The Impact of Nutrition on Bone Growth in Osteogenesis Imperfecta

Data developed at the Kennedy Krieger Institute supports the previous observation that bone mass and growth rates are decreased in most OI children regardless of phenotype. Our experience indicates that feeding problems are not uncommon in OI children. Although nutritional factors are accepted as influencing both bone mass and growth, the impact of dietary deficiencies in OI children has not been investigated. 

To investigate the relationship of nutrition to bone mass and growth rates we propose the following Hypotheses: 1) The development of a nutritional database for OI children will reveal specific deficiencies in bone-related nutrients as compared to the Diet Reference Intake (DRI) standard; 2) Nutritional deficiencies will correlate to bone mass, extremity length and body height and weight. 

To test these hypotheses we propose the following: 1) Based on 24 hour recall telephone interviews, we will characterize the intake of specific bone-related nutrients for OI children 2 to 10 years of age; 2) Next, this data will be analyzed against serum biochemistry tests, serum bone biomarkers, DXA bone mineral content, extremity bone length and growth rates during 12 months of observation. 3) However, If diet analysis at 6 months reveals significant diet deficiencies ( < 66% DRI) for total calories, protein, vitamin D or calcium, or, serum vitamin D levels are lower than 32 ng/ml, the impact of supplementation on bone growth and linear growth over a subsequent 12 month period will be tested. 

This is a 3- year collaborative effort between the Kennedy Krieger Institute and the A.I. duPont Hospital for Children and Children's Hospital of Philadelphia (CHOP). A total of 50 children will be included in the study to permit 4 dropouts and approximately 10 who may be moved to the treatment cohort: It is estimated that 36 children will be included for statistical purposes. 20 at KKI, 10 at AI. duPont and 6 at CHOP. 

This database will provide the first longitudinal assessment of diet and its impact on bone and growth in OI children.