Reduced bone mineral content and density in neurofibromatosis type 1 and its association with nutrient intake

• Autor(a) principal: Márcio Leandro Ribeiro de Souza
• Data de Publicação: 2020
• Outros Autores: Ann Kristine Jansen, Luiz Oswaldo c Rodrigues, Darlene Vilela, Adriana Kakehasi, Aline Stangherlin Martins, Juliana Ferreira de Souza, Nilton Alves Resende
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UFMG
• Texto Completo: http://hdl.handle.net/1843/60542
• topic: neurofibromatosis type 1; Bone; Nutrient; Food intake; Nutrition; Neurofibromatosis 1; Eating; Nutritional Requirements; Nutrients

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease characterized by multisystem involvement including low bone mineral density (BMD).OBJECTIVE: To assess the bone phenotype of individuals with NF1 and verify its associationwith nutrient intake.METHODS: Twenty-six adults with NF1 underwent bone phenotype assessments using dual-energy X-ray absorptiometry (DXA) andfood intake evaluations. They were compared to 26 unaffected matched control patients. Weight, height, and waist circumference(WC) were measured. DXA provided total body, spine, and hip BMDs and bone mineral content (BMC) for all patients. Food intake was evaluated for energy, macro- and micro-nutrients.RESULTS: Height (1.68 ± 0.1; 1.61 ± 0.1 cm; P = 0.003) and BMC (2.3 ± 0.4; 2.0 ± 0.5 kg; P = 0.046) were lower in the NF1 group. Individuals with NF1 also presented lower total body and spine BMDs (g/cm2) (1.1 ± 0.1, 1.0 ± 0.1, P = 0.036; 1.0 ± 0.1, 0.9 ± 0.1; P = 0.015,respectively). The frequency of total body bone mass below the expected level for patients’ ages was higher in the NF1 group (7.7%;34.6%, P = 0.016). There were no differences in energy consumption. No correlations between BMC and BMD with nutrient intake were observed in the NF1 group.CONCLUSIONS: The NF1 group presented lower BMCs and BMDs. Although a lower consumption of calcium, iron, and vitamin A, and a higher intake of sodium and omega-6 were observed, there was no relationship between bone phenotype and nutrient intake.