Original articleUse of body mass index percentile to identify fat-free mass depletion in children with cystic fibrosis☆
Introduction
Cystic fibrosis (CF) is one of the most common life-threatening inherited disorders in the Caucasian population. In the past decades, the median predicted survival of patients with CF has increased from 10–12 to over 37 years.1 One of the major factors contributing to this increased survival has been the understanding of the role of optimal nutrition on the overall health status of individuals with CF. Studies showed that in children with CF, better nutritional status was associated with improved linear growth, better pulmonary functions, and exercise tolerance,2, 3, 4 and interventions to establish weight gain led to improvements in pulmonary functions.5 Since there is a strong association between BMI and overall health metrics, especially lung health,6 the Cystic Fibrosis Foundation recommends that children with CF maintain a body mass index (BMI) ≥50th percentile. However, malnutrition is still prevalent in children with CF.7
BMI is currently the universally accepted method to determine malnutrition in children. However, low body weight and BMI do not differentiate between fat mass (FM) and fat-free mass (FFM), and weight-for-height measurements underestimated the prevalence of malnutrition defined after body composition measurement using total body potassium8 or skinfold thickness measurements.9 FFM depletion commonly occurs in adults with CF.10, 11, 12, 13, 14 Previous studies showed that while FFM was low, FM was maintained in 40% of these patients (hidden depletion of FFM),10, 11 and 54% of the patients with a normal body weight for height had low values for lean body mass.15 Apparent or hidden FFM depletion rather than low body weight in CF was associated with overall disease severity as indicated by decreased lung function, respiratory muscle weakness, increased systemic inflammatory activity, low bone mineral density, and increased frequency of exacerbations.10, 11, 15
Studies in pediatric subjects with CF16, 17, 18 found differences in body composition between young and post-pubertal children with CF. Limited studies are available examining the prevalence of (hidden) FFM depletion in pediatric subjects with CF. A study in 22 children with CF reported that 45% of the patients with FFM had z-scores below −2 and half of the normal-weight patients (% ideal weight for height >85%) had FFM z-scores below −2.19 Furthermore, it is unclear at what age, changes in tissue distribution occur in CF and whether FFM depletion in children with CF is associated with increased morbidity (lung function, bone loss, disease severity) as previously observed in adults with CF.10, 11, 12, 13, 14
The hypotheses for this cross-sectional study were that FFM measurement in children with CF is a better method to screen for malnutrition than the use of BMI percentiles alone, and that depletion of FFM is associated with increased morbidity and worsening of lung health. In this study, we examined a group of randomly selected children with CF to determine (1) the prevalence of underweight and (hidden) FFM depletion, (2) whether (hidden) FFM depletion was associated with changes in body composition on whole body and subregional (trunk and extremities) level, and increased morbidity (reduced lung function, loss of bone mineral density), and (3) whether specific BMI percentiles can be used to predict FFM depletion and increased morbidity.
Section snippets
Study population
Seventy-seven children, age 8–21 years, with CF who received care at Arkansas Children's Hospital CF Care Center from June 2002 through March 2010 were consecutively enrolled and studied retrospectively. Data were obtained from the electronic medical records of children presenting for routine clinical visits and before hospital discharge when they were admitted for an exacerbation. Approval from the Institutional Review Board of the University of Arkansas for Medical Sciences (IRB # 110166) and
Patients characteristics
The study group consisted of 77 children with CF (40 females, 37 males), with a mean age of 14.8 ± 2.9 years. Characteristics were as follows: 63% homozygote for ΔF508, 26% heterozygote for ΔF508, 11% gene combination without ΔF508, 97% pancreatic insufficient, 13% Cystic Fibrosis-related diabetes, 27% liver disease, 30% received nocturnal enteral nutrition, and 6% had a history of short bowel syndrome.
Discussion
In the present study, we found that FFM depletion in children with CF was poorly detected when using the 10% BMIp cut-off criteria defined by the CFF as nutritional failure, and the results support the CFF recommendations for a good nutritional status with a BMIp ≥50. Fifty-eight percent (58%) of the CF patients with FFM depletion had BMIp larger than 10% (hidden FFM depletion), indicating that a large portion of patients would have been missed when malnutrition was purely defined by these
Funding
The authors have no financial disclosure.
Statement of authorship
Each author has participated sufficiently, intellectually or practically, in the work to take public responsibility for the content of the article, including the concept, design, and conduction of the experiment and for data interpretation (authorship).
Conflict of interest
There is no conflict of interest to declare.
Acknowledgments
MPKJ Engelen was involved in data collection and analysis, and writing of the manuscript, R Schroder and K van der Hoorn were involved in data collection and analysis, NEP Deutz was involved in data analysis and reviewing of the manuscript, GC was involved in data collection and writing of the manuscript.
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Cited by (0)
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The work was performed at Arkansas Children's Hospital and University of Arkansas for Medical Sciences, Little Rock, AR, USA.