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• W1965190302 abstract "Fanconi Anemia (FA) is a rare disease characterized by bone marrow failure, congenital abnormalities and a predisposition to cancer. Patients (pts) with FA usually have low birth weight, short stature and fatigue. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice for pts that develop hematological abnormalities but it increases energy expenditure due to complications related to this procedure and decreases oral ingestion by the presence of gastrointestinal symptoms. Evaluate the changes in nutritional status, in food intake, in bone mineral density (BMD) and in vitamin D serum levels in children and adolescents with FA who underwent allogeneic HSCT in a single institution. Pts were evaluated before and 180 days after HSCT. We evaluated the lean mass (LM) by electrical bioimpedance, nutritional status by anthropometric measurements, the food intake by a 24 hours recall, the BMD by DXA (HOLOGIC-1000) and the serum 25-hydroxyvitamin D (25-OHD). Between 08/2006 and 09/2008, twenty-four patients were included in the study. Seven pts (29%) died before D+180 and were excluded from this analysis. 17 pts were able to complete this survey. The median age at admission to the BMT Unit was 8,5ys (5-16ys), 9 pts (53%) were female and 10 pts (59%) received TCTH from unrelated donors. Most patients (88%) developed mucositis while 7 pts (41%) developed acute or chronic graft-versus-host disease (GVHD). Prior to HSCT, 6 patients (35%) had moderate to severe malnutrition, 10 (59%) presented short stature, 2 (12%) had low BMD for age and 11 (65%) had low vitamin D serum levels. The energy consumption was inadequate in 6 (35%) patients and no patient had inadequate protein intake. At 180 days post-HSCT, six (35%) patients had inadequate caloric intake, 1 (5%) patient had inadequate protein intake and 13 patients (76%) had low vitamin D serum levels. A severe weight loss (greater than 5%) occurred in 6 (35%) pts, the LM decreased in 8 (47%) patients and 10 (69%) had a reduction in BMD. The prevalence of weight, LM and BMD reduction was high after six months of HSCT. The vitamin D serum levels were low before and after HSCT. Pre-transplant nutritional care and nutritional status monitoring of these patients are essential to ensure proper growth and development, reducing at the most as possible the nutritional deficit during treatment." @default.
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• W1965190302 date "2010-02-01" @default.
• W1965190302 modified "2023-09-27" @default.
• W1965190302 title "Hematopoietic Stem Cell Transplantation In Patients With Fanconi Anemia: What Are The Nutritional Consenquences?" @default.
• W1965190302 doi "https://doi.org/10.1016/j.bbmt.2009.12.291" @default.
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