title: Do patients with osteogenesis imperfecta need individualized nutritional support?
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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 D(3) were also evaluated.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Nutrition, School of Public Health, University of São Paulo, São Paulo, São Paulo, Brazil. cchagas@usp.br
authors.forename: Carlos Eduardo Andrade
authors.initials: CE
authors.lastname: Chagas
authors.forename: Janaína Pivetta
authors.initials: JP
authors.lastname: Roque
authors.forename: Bárbara
authors.initials: B
authors.lastname: Santarosa Emo Peters
authors.forename: Marise
authors.initials: M
authors.lastname: Lazaretti-Castro
authors.forename: Lígia Araújo
authors.initials: LA
authors.lastname: Martini
mesh_terms: D000273:Adipose Tissue; D000293:Adolescent; D000328:Adult; D001823:Body Composition; D001825:Body Fluid Compartments; D015992:Body Mass Index; D015519:Bone Density; D002118:Calcium / Q000008:administration & dosage / Q000097:blood / Q000172:deficiency; D016022:Case-Control Studies; D019587:Dietary Supplements; D005260:Female; D050723:Fractures, Bone / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D008297:Male; D009751:Nutritional Requirements; D009752:Nutritional Status; D018529:Nutritional Support; D009765:Obesity / Q000097:blood / Q000150:complications*; D010013:Osteogenesis Imperfecta / Q000097:blood / Q000150:complications / Q000628:therapy*; D015995:Prevalence; D014807:Vitamin D / Q000008:administration & dosage / Q000097:blood; D014808:Vitamin D Deficiency / Q000453:epidemiology; D055815:Young Adult
chemical_list: D014807:Vitamin D; D002118:Calcium