Elsevier

Nutrition

Volume 28, Issue 2, February 2012, Pages 138-142
Nutrition

Applied nutritional investigation
Do patients with osteogenesis imperfecta need individualized nutritional support?

https://doi.org/10.1016/j.nut.2011.04.003Get rights and content

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 D3 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.

Introduction

Osteogenesis Imperfecta (OI) is a genetic disease that leads to bone fragility and decreased bone mass not secondary to any other clinical condition [1]. According to their characteristics, patients with OI can be classified in seven different subgroups [2]. Type I, the less severe form, is associated with normal or very minor height impairment, some bone fractures, and moderate scoliosis. Type III, a more severe form, is associated with a high prevalence of bone fractures and abnormalities, severe stature impairment, and scoliosis. Although pharmacologic therapy with bisphosphonates is encouraged [3], patients with OI still have increased bone pain and fractures.

An adequate dietary intake is required to increase bone health and to decrease bone fractures throughout life [4]. Given the importance of improving bone development and function in subjects with OI, nutrients related to bone health, such as calcium, vitamin D, and protein, may be relevant during the treatment of this disease. Body composition also plays an important role in bone physiology, and fat mass and lean body mass (LBM) seem to be involved [5]. However, surprisingly, there is no information regarding nutritional status, dietary intake, and body composition of patients with OI.

The aim of the present study is to evaluate the nutritional status and body composition of subjects diagnosed with OI.

Section snippets

Subjects

This was a cross-sectional study that included 26 men and women diagnosed with OI (13 diagnosed with type I and 13 diagnosed with type III) according to previously described criteria [2]. All patients were under ambulatory treatment in the Ambulatory of Bone Fragilities at the Department of Medicine, Federal University of São Paulo. All patients were using the bisphosphonate pamidronate. From the time of OI diagnosis until the time that subjects with OI were enrolled in the study, the number of

Anthropometry and body composition

Anthropometry and body composition measurements of controls and patients with OI are presented in Table 1. The difference between the height and length observed in patients with type III OI was related to the severity of the disease. Only four patients with type III were able to stand in the upright position. Therefore, because of the high prevalence of bone fractures and the severe stature impairment and scoliosis, the other nine patients with OI had only the body length measured and not

Discussion

The results of the present study highlighted body composition as an important risk factor for bone fracture in patients diagnosed with OI. A possible limitation of the present study is the small number of patients with OI evaluated. However, to the best of our knowledge, this is the first study in which a nutritional evaluation was performed in subjects with OI. An important observation regarding a modifiable risk factor, i.e., improvement in diet and body composition, was demonstrated.

Body

Conclusion

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.

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      We also highlight that performance of universally accepted definitions of obesity and slimness do not perform well in this population and we propose new optimal cut-offs. Low lean mass has previously been reported as a risk factor for fracture in children with OI (18). Both OI types in our cohort had significantly lower LMI compared to a healthy age and sex matched control population.

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      Clinically, OI's skeletal involvement ranges from a mild predisposition to fractures to severe deformities or even perinatal death [3]. In addition to bone phenotype, it has been reported that primarily children and adolescent patients with OI display low muscle mass and function [4–6], normal or increased fat mass [7–9], and are in a form of “hypermetabolic” state [10–12]. In a previous study using a mouse model mimicking dominant moderate-to-severe OI, the Col1a1Jrt/+ mouse model, we found a distinctive sex-dependent metabolic phenotype with increased insulin levels in males, improved glucose tolerance in females, lower levels of random glucose and low adiposity in both sexes, and increased energy expenditure [5,6,13].

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    The present study was supported by FAPESP (grant 08/51095-3). J. P. R. was supported by a fellowship from FAPESP.

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