Short-term follow-up of the nutritional status of children with Fanconi anemia undergoing hematopoietic stem cell transplant.

OBJECTIVE: The objective of this study was to evaluate the nutritional status of children diagnosed with Fanconi anemia (FA) during hematopoietic stem cell transplant (HSCT), comparing it with healthy children and children with other hematologic diseases. METHODS: Observational retrospective study was conducted with patients submitted to HSCT in a period of 5 years. We assessed anthropometric and biochemical data, food intake, and gastrointestinal complications in 49 FA patients. We compared the anthropometric information with those of transplanted patients with other diagnoses (n = 54) in three periods (pre-transplant, 15 and 30 days after the HSCT), and with a group of healthy children (n = 24). RESULTS: Throughout the post-HSCT period, there was a significant decline in the nutritional status of FA patients: 83.3% presented weight loss equal to or greater than 5%. A progressive decrease in food intake after the transplantation was observed, with weekly deficits reaching 7841.3 kcal and 347.6 g of protein (both p < 0.05). When comparing FA with other diagnoses patients, the former displayed a poorer nutritional status prior to HSCT (p < 0.01 for BMI/age z-score), and that difference was maintained during the transplant (p < 0.01 for the same parameter), with similar weight loss values for both groups (8.99 vs 7.91%, respectively; p > 0.05). When compared to the control group of healthy children, FA patients prior HSCT showed substantially lower z-scores for Ht./age (p < 0.01) and BMI/age (p < 0.05). CONCLUSION: Although FA patients demonstrated poorer nutritional status as compared to other diagnosis and healthy children, the decline of anthropometric measures along the treatment is similar to other transplanted patients, imposing a greater risk to FA patients.

Pre-transplant arm muscle area: a simple measure to identify patients at risk.

PURPOSE: This study aims to explore parameters of nutritional assessment and food intake as predictors of clinical outcomes after hematopoietic stem cell transplant (HSCT). METHODS: The study was conducted retrospectively with 56 adult patients undergoing allogeneic HSCT. Energy and protein daily intake were used to calculate the energy and protein cumulative deficit. The nutritional assessment tools were weight, body mass index (BMI), triceps skinfold (TSF), and arm muscle area (AMA) before HSCT and after HSCT. RESULTS: Food intake decreased immediately after the transplant and the energy and protein cumulative deficit increased during hospitalization (p < 0.01). Almost 70 % of patients had severe weight loss (greater than 5 %), and the average percentage of weight loss was 8.5 ± 3.9 %. BMI and weight loss percentage were not correlated with the presence of acute graft versus host disease (GVHD) and mortality within 180 days in this population. On the other hand, correlation was found between the initial (pre-HSCT) AMA equal to or below the 15th percentile, with the presence of acute GVHD (p = 0.024), and mortality within 180 days after HSCT (p = 0.010). CONCLUSIONS: The AMA measured pre-HSCT showed to be a potential predictor of acute GVHD and mortality up to 180 days after transplant in adult patients.

Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: children and adolescents.

The Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: Children and Adolescents was developed by dietitians, physicians, and pediatric hematologists from 10 Brazilian reference centers in hematopoietic stem cell transplantation. The aim was to emphasize the importance of nutritional status and body composition during treatment, as well as the main characteristics related to patient´s nutritional assessment. This consensus is intended to improve and standardize nutrition therapy during hematopoietic stem cell transplantation. The consensus was approved by the Brazilian Society of Bone Marrow Transplantation.

Phase angle as a screening tool for mortality risk among hematopoietic stem cell transplanted adult patients.

BACKGROUND: Mortality among adult patients undergoing hematopoietic stem cell transplantation (HSCT) is high, especially within the first 100 days after the event. Therefore, identifying prognostic factors would be useful as screening tools to protect patients at risk throughout early intervention. In our previous work, the standardized phase angle (SPA) was explored as a useful indicator of survival and nutritional status among children and adolescent within the first 180 days after HSCT. The aim of this study was to evaluate the SPA and the arm muscle area (AMA) as prognostic indicators of mortality and nutritional status among adults in the same population. METHODS: This study was conducted with 29 adult patients undergoing allogeneic HSCT and 28 controls. Anthropometric assessment as well as body composition and laboratory data were analyzed. The phase angle was standardized according to reference values for healthy population. The correlation of SPA and AMA with other variables was verified and sensibility and specificity were tested by constructing ROC curves considering mortality and nutritional status as outcomes. Kaplan-Meier analysis was applied to calculate survival considering the cut-off points found in ROC curves. Chi-squared test and Kappa coefficient were used for evaluate the agreement among methods of nutritional assessment. RESULTS: SPA presented a predictive value for mortality and nutritional status considering the cut-off point at -0.19. In fact, the mortality incidence was higher among patients with values below the cut-off point for SPA as compared to the ones with SPA above this value up to 90 days after the HSCT. Regarding to AMA, mortality was higher using the values bellow P15 (percentile 15) as reference. The average SPA decreased after the beginning of conditioning and after the HSCT, while the decrease of AMA was observed only 90 days after the transplant. CONCLUSIONS: In this study SPA was confirmed as a prognostic tool for adult HSCT patients. In addition, it seems that SPA is more sensitive to detect structural body changes among the transplanted patients as compared to AMA. More studies are needed to confirm it as a tool to screen patients at risk of mortality for early intervention.

Body composition of Fanconi anemia patients after hematopoietic stem cell transplantation.

INTRODUCTION: Fanconi anemia is a rare genetic disease linked to bone marrow failure; a possible treatment is hematopoietic stem cell transplantation. Changes in the nutritional status of Fanconi anemia patients are not very well known. This study aimed to characterize body composition of adult, children and adolescent patients with Fanconi anemia who were submitted to hematopoietic stem cell transplantation or not. METHODS: This cross-sectional study enrolled 63 patients (29 adults and 34 children and adolescents). Body composition was assessed based on diverse methods, including triceps skin fold, arm circumference, arm muscle area and bioelectrical impedance analysis, as there is no established consensus for this population. Body mass index was also considered as reference according to age. RESULTS: Almost half (48.3%) of the transplanted adult patients were underweight considering body mass index whereas eutrophic status was observed in 66.7% of the children and adolescents submitted to hematopoietic stem cell transplantation and in 80% of those who were not. At least 50% of all groups displayed muscle mass depletion. Half of the transplanted children and adolescents presented short/very short stature for age. CONCLUSION: All patients presented low muscle stores, underweight was common in adults, and short stature was common in children and adolescents. More studies are needed to detect whether muscle mass loss measured at the early stages of treatment results in higher risk of mortality, considering the importance of muscle mass as an essential body component to prevent mortality related to infectious and non-infectious diseases and the malnutrition inherent to Fanconi anemia.

Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: children and adolescents / Consenso Brasileiro de Nutrição em Transplante de Células-Tronco Hematopoiéticas: crianças e adolescentes

ABSTRACT The Brazilian Nutritional Consensus in Hematopoietic Stem Cell Transplantation: Children and Adolescents was developed by dietitians, physicians, and pediatric hematologists from 10 Brazilian reference centers in hematopoietic stem cell transplantation. The aim was to emphasize the importance of nutritional status and body composition during treatment, as well as the main characteristics related to patient´s nutritional assessment. This consensus is intended to improve and standardize nutrition therapy during hematopoietic stem cell transplantation. The consensus was approved by the Brazilian Society of Bone Marrow Transplantation.

RESUMO O Consenso Brasileiro de Nutrição em Transplante de Células-Tronco Hematopoiéticas: crianças e adolescentes foi elaborado com a participação de nutricionistas, médicos nutrólogos e médicos hematologistas pediátricos de 10 centros brasileiros que são referência em transplante de células-tronco hematopoiéticas. O objetivo foi salientar a importância do estado nutricional e da composição corporal durante o tratamento, bem como as principais características relacionadas à avaliação nutricional do paciente. As intenções, ao se estabelecer o consenso, foram aprimorar e padronizar a terapia nutricional durante o transplante de células-tronco hematopoiéticas. O consenso foi aprovado pela Sociedade Brasileira de Transplante de Médula Óssea.

Vitamin D deficiency in children and adolescents submitted to hematopoietic stem cell transplantation.

BACKGROUND: Sub-optimal levels of vitamin D have been found to be highly prevalent in all age groups, with epidemiologic studies demonstrating a link between vitamin D deficiency and disease susceptibility, such as infection and cancer, and mortality rates. In adult transplant patients, it has been suggested that the immunomodulatory properties of vitamin D may have an important role in the prevention and treatment of graft-versus-host disease. OBJECTIVE: The objective of this study was to assess serum 25-hydroxyvitamin D levels of children and adolescents submitted to allogeneic hematopoietic stem cell transplantation. METHODS: Serum 25-hydroxyvitamin D levels of 66 patients, aged 4-20 years, were assessed at three stages: before hospitalization for hematopoietic stem cell transplantation and at 30 and 180 days after hematopoietic stem cell transplantation. The control group consisted of 25 healthy children. RESULTS: At the pre-hematopoietic stem cell transplantation stage, patients had lower levels of 25-hydroxyvitamin D compared to controls (25.7 ± 12.3 ng/mL vs. 31.9 ± 9.9 ng/mL; p-value = 0.01), and a higher prevalence of 25-hydroxyvitamin D deficiency (32% vs. 8%; p-value = 0.01). Prevalence increased significantly after hematopoietic stem cell transplantation (p-value = 0.01) with half of the patients having vitamin D deficiency at 180 days after transplantation. At this stage, mean serum 25-hydroxyvitamin D levels were 20.9 ± 10.9 ng/mL, a significant decline in relation to baseline (p-value = 0.01). No correlation was found between 25-hydroxyvitamin D levels and vitamin D intake, graft-versus-host disease, corticoid use or survival rates. CONCLUSION: Low levels of 25-hydroxyvitamin D were detected even before hematopoietic stem cell transplantation and were significantly lower at 180 days after hematopoietic stem cell transplantation, thus recommending vitamin D supplementation for children and adolescents submitted to hematopoietic stem cell transplantation.

Bone mineral density, vitamin D, and nutritional status of children submitted to hematopoietic stem cell transplantation.

OBJECTIVE: The aim of the study was to evaluate the effect of allogeneic hematopoietic stem cell transplantation (HSCT) on bone mineral density (BMD), serum vitamin D levels, and nutritional status of 50 patients between ages 4 and 20 y. METHODS: We conducted pre-HSCT and 6-mo post-HSCT evaluations. We measured BMD at the lumbar spine (LS) and total body (TB) by dual energy x-ray absorptiometry (DXA); body composition by bioimpedance analysis, and dietary intakes of calcium and vitamin D using the 24-h recall and semiquantitative food frequency questionnaire methods. RESULTS: We observed a significant reduction in BMD 6 mo post-HSCT. Nearly half (48%) of patients had reductions at the LS (average -9.6% ± 6.0%), and patients who developed graft-versus-host disease (GVHD) had the greatest reductions (-5.6% versus 1.2%, P < 0.01). We also found reductions in serum levels of 25-hydroxyvitamin D (25-OHD), from 25.6 ± 10.9 ng/dL to 20.4 ± 11.4 ng/dL (P < 0.05), and in body weight. Corticosteroid treatment duration, severity of chronic GVHD, serum 25-OHD levels, and family history of osteoporosis were all risk factors associated with variations in BMD at the LS. CONCLUSION: HSCT in children and adolescents negatively effects their BMD, nutritional status, and vitamin D levels. We suggest that early routine assessment be done to permit prevention and treatment.

Segurança e Benefício do Uso de Probióticos em Pacientes Submetidos ao TCTH: Revisão Integrativa / Safety and Benefit of Using Probiotics in Patients Undergoing HSCT: Integrative Review / Seguridad y Beneficio del Uso de Probióticos en Pacientes Sometidos al TCTH: Revisión Integradora

Introdução: O transplante de células-tronco hematopoiéticas (TCTH) é um dos potenciais tratamentos curativos utilizados para pacientes com doenças hematológicas e outras doenças imunes. Durante o transplante, o paciente é submetido ao condicionamento e a outros tratamentos, como radioterapia e quimioterapia, o que pode causar a perda da diversidade da microbiota intestinal. A manipulação da microbiota intestinal com probióticos vem sendo apontada como uma estratégia de prevenção de complicações nos pacientes submetidos ao TCTH. Objetivo: Identificar se há evidências científicas relacionadas à segurança e aos benefícios da utilização de probióticos em pacientes submetidos ao TCTH. Método: Revisão integrativa com base em estudos que abordassem o uso de probióticos para o caso específico de pacientes submetidos ao TCTH publicados entre 2000 a 2018. Resultados: Foram selecionados cinco estudos que atenderam aos critérios de inclusão e exclusão, com um total de 52 pacientes. A utilização de probióticos na prevenção e/ou tratamento da diarreia tem mostrado resultados positivos em pacientes com diarreia induzida por antibióticos ou por infecções bacterianas, porém os estudos ainda não destacam benefícios no uso de probióticos no caso específico de pacientes submetidos ao TCTH. Poucos estudos mostram o uso de probióticos para auxílio na melhora dos sintomas associados a infecções ou bacteremias em pacientes imunossuprimidos. Conclusão: O uso de probióticos na população submetida ao TCTH e em imunossuprimidos ainda é controverso, sendo necessários mais estudos que demonstrem os benefícios no uso dessa estratégia para esse público.

Introduction: Hematopoietic stem cell transplantation (HSCT) is one of the potential curative treatments used for patients with hematological and other immune diseases. During transplantation, the patient undergoes conditioning and other treatments, such as radiotherapy and chemotherapy, which may cause loss of the intestinal microbiota diversity. The manipulation of the intestinal microbiota with probiotics has been pointed out as a strategy to prevent complications in patients undergoing HSCT. Objective: To identify if there is scientific evidence related to the safety and benefits of the use of probiotics in patients submitted to HSCT. Method: Integrative review based on studies addressing the use of probiotics for the specific case of patients undergoing HSCT published between 2000 and 2018. Results: Five studies that met the inclusion and exclusion criteria were eligible, with a total of 52 patients. The use of probiotics in the prevention and/or treatment of diarrhea has shown positive results in patients with antibiotic-induced diarrhea or bacterial infections, but the studies do not yet emphasize the benefits of using probiotics in the specific case of patients submitted to HSCT. Few studies show the use of probiotics to help the improvement of the symptoms associated to infections or bacteremia in immunosuppressed patients. Conclusion: The use of probiotics in the population submitted to HSCT and immunosuppressed is still controversial, and further studies are necessary to demonstrate the benefits of using probiotics for this public.

Introducción: El trasplante de células madre de las hematopoyéticas (TCTH) es uno de los posibles tratamientos curativos utilizados para pacientes con enfermedades hematológicas y otras enfermedades inmunes. Durante el transplante, el paciente es sometido al condicionamiento ya otros tratamientos, como radioterapia y quimioterapia, lo que puede causar la pérdida de la diversidad de la microbiota intestinal. La manipulación de la microbiota intestinal con probióticos viene siendo apuntada como una estrategia de prevención de complicaciones en los pacientes sometidos al TCTH. Objetivo: Identificar si hay evidencias científicas relacionadas con la seguridad y beneficios de la utilización de probióticos en pacientes sometidos al TCTH. Método: Revisión integradora basada em estúdios que abordan el uso de probióticos para el caso específico de pacientes sometidos a TCMH publicados entre 2000 y 2018. Resultados: Fueron elegibles 4 estudios que atendieron a los criterios de inclusión y exclusión, con un total de 52 pacientes. La utilización de probióticos en la prevención y/o tratamiento de la diarrea ha mostrado resultados positivos en pacientes con diarrea inducida por antibióticos o por infecciones bacterianas, pero los estudios aún no aportan beneficios en el uso de probióticos en pacientes sometidos al TCTH. Pocos estudios muestran infecciones o bacterias en pacientes inmunosuprimidos que utilizaron probióticos para ayudar en la mejora de los síntomas asociados al tratamiento. Conclusión: El uso de probióticos en la población sometida al TCTH e inmunosuprimidos aún es controvertido, siendo necesarios más estudios que comprueben los beneficios en el uso de probióticos para este público.

Phase angle from BIA as a prognostic and nutritional status tool for children and adolescents undergoing hematopoietic stem cell transplantation.

BACKGROUND & AIMS: The phase angle (PA) has been considered a prognostic and nutritional status indicator in several clinical situations considering its use as a body cell mass estimate value. The aim of this study was to evaluate the association between PA and other methods of nutritional assessment, as well as its prognostic value for children and adolescents undergoing hematopoietic stem cell transplantation (HSCT). METHODS: The nutritional status of 67 patients and 35 controls was assessed by bioelectric impedance analysis and anthropometric measurements. The phase angle was calculated and expressed in degrees and standardized (SPA) according to reference values. The Kaplan-Meier method was used to calculate survival and Kappa coefficient to determine the concordance between the SPA and other parameters of nutritional assessment. The correlation was established by using Pearson's correlation analysis. RESULTS: SPA progressively decreased during the treatment. The agreement between SPA and the diagnosis of malnutrition was moderate to %ideal weight, %TSF and %AMC and weak for BMI and weight/age z-score. Patients who had severe weight loss after transplantation had lower levels of SPA as compared to the group that did not lose weight (p < 0.001). Patients who developed chronic graft-versus-host disease had lower levels of SPA (p = 0.02), as well as patients who had been using corticosteroids during the post-transplant phase (p = 0.03). In this case, there was an inverse correlation between drug dose and SPA (p = 0.01). Patients with SPA ≤ 0 SD had a lower survival time as compared to the group of patients with SPA > 0 SD (p = 0.02) and showed an increased risk of death of 5.1as compared to the other group (95% CI: 1.41-18.94). CONCLUSION: The present study suggests that SPA could be useful as an indicator of survival and nutritional status for patients undergoing HSCT.

Body composition of Fanconi anemia patients after hematopoietic stem cell transplantation

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Vitamin D deficiency in children and adolescents submitted to hematopoietic stem cell transplantation

Background: Sub-optimal levels of vitamin D have been found to be highly prevalent in all age groups, with epidemiologic studies demonstrating a link between vitamin D deficiency and disease susceptibility, such as infection and cancer, and mortality rates. In adult transplant patients, it has been suggested that the immunomodulatory properties of vitamin D may have an important role in the prevention and treatment of graft-versus-host disease. Objective: The objective of this study was to assess serum 25-hydroxyvitamin D levels of children and adolescents submitted to allogeneic hematopoietic stem cell transplantation. Methods: Serum 25-hydroxyvitamin D levels of 66 patients, aged 4-20 years, were assessed at three stages: before hospitalization for hematopoietic stem cell transplantation and at 30 and 180 days after hematopoietic stem cell transplantation. The control group consisted of 25 healthy children. Results: At the pre-hematopoietic stem cell transplantation stage, patients had lower levels of 25-hydroxyvitamin D compared to controls (25.7 ± 12.3 ng/mL vs. 31.9 ± 9.9 ng/mL; p-value = 0.01), and a higher prevalence of 25-hydroxyvitamin D deficiency (32% vs. 8%; p-value = 0.01). Prevalence increased significantly after hematopoietic stem cell transplantation (p-value = 0.01) with half of the patients having vitamin D deficiency at 180 days after transplantation. At this stage, mean serum 25-hydroxyvitamin D levels were 20.9 ± 10.9 ng/mL, a significant decline in relation to baseline (p-value = 0.01). No correlation was found between 25-hydroxyvitamin D levels and vitamin D intake, graft-versus-host disease, corticoid use or survival rates...