Do patients with osteogenesis imperfecta need individualized nutritional support?

• Published in: Nutrition (Burbank, Los Angeles County, Calif.) Vol. 28; no. 2; pp. 138 - 142
• Main Authors: Chagas, Carlos Eduardo Andrade, Roque, Janaína Pivetta, Santarosa Emo Peters, Bárbara, Lazaretti-Castro, Marise, Martini, Lígia Araújo
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.02.2012; Elsevier; Elsevier Limited
• Subjects: Adipose Tissue; administration & dosage; Adolescent; Adult; Biological and medical sciences; blood; Body Composition; body fat; Body Fluid Compartments; Body Mass Index; Bone Density; bone fractures; Bone mineral density; Bones; Calcium; Calcium - administration & dosage; Calcium - blood; Calcium - deficiency; Case-Control Studies; Colleges & universities; complications; correlation; Dairy products; deficiency; Dietary Supplements; Disease; dual-energy X-ray absorptiometry; epidemiology; etiology; Feeding. Feeding behavior; Female; Food; food records; Fractures, Bone; Fractures, Bone - etiology; Fractures, Bone - prevention & control; Fundamental and applied biological sciences. Psychology; Gastroenterology and Hepatology; Gastrointestinal diseases; Humans; lean body mass; lumbar spine; Male; nutrition information; Nutritional Requirements; Nutritional Status; Nutritional Support; Obesity; Obesity - blood; Obesity - complications; Osteogenesis Imperfecta; Osteogenesis Imperfecta - blood; Osteogenesis Imperfecta - complications; Osteogenesis Imperfecta - therapy; parathyroid hormone; patients; physical therapy; Prevalence; prevention & control; Proteins; Risk factors; Scoliosis; serum albumin; Spine; therapy; Vertebrates: anatomy and physiology, studies on body, several organs or systems; Vitamin D; Vitamin D - administration & dosage; Vitamin D - blood; Vitamin D Deficiency; Vitamin D Deficiency - epidemiology; Young Adult; Osteogenesis imperfecta; Nutritional status; Body composition; Human; Vertebrata; Need; Mammalia; Diseases of the osteoarticular system; Osteochondrodysplasia; Genetic disease; Food supplement
• ISSN: 0899-9007; 1873-1244; 1873-1244
• DOI: 10.1016/j.nut.2011.04.003

Information regarding nutrition and body composition in patients diagnosed with osteogenesis imperfecta (OI) is scarce. In the present study, nutritional status, bone mineral density, and biochemical parameters of subjects with OI were evaluated. Patients with type I OI (n = 13) and type III OI (n = 13) and healthy controls (n = 8) were selected. Nutritional status and bone mineral density were assessed by a 3-d food diary and dual-energy X-ray absorptiometry at the lumbar spine, respectively. Body mass index, serum albumin, calcium, creatinine, cross-linked C-telopeptide, parathyroid hormone, and 25-hydroxivitamin D3 were also evaluated. Patients with OI had lower bone mineral density (P < 0.05 versus controls). Patients with type III OI had the highest body mass index (P < 0.05 versus patients with type I OI and controls) and the lowest lean body mass (P < 0.05 versus patients with type I OI and controls). In patients with OI, the number of fractures was positively correlated with body mass index (r = 0.581, P = 0.002) and the percentage of body fat (r = 0.451, P = 0.027) and negatively correlated to lean body mass (r = −0.523, P = 0.009). Even when taking dietary supplements, 58% and 12% of subjects with OI did not achieve the calcium and vitamin D recommendations, respectively. Body composition is a risk factor for bone fractures in subjects with OI. Individualized nutritional support is recommended not only to improve body composition but also to potentiate pharmacologic and physical therapies.