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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
Among possible causes of chronic hepatitis in adolescents most common are infections, autoimmune disorders and metabolic diseases. Thus, diagnostic procedures should be multidirectional. This study reports diagnosis and treatment difficulties in an 18-year-old male patient with hereditary hemochromatosis (HH), ulcerative colitis (UC), chronic hepatitis B (CHB) and Gilbert syndrome. The presented case illustrates problems in diagnostics related to the presence of numerous disease conditions in one patient. It should be taken into consideration that these diseases coexisting in one patient can mutually affect their symptoms creating specific diagnostic difficulties.
EN
Objective: To determine transforming growth factor (TGF) β1, interleukin (IL) 4, and IL-10 concentrations in human milk and to assess the relationship between allergic disorders in mothers and the content of the interleukins in their milk. Material and methods: Thirty allergic and 46 healthy mothers were included in the study. Colostrum was collected 2-3 days after delivery. Cytokine concentrations were determined with commercial enzyme-linked immunosorbent systems. Results: TGF-β1was found in milk from 23 women in the control group (53.49%) and 11 in the allergy group (37.93%). When TGF-β1 was present, the median concentration was higher in the allergy group than in the control (61.5 and 30.4 pg/mL, respectively; P < 0.004). IL-10 was present in the colostrum of all the women and the median IL-10 concentration did not differ between the allergy (50.5 pg/mL) and control (51.5 pg/mL) groups. The probability of occurrence of a positive IL-4 value in the allergy group was greater than in the control group (chi-squared [df=1] = 2.60, P < 0.053). Median IL-4 level did not differ significantly between the two groups (0.5 and 0.5 pg/mL respectively). Conclusions: TGF-β1 was detected less often in the colostrum of allergic mothers than in that of mothers without allergy (but the difference was not statistically significant). IL-4 was found more often in the colostrum of allergic mothers than nonallergic ones. The allergy status did not correlate with IL-10 concentration.
EN
Background: Juvenile idiopathic arthritis (JIA) is a chronic, heterogenous inflammatory disease of unclear pathogenesis. JIA is hypothesized to be linked to a defective immune regulation. Anti-inflammatory cytokines belong to the best known regulatory factors. T-regulatory cells are a crucial cellular component of immune tolerance. One of their functions is synthesis of interleukin 10 (IL-10) and transforming growth factor beta1 (TGF-β1). The aim of this study was to determine the proportion of T-regulatory cells (CD4+CD25highFOXP3+) in peripheral blood, and serum levels of TGF-β1 and IL-10 in patients with JIA. Methods: The study included 25 patients with newly diagnosed JIA: oligoarthritis (n=17) and polyarthritis (n=8). The control group was comprised of 17 healthy children. CD4+CD25highFOXP3+ T cells in peripheral blood were quantified by means of three-color flow cytometry. Serum concentrations of TGF-β1 and IL-10 were estimated with ELISA. Results: The proportion of peripheral CD4+CD25highFOXP3+ cells in patients with JIA was significantly higher than in the controls (p=0.04). The two groups did not differ significantly in terms of their TGF-β1 and IL-10 concentrations. Conclusions: At the time of diagnosis, children with JIA presented with an elevated proportion of T-regulatory cells (CD4+CD25highFOXP3+) in peripheral blood. Anti-inflammatory cytokines, IL-10 and TGF-β1, are not upregulated in the serum of patients with JIA, and therefore should not be considered as biomarkers of this condition.
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