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Background Cystic fibrosis(CF),despite much progress in therapy, remains the disease which affects nutrition. Nutrition is an important prognostic factor of the outcome of the disease. We want to evaluate physical development, nutrition and body composition in CF children. Material and methods 75 children diagnosed with CF (9 months to 18 years old) were included into the study. 33 healthy children (9 months to 18 years old) constituted the control group. The study consisted of 2 stages. In the first the differences between groups were investigated. The second, took place a year later. At each time point the following measurements were performed: height, body mass, skin fold, arm circumference; BMI, FFM%, FM% and Frisancho index. FFM(fat free mass), FM(fat mass), muscle mass, TBW(total body water) were evaluated by mans of BIA(bioimpedance). Results CF children were shorter than healthy children. Stunting affected 18,67% of CF patients at first examination and 21,6% a year later. Underweight was diagnosed in 28% of patients at the beginning and in 41.2% a year after. Underweight was the result of both little FM and scarce muscle mass. Conclusions Many children with cystic fibrosis suffers from short stature and underweight, which progresses within time. FFM decreases with the disease progress
EN
Background: Cystic fibrosis(CF),despite much progress in therapy, remains the disease which affects nutrition. Nutrition is an important prognostic factor of the outcome of the disease. We want to evaluate physical development, nutrition and body composition in CF children. Material and methods: 75 children diagnosed with CF (9 months to 18 years old) were included into the study. 33 healthy children (9 months to 18 years old) constituted the control group. The study consisted of 2 stages. In the first the differences between groups were investigated. The second, took place a year later. At each time point the following measurements were performed: height, body mass, skin fold, arm circumference; BMI, FFM%, FM% and Frisancho index. FFM(fat free mass), FM(fat mass), muscle mass, TBW(total body water) were evaluated by mans of BIA(bioimpedance). Results: CF children were shorter than healthy children. Stunting affected 18,67% of CF patients at first examination and 21,6% a year later. Underweight was diagnosed in 28% of patients at the beginning and in 41.2% a year after. Underweight was the result of both little FM and scarce muscle mass. Conclusions: Many children with cystic fibrosis suffers from short stature and underweight, which progresses within time. FFM decreases with the disease progress
EN
The spinal muscular atrophy is a rare autosomal recessive genetic disease characterized by the progressive loss of muscular strength. In its natural course the disease leads to death. Diabetes mellitus type 1 is an autoimmune metabolic disorder characterized by the disturbed insulin synthesis. This is a case report of an 8-year-old girl suffering from Werdnig Hoffman disease in whom DM1 was diagnosed. The unspecific clinical manifestation and diagnostic difficulties are presented in this paper. To the authors' knowledge, this is the first publication concerning the co-existence of these two medical conditions.
EN
The aim of this study was to analyze the usefulness of fecal lactoferrin in the diagnosis and monitoring of inflammatory bowel disease (IBD) in children. The study included 52 children with IBD (24 with Crohn's disease and 28 with ulcerative colitis) aged between 0.92 and 18 years, and 41 IBD-free controls of similar age. Fecal concentration of lactoferrin was determined with a quantitative immunoenzymatic test. Fecal concentration of lactoferrin in children with IBD was significantly higher than in the controls. The cut-off value of fecal lactoferrin concentration optimally distinguishing between the children with IBD and the controls was identified as 13 μg/g. The sensitivity and specificity of this cut-off value equaled 80.7% and 92.7%, respectively, and its positive and negative prognostic values were 96.8% and 63.3%, respectively. Patients diagnosed with moderate Crohn's disease had significantly higher fecal concentrations of lactoferrin than children with the mild or inactive disease. Similarly, children with moderate ulcerative colitis showed significantly higher fecal concentrations of lactoferrin than individuals with the mild condition. No significant relationship was found between the fecal concentration of lactoferrin and the severity of endoscopic lesions. Patients with IBD and a positive result of fecal occult blood test were characterized by significantly higher concentrations of lactoferrin than the individuals with IBD and a negative result of this test. In conclusion, fecal concentration of lactoferrin seems to be a useful parameter for diagnosis and monitoring of IBD in children.
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