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Type:	Artigo de Periódico
Title:	Reduced bone mineral content and density in neurofibromatosis type 1 and its association with nutrient intake
Authors:	Márcio Leandro Ribeiro de Souza Ann Kristine Jansen Luiz Oswaldo c Rodrigues Darlene Vilela Adriana Kakehasi Aline Stangherlin Martins Juliana Ferreira de Souza Nilton Alves Resende
Abstract:	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.
Subject:	Neurofibromatosis 1 Eating Nutritional Requirements Nutrients
language:	eng
metadata.dc.publisher.country:	Brasil
Publisher:	Universidade Federal de Minas Gerais
Publisher Initials:	UFMG
metadata.dc.publisher.department:	ENF - DEPARTAMENTO DE ENFERMAGEM APLICADA ENF - DEPARTAMENTO DE NUTRIÇÃO MED - DEPARTAMENTO DE APARELHO LOCOMOTOR MED - DEPARTAMENTO DE CLÍNICA MÉDICA
Rights:	Acesso Aberto
metadata.dc.identifier.doi:	10.1590/1806-9282.66.5.666
URI:	http://hdl.handle.net/1843/60542
Issue Date:	2020
metadata.dc.url.externa:	http://dx.doi.org/10.1590/1806-9282.66.5.666
metadata.dc.relation.ispartof:	Revista da Associação Médica Brasileira
Appears in Collections:	Artigo de Periódico

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