ABSTRACT
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.
Subject(s)
Fanconi Anemia/diet therapy , Hematopoietic Stem Cell Transplantation/methods , Nutritional Status/physiology , Transplantation Conditioning/methods , Adolescent , Child , Child, Preschool , Fanconi Anemia/pathology , Female , Follow-Up Studies , Humans , Male , Retrospective StudiesABSTRACT
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Subject(s)
Nutritional Status , Outcome Assessment, Health Care , Hematopoietic Stem Cell TransplantationABSTRACT
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.