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.
The diagnosis of Behçet disease is based upon clinical criteria because of the lack of pathognomonic laboratory findings. Recurrent episodes of oral and genital ulcerations, skin lesions, and ocular manifestations are seen. The disease may also involve the central nervous system, gastrointestinal tract and, less frequently, the large vessels. In general, manifestations occur in the third or fourth decade of life and are not common in children. Therefore few data concerning this age group have been found in the literature. In this study we report a child with Behçet disease beginning at 1 year of age whose cutaneous manifestations were exuberant acne-like and folliculitis-like lesions, which were crucial for diagnostic confirmation.
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