Prospective evaluation of growth, nutritional status, and body composition in children with cystic fibrosis

Background/Aims: Children with cystic fibrosis (CF) are of increased risk of reduced fat body mass (FBM) and lean body mass (LBM). Serum concentrations of insulin-like growth factor-1 (IGF-1)and leptin could be markers of LBM and/or FBM depletion. To evaluate the relationships between disease activity, body composition, IGF-1and leptin concentrations in CF children. Methods: A cross-sectional study with 26 CF children aged 5.0–15.5 years and 33 healthy controls, mean age 9.4 years. Body composition was evaluated by dual-energy X-ray absorptiometry. Fasting blood samples were analyzed for leptin, IGF-1and IGFBP-3. Results: FBM standard deviation score (SDS; CF boys –0.02 ± 0.88 vs. 0.78 ± 0.65, p < 0.01; CF girls –0.37 ± 1.15 vs. 0.70 ± 0.97, p < 0.05), leptin concentration (CF boys 2.07 ± 0.79 vs. 3.07 ± 1.28 ng/ml, p < 0.05; CF girls 2.71 ± 0.86 vs. 5.00 ± 2.95 ng/ml, p < 0.05) and IGF-1SDS (CF boys –1.43 ± 1.50 vs. –0.32 ± 0.88, p < 0.05; CF girls –0.66 ± 1.66 vs. 0.64 ± 0.57, p < 0.01) were lower in CF children compared to controls. Shwachman score was the strongest predictor of lean body mass (R = 0.63). Leptin levels explain 60% of the variability in FBM. Conclusion: Serum concentrations of IGF-1 and leptin are decreased in children with CF and are associated with clinical conditions and body composition.

show abstract

“…Growth impairment is still common in CF children [3, 21, 22]. In our study, the CF children were shorter and lighter than the control group.…”

Section: Discussionmentioning

confidence: 42%

Insulin-Like Growth Factor-1, Leptin, Body Composition, and Clinical Status Interactions in Children with Cystic Fibrosis

et al. 2007

View full text Add to dashboard Cite

show abstract

“…13 In contrast, the growth of boys with CF was impaired on the basis of height, fat free mass, and fat mass when observed longitudinally. 14 In the study by Corey et al, male patients had milder disease than females, as reflected by a smaller decline in FEV 1 , decreased mortality, and higher survival. 22 No significant differences between males and females have been found in other studies.…”

Section: Discussionmentioning

confidence: 95%

Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project

Steinkamp

Wiedemann²

2002

Thorax

272

183

View full text Add to dashboard Cite

Background: The German cystic fibrosis (CF) quality assurance (CFQA) project is a patient registry for CF which was founded in 1995. Relevant clinical and laboratory data, respiratory function test results, complications, and CF treatments are entered into the database once a year for each patient. Using the database, a study was undertaken to elucidate the relationship between nutrition and lung function in a large patient cohort by cross sectional and longitudinal analysis. Methods: A cohort of 3298 patients above 2 years of age was analysed. Patients were grouped according to the presence or absence of malnutrition (wasting and/or stunting). Cross sectional and longitudinal analyses over 2 and 3 years including mixed model analyses were performed. Results: The prevalence of abnormal weight for height (<90% predicted) increased with age from 19% in children aged <6 years to 38% in adults with CF. Patients with malnutrition had significantly lower mean values of vital capacity, arterial oxygen tension (PO 2 ), and forced expiratory volume in 1 second (FEV 1 ) and higher serum IgG (p<0.05). Pseudomonas aeruginosa infection was also associated with decreased pulmonary function. Malnourished adolescents aged 12-18 years experienced a serious decline in FEV 1 of about 20% predicted, whereas mean FEV 1 values remained stable at above 80% predicted in adolescents of normal weight. Longitudinal follow up showed that malnourished patients of all ages and those with P aeruginosa infection had significantly worse lung function than their normally nourished counterparts and a greater yearly loss of FEV 1 % predicted. During 1 year of observation adolescents who experienced a >5% predicted decrease in weight for height had a concomitant mean loss of FEV 1 of 16.5% predicted during that year, whereas patients who gained relative weight had a parallel increase in FEV 1 of 2.1% predicted. Conclusions: These data emphasise the close relationship between nutrition, lung function, and clinical course in CF. Normal body weight and absence of P aeruginosa infection was associated with better preservation of lung function.

show abstract

“…Longitudinal studies of BMD from childhood to adulthood may help to identify whether the sex bias in survival, or other factors, such as greater nutritional imbalances in males, are responsible for the observed sex differences in BMD in adults with CF. There is evidence suggesting that males with CF experience greater nutritional deficits than do females [27][28][29].…”

Section: Discussionmentioning

confidence: 99%

Reduced bone density in cystic fibrosis: ΔF508 mutation is an independent risk factor

et al. 2005

View full text Add to dashboard Cite

The aim of this cross-sectional study was to determine the prevalence and identify determinants of reduced bone mineral density (BMD) in adults with cystic fibrosis (CF).Adults (88) with CF (mean¡SD age 29.9¡7.7 yrs; forced expiratory volume in one second (FEV1) 58.2¡21.5% of the predicted value) were studied. BMD at the lumbar spine (LS) and femoral neck (FN) and body composition were measured using dual-energy X-ray absorptiometry. Blood and urine were analysed for hormones, bone turnover markers, and the cytokines tumour necrosis factor-a, and interleukin-6 and -1b. FEV1 (% pred); CF genotype; malnutrition; history of growth, development or weight gain delays; and corticosteroid use were analysed.BMD Z-scores were -0.58¡1.30 (mean¡SD) at the LS and -0.24¡1.19 at the FN. Z-scores of ,-2.0 were found in 17% of subjects. Subjects who were homozygous or heterozygous for the DF508 mutation exhibited significantly lower Z-scores than those with no DF508 allele. Multiple linear regression showed that the DF508 genotype and male sex were independently associated with lower BMD at both sites. Other factors also independently associated with lower BMD included malnutrition, lower 25-hydroxyvitamin D level, lower fat-free mass and lower FEV1 (% pred).In conclusion, reduced bone mineral density in cystic fibrosis is associated with a number of factors, including DF508 genotype, male sex, greater lung disease severity and malnutrition.

show abstract

Prospective evaluation of growth, nutritional status, and body composition in children with cystic fibrosis

Abstract: Despite comprehensive care, the growth of boys with CF was impaired on the basis of height, fat-free mass, and fat mass, when observed longitudinally. Caution should be used when interpreting cross-sectional measurements because they often do not detect suboptimal growth.

Cited by 47 publications

References 32 publications

Insulin-Like Growth Factor-1, Leptin, Body Composition, and Clinical Status Interactions in Children with Cystic Fibrosis

Insulin-Like Growth Factor-1, Leptin, Body Composition, and Clinical Status Interactions in Children with Cystic Fibrosis

Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project

Reduced bone density in cystic fibrosis: ΔF508 mutation is an independent risk factor

Contact Info

Product

Resources

About