Body composition of Fanconi anemia patients after hematopoietic stem cell transplantation

Abstract

IntroductionFanconi 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.MethodsThis 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.ResultsAlmost 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.ConclusionAll 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.

Nutritional and Body Composition Changes in Paediatric β-Thalassemia Patients Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Study Using Bioelectrical Impedance Analysis

“…These alterations underscore the substantial physiological impact of HSCT on pediatric patients with β-TM. Studies have revealed that most children undergoing HSCT have normal BMI at admission, 36 however, decreases in BMI, and weight loss during the post-transplant weeks have been detected. 37 The American Society for Parenteral and Enteral Nutrition (A.S.P.E.N) and the European Society for Parenteral and Enteral Nutrition (E.S.P.E.N) have stated that children undergoing HSCT are at nutritional risk and that all children undergoing HSCT should be evaluated for nutrition status.…”

Development of specific growth charts for children with Fanconi anemia

“…This demonstrates that children with FA, especially males, can be naturally thin, although there is greater variability in weight than height (Figures 1 and 2). Low weight in individuals with FA may be due to nutritional deficits (Petryk et al, 2015), lower muscle mass (Silva et al, 2017), and lower bone mass (Giri et al, 2007); however, other studies have found normal bone mineral density (BMD) when…”

“…This demonstrates that children with FA, especially males, can be naturally thin, although there is greater variability in weight than height (Figures 1 and 2). Low weight in individuals with FA may be due to nutritional deficits (Petryk et al, 2015), lower muscle mass (Silva et al, 2017), and lower bone mass (Giri et al, 2007); however, other studies have found normal bone mineral density (BMD) when adjusting for height age (Rose et al, 2012). Based upon the weight and BMI differences identified in our study and the increased risk of insulin resistance present in individuals with FA (Petryk et al, 2015), further studies characterizing lean and fat mass in FA are warranted.…”

“…Nutritional factors leading to poor growth in FA are due to a combination of insulin deficiency and/or sensitivity, increased caloric needs, and decreased oral intake (potentially due to underlying gastrointestinal issues) (Petryk et al, 2015). Low weight and BMI in FA may also be due to low muscle mass (Silva et al, 2017). However, body mass index (BMI) has been reported by Rose et al (2012) to be −0.6 SDS for males and −0.8 SDS for females, while Giri et al (2007) also found BMI to be normal for 51% of individuals with FA and overweight for 27%.…”

Body composition assessment in hematopoietic stem cell transplantation

“…In this issue of the Revista Brasileira de Hematologia e Hemoterapia , da Silva et al present data from a cross-sectional study evaluating a diverse set of body composition parameters in Fanconi Anemia patients aged 2–40 years, most of whom underwent HSCT. 9 The authors assessed BMI, referenced according to age in children and adolescents, and LBM of transplant recipients at various lengths of follow-up (0.5–27 years). They also analyzed a variety of complimentary assessment tools: triceps skin fold (TSF), arm circumference (AC), arm muscle area (AMA), and BIA with a standardized calculation for phase angle.…”