Article

Bone mineral density in Australian children, adolescents and adults with cystic fibrosis: a controlled cross sectional study.

Department of Respiratory Medicine, Royal Children's Hospital, Herston, QLD 4029, Australia.
Thorax (Impact Factor: 8.56). 03/2004; 59(2):149-55.
Source: PubMed

ABSTRACT Low bone mineral density (BMD) is recognised in individuals with cystic fibrosis (CF) although the pathogenesis remains unclear. The aims of this study were to compare BMD over a broad continuum of Australian individuals with CF with healthy controls and to examine the relationship between BMD and clinical parameters including physical activity, nutrition, and vitamin D levels.
BMD of the lumbar spine (LS), total body (TB), femoral neck (FN), cortical wrist (R33%), and distal wrist (RUD) was examined in 153 individuals with CF aged 5.3-55.8 years (84 males) and in 149 local controls aged 5.6-48.3 years (66 males) using dual energy x ray absorptiometry. Anthropometric variables, body cell mass, markers of disease severity, corticosteroid usage, measures of physical activity, dietary calcium and caloric intake and serum vitamin D were assessed and related to BMD.
Compared with controls, mean BMD was not significantly different in children aged 5-10 years with CF. Adolescents (females 11-18 years, males 11-20 years) had reduced TB and R33% BMD when adjusted for age, sex, and height (difference in BMD (g/cm2) adjusted means between control and CF: TB=0.04 (95% CI 0.01 to 0.07); R33%=0.03 (95% CI 0.01 to 0.06)). BMD was reduced at all sites except R33% in adults (difference in BMD (g/cm2) adjusted means between control and CF: TB=0.05 (95% CI 0.02 to 0.09); LS=0.08 (95% CI 0.03 to 0.14); FN=0.09 (95% CI 0.03 to 0.15); RUD=0.03 (95% CI 0.01 to 0.05)). In children/adolescents BMD was weakly associated with nutritional status and disease severity.
BMD was normal in a well nourished group of prepubertal children with CF. A BMD deficit appears to evolve during adolescence and becomes more marked in adults. Individuals with CF should optimise nutrition, partake in physical activity, and maximise lung health in order to optimise BMD. Further longitudinal studies are required to understand the evolution of reduced BMD in young people and adults with CF.

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    • "One can imagine that a vertebral or rib fracture would make it difficult to maintain a regimen of airway clearance that is needed for the prevention of CF exacerbations and the maintenance of lung function. Although most of the pathologic consequences occur during adulthood in the CF population, there have been several studies that indicate that during childhood and puberty the CF patients achieve approximately half of the bone density than their healthy counterparts [18] [19] [20] [21] [22]. Puberty is especially important for the development of bone density and is a time where there is both peak growth velocity and bone density accrual. "
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    ABSTRACT: Cystic fibrosis (CF) is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopausal population, bone disease in CF begins at a young age and is associated with significant morbidity due to fractures, kyphosis, increased pain, and decreased lung function. The maintenance of bone health is essential for the CF population during their lives to prevent pain and fractures but also as they approach lung transplantation since severe bone disease can lead to exclusion from lung transplantation. Early recognition, prevention, and treatment are key to maintaining optimal bone health in CF patients and often require a multidisciplinary approach. This article will review the pathophysiology, current clinical practice guidelines, and potential future therapies for treating CF-related bone disease.
    12/2010; 2011:926045. DOI:10.4061/2011/926045
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    • "However, this expansion of life span has brought about additional clinical complications of osteoporosis and musculoskeletal problems. Research since 1979 has established that patients with CF are at a significantly greater risk for developing osteoporosis, thoracic kyphosis and pathological fractures, decades earlier than expected [1] [2] [3] [4] [5] [6] [7] [8] [9] [10].Osteoporosis, occurring in between 50 and 70% of patients with CF [10], can also be a factor for exclusion from lung transplantation operations that may be life saving in the later stages of the disease [10] [11]. The development of osteoporosis in patients with CF begins early in life, due to inadequate bone mineral accrual [1,12–15], from impeded bone calcium accretion and an uncoupling in the normal balance between bone formation and bone resorption, leading to a net loss of bone [17]. "
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    ABSTRACT: Inadequate bone mineral accrual during growth and accelerated bone demineralisation in adulthood are recognised as additional and serious complications for patients with cystic fibrosis (CF). However, little attention has been given to preventative strategies for this population. Inadequate bone accrual during childhood and adolescence, and premature bone loss, lead to a failure to attain an optimal peak bone mass, osteoporosis and fracture in patients with CF. Pharmaceutical treatments may assist in the improvement of bone in patients with CF, but these are usually not preferable for use in children. Evidence indicates that regular, weight-bearing exercise significantly enhances bone accrual in healthy children. This paper reviews the literature concerning the potential for weight-bearing exercise to improve bone mineral accrual in children with CF. All relevant literature since 1979 was obtained and reviewed from the Medline, PubMed, Cochrane and PEDro data base. Evidence concerning the efficacy of exercise for bone health in CF is lacking. There have been no controlled trials investigating the value of weight-bearing exercise for bone accrual in children with CF. As exercise may offer an effective and enjoyable strategy to improve the bone development in children who have CF, exercise should be a high priority for randomised controlled trials in this population.
    Journal of Cystic Fibrosis 08/2008; 7(4):270-6. DOI:10.1016/j.jcf.2008.02.001 · 3.82 Impact Factor
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    • "The study sample consisted of children diagnosed with CF over the age of 5 years, who were recruited from the Royal Children's Hospital, Brisbane, Australia. The children were a part of a longitudinal study concerned with bone mineral density and body composition (Buntain et al. 2004). Only those who completed longitudinal body composition measurements are reported here. "
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    ABSTRACT: The importance of nutritional intervention for children with cystic fibrosis (CF) is well recognised. It would be expected that the increase in knowledge over the past decade would be reflected in improvements in nutritional status for the CF paediatric population. The aim of the present paper was to evaluate the nutritional status of children with CF, cross-sectionally and longitudinally. Body cell mass adjusted for gender and size (BCM/Htp) was measured in sixty-four children with CF to represent nutritional status and expressed as a Z-score. The cross-sectional results showed a mean BCM/Htp Z-score of 0.54 (sd 1.21), with males having a slightly higher Z-score than females but with a larger variation. At the initial measurement, only one female and one male were considered sub-optimally nourished. The longitudinal analysis after 2 years showed that the mean population had a significantly decreased BCM/Htp Z-score; however, when each gender was analysed separately, this decrease was significant only in the males. At the final measurement, only two females and three males were considered sub-optimally nourished. It is evident from our results that children with CF are well nourished, with only a small percentage considered malnourished. It appears that nutritional status decreases with age, with this decline being more evident in males. These results signify that although children with CF are better nourished with current treatment support, intervention needs to continue throughout a CF patient's life to counteract the changes that occur with age.
    British Journal Of Nutrition 03/2006; 95(2):321-4. DOI:10.1079/BJN20051611 · 3.34 Impact Factor
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