión: hubo diferencia en cuanto a las razones que llevaron al retrasplante de los sujetos analizados. Este estudio concluyó que la razón predictiva del retrasplante, en la muestra estudiada, fue la recidiva de la enfermedad.

Neurotoxicidades apresentadas por pacientes submetidos ao transplante de células-tronco hematopoéticas: uma revisão de escopo / Neurotoxicidades en pacientes sometidos al trasplante de células madre hematopoyéticas: una revisión de alcance / Neurotoxicity presented by patients undergoing hematopoietic stem cell transplantation: a scoping review

Resumo Objetivo Mapear os tipos de neurotoxicidades apresentadas por pacientes submetidos ao Transplante de Células-Tronco Hematopoéticas. Métodos Trata-se de uma Scoping Review, orientada a partir do método proposto pelo Joanna Briggs Institute, e seguiu as recomendações do Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews. A busca pelos estudos foi realizada entre os meses de julho e agosto de 2020 e ocorreu nas bases de dados e em portais de teses e dissertações nacionais e internacionais. Resultados A amostra final foi composta por 71 artigos científicos, todos na língua Inglesa. Houve destaque para o ano de 2018 com 14 (19%) publicações. Observou-se prevalência de estudos realizados nos Estados Unidos da América com 29 (40,8%). No tocante a população, todos (100%) os artigos são sobre pacientes submetidos ao transplante de células-tronco hematopoéticas que apresentaram neurotoxicidades. Acerca dos quimioterápicos utilizados no regime de condicionamento pré-transplante de células-tronco hematopoéticas sete (9,8%) utilizaram a combinação de Fludarabina e Ciclofosfamida, seguida da Ciclosporina e Tacrolimus em seis (8,4%), ciclosporina em quatro (5,6%), Fludarabina em três (4,2%). Quanto às neurotoxicidades apresentadas em pacientes submetidos ao transplante, evidenciou-se a síndrome de encefalopatia reversível posterior em 19 (26,7%) estudos. Cabe ressaltar que outros estudos identificaram essa síndrome, porém relataram sintomas diferentes. Conclusão As neurotoxicidades apresentadas por pacientes submetidos ao transplante de células-tronco hematopoéticas, são encefalopatia reversível posterior, leucoencefalopatia reversível posterior, encefalopatia de Wernicke, encefalopatia hipertensiva, encefalopatia metabólica, encefalopatia límbica, complicações hemorrágicas e convulsões.

Resumen Objetivo Mapear los tipos de neurotoxicidades en pacientes sometidos al trasplante de células madre hematopoyéticas. Métodos Se trata de una Scoping Review, orientada a partir del método propuesto por el Joanna Briggs Institute, que siguió las recomendaciones del Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews. La búsqueda de los estudios se realizó entre los meses de julio y agosto de 2020 y ocurrió en las bases de datos y en portales de tesis de doctorado y maestría nacionales e internacionales. Resultados La muestra final estuvo compuesta por 71 artículos científicos, todos en lengua inglesa. Se destacó el año 2018 con 14 publicaciones (19 %). Se observó una prevalencia de estudios realizados en Estados Unidos de América con 29 (40,8 %). En lo que se refiere a la población, todos los artículos (100 %) tratan sobre pacientes sometidos al trasplante de células madre hematopoyéticas que presentaron neurotoxicidades. Sobre los quimioterápicos utilizados en el régimen de acondicionamiento previo al trasplante de células madre hematopoyéticas, siete (9,8 %) utilizaron la combinación de Fludarabina y Ciclofosfamida, seguida de la Ciclosporina y Tacrolimus en seis (8,4 %), ciclosporina en cuatro (5,6 %), Fludarabina en tres (4,2 %). Con relación a las neurotoxicidades en pacientes sometidos al trasplante, se evidenció el síndrome de encefalopatía reversible posterior en 19 estudios (26,7 %). Cabe destacar que otros estudios identificaron ese síndrome, pero refirieron síntomas distintos. Conclusión Las neurotoxicidades presentadas por pacientes sometidos al trasplante de células madre hematopoyéticas son encefalopatía reversible posterior, leuco encefalopatía reversible posterior, encefalopatía de Wernicke, encefalopatía hipertensiva, encefalopatía metabólica, encefalopatía límbica, complicaciones hemorrágicas y convulsiones.

Abstract Objective To map the types of neurotoxicity presented by patients undergoing Hematopoietic Stem Cell Transplantation. Methods This is a scoping review, guided by the method proposed by the Joanna Briggs Institute, and followed the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews recommendations. The search for studies was carried out between the months of July and August 2020 and took place in databases and in national and international theses and dissertations portals. Results The final sample consisted of 71 scientific articles, all in English. There was a highlight for the year 2018 with 14 (19%) publications. There was a prevalence of studies carried out in the United States of America with 29 (40.8%). Regarding the population, all (100%) articles are about patients undergoing hematopoietic stem cell transplantation who presented neurotoxicity. Regarding the chemotherapeutic agents used in the pre-transplantation regimen of hematopoietic stem cells, seven (9.8%) used the combination of Fludarabine and Cyclophosphamide, followed by Cyclosporine and Tacrolimus in six (8.4%), Cyclosporine in four (5.6%), Fludarabine in three (4.2%). As for the neurotoxicity presented in patients undergoing transplantation, the posterior reversible encephalopathy syndrome was evidenced in 19 (26.7%) studies. It should be noted that other studies have identified this syndrome, but have reported different symptoms. Conclusion The neurotoxicity presented by patients undergoing hematopoietic stem cell transplantation are posterior reversible encephalopathy, posterior reversible leukoencephalopathy, Wernicke's encephalopathy, hypertensive encephalopathy, metabolic encephalopathy, limbic encephalopathy, hemorrhagic complications and seizures.

Bone Marrow Transplantation: bereavement due to the impact of bodily and psychosocial changes from the perspective of adolescent recipients and their parents / Transplante de medula óssea: luto decorrente do impacto das modificações corporais e psicossociais na perspectiva de pacientes adolescentes e seus pais

The diagnosis and treatment of a life-threatening disease have adverse consequences on child development and impact the family. This study aimed to investigate bereavement due to the impact of bodily and psychosocial changes resulting from Bone Marrow Transplantation from the perspective of adolescent patients and their parents. This is a qualitative, descriptive, exploratory, cross-sectional study. Nine dyads (patients and mothers / fathers) were included. Data collection took place through semi-structured audio-recorded interviews. The thematic analysis made it possible to elaborate three categories: losses due to illness; new losses experienced with treatment; learning to appreciate the gains of adverse experience. The results show that transplantation has repercussions that transcend the physical dimension and include social harm, changes in body image, and loss of friends. However, once the radical journey of transplantation is over, the participants positively connoted their experience, identifying benefits that go beyond health recovery, such as emotional maturation, improvement of self-esteem, and the ability to socialize.

O diagnóstico e tratamento de uma doença ameaçadora à continuidade da vida repercutem no desenvolvimento infantil e impactam a família. Este estudo teve por objetivo investigar o luto relacionado ao impacto das modificações corporais e psicossociais decorrentes do transplante de medula óssea na perspectiva de pacientes adolescentes e seus pais. Trata-se de um estudo qualitativo, descritivo e exploratório, de corte transversal. Participaram do estudo nove díades (pacientes e mães/pais). A coleta de dados se deu por meio de entrevistas semiestruturadas audiogravadas. A análise temática possibilitou elaborar três categorias: perdas decorrentes do adoecimento; novas perdas vivenciadas com o tratamento e aprendendo a apreciar os ganhos da experiência adversa. Os resultados mostram que o transplante repercute em agravos que transcendem a dimensão física e incluem prejuízos sociais, alterações da imagem corporal e perda de amigos. No entanto, uma vez ultrapassada a jornada radical do transplante, os participantes conotam positivamente a experiência vivida, identificando benefícios que ultrapassam a recuperação da saúde física, como amadurecimento emocional, melhora na autoestima e na capacidade de socialização.

O papel da odontologia no diagnóstico da doença enxerto-contra-hospedeiro / The role of dentistry in the diagnosis of Graft-versus-host disease

Objetivo: Elucidar quais as formas mais adequadas de se proceder para que haja um efetivo diagnóstico da Doença Enxerto-Contra-Hospedeiro pelo cirurgião-dentista. Revisão de literatura: A Doença Enxerto Contra Hospedeiro acomete diversos pacientes que realizam transplante de células tronco hematopoiéticas. Os sinais clínicos muitas vezes são manifestados apenas na cavidade bucal e, por isso, é importante que o cirurgião-dentista tenha conhecimento. Esta patologia é proveniente de uma complicação do TCTH (Transplante de Células Tronco Hematopoiéticas) alogênico, onde ocorre uma resposta de ativação de linfócitos T do doador e um reconhecimento de antígenos contra o receptor. Discussão: As alterações mais evidentes são lesões ulceradas, estrias brancas, mucocele, leucoedema, lesões bolhosas, ardência, dor e xerostomia. Estes aspectos tendem a corresponder a um diagnóstico de lesões malignas, síndrome de Sjogren, lúpus e líquen plano. A biópsia é fundamental para o diagnóstico, assim como para graduar a severidade da Doença Enxerto Contra Hospedeiro. Conclusão: Caso o cirurgião-dentista identifique algum dos sinais, deverá alertar à equipe médica do paciente para iniciar o tratamento e evitar a recidiva da doença original.

Aim: elucidate which are the most appropriate ways to proceed so that there is an effective diagnosis of Graft--versus-Host Disease by the dentist. Literature review: Graft-versus-host disease affects several patients who undergo hematopoietic stem cell transplantation. Clinical signs are often manifested in the oral cavity only, rea-son why it is important the dentist to be aware of them. This pathology is caused by a complication of allogeneic HSCT, in which there is an activation response of donor T lymphocytes and a recognition of antigens against the recipient. Discussion: The most evident changes are ulcerated lesions, white streaks, mucocele, leukedema, bullous lesions, burning, pain and Xerostomia. These aspects tend to correspond to a diagnosis of malignant lesions, Sjogren's syndrome, lupus and lichen planus. A biopsy is essential for diagnosis as well as for grading the severity of Graft versus Host Disease. Conclusion: If the dental surgeon identifies any of the mentioned signs, he must alert the patient's medical team to start treatment to prevent the recurrence of the original disease.

Effects of physical exercise for children and adolescents undergoing hematopoietic stem cell transplantation: a systematic review and meta-analysis

Hematopoietic stem cell transplantation (HSCT) is a treatment that requires long periods of hospitalization. The mobility restrictions result in physical, functional and psychological impairments. Physical exercise is a therapy that can restore physical and functional capacities; however, it is necessary to understand the effects of its practice in post-HSCT individuals. The purpose of this systematic review (SR) was to assess the impact of physical exercise in children and adolescents undergoing HSCT. The SR was conducted following the PRISMA guidelines through search in the electronic databases Embase, Lilacs, PEDro, PubMed and SCOPUS, without limitation of dates and languages. Randomized or non-randomized clinical trials with children and adolescents who underwent HSCT, aged between 3 to 19 years old, who participated in a regular physical activity program, were assessed. After removing duplicates and selecting studies according to the eligibility criteria, seven parallel studies incorporating hospitalized and discharged participants undertaking aerobic and strengthening exercises were included in this study. The main outcomes analyzed were exercise capacity, quality-of-life, body composition and freedom. Five studies comprised the meta-analysis regarding the effects of the distance walked in the 6-min walk test and quality-of-life. Physical exercise is considered to be safe, feasible and efficacious to prevent the decline of the quality-of-life in children and adolescents undergoing HCST, as well as a considerable improvement in physical capacity.