Introduction: Cystic fibrosis (CF) is inherited, metabolic, multisystem disease with various clinical symptoms. In the airways neutrophilic inflammation and increased levels of neutrophil elastase (NE) are observed even in young children. The method of electrophoretic mobility of cell nuclei (EMN) is a way of assessment cell maturity. Observed distorted nuclei mobility is mostly dependent on chromatin distribution and remodeling. Purpose: To evaluate the EMN index in buccal cells collected from CF patients. Materials and methods: The study was conducted in the group of 15 CF patients aged 5-26 years. Results: In the healthy subjects we have observed that the lowest EMN index values were reach around 2 year of life (7.6 ± 3.1) and in the late senescence (8.5 ± 2.5) with the peak values around 16-21 years (61.4 ± 2.5). The results differed significantly from the healthy controls. Conclusion: EMN index in CF buccal cells may be a simple method to quickly assess the chromatin remodeling.
Purpose: Cystic fibrosis (CF) is a genetic, metabolic disease. Long-term therapy often leads to inappropriate calcification of bones. Dual X-ray absorptiometry (DXA) is considered a „gold-standard” for bone mineral density (BMD) assessment, but high usage costs can limit its availability. This paper compares two methods for BMD assessment in CF patients: hand radiograms method and densitometry using DXA method. Materials and methods: The study was performed in a group of 26 CF patients (10F, 16M), aged 7-30 years. In all cases, DXA measurements were performed, along with bone mass assessment using DENSY2004 system for digital assessment of hand radiograms. Stepwise binary logistic regression was used to examine the contribution of bone age, BMI, Cole's index and hand radiograms parameters to low BMD expressed as Z-score ≤-1SD. Results: Statistical analysis of the gathered data revealed that hand radiograms method allow for estimation of Z-score below -1SD with accuracy of 84.62% comparing to DXA. Sensitivity and specificity of this estimation in the studied group of CF patients was 86.67% and 81.82%. Conclusions: Hand radiograms method has good accuracy, sensitivity and specificity; therefore, it can be an alternative for DXA in BMD assessment. It can be implemented in chronic diseases affecting BMD like cystic fibrosis.
Purpose: Cystic fibrosis (CF) is inherited, congenital disease of multi-organ expression. Carnitine play a role as a lipid acid transporter to mitochondrium for beta-oxydation. Acylation of carnitine is inevitable for detoxication processes in cells. Low lean body mass In CF patients can lead to decreased levels. The aim of the study was the evaluation of free carnitine, acylcarnitine and acylcarnitine/free carnitine ratio in serum and urine of children with cystic fibrosis. Material and methods: The study was conducted in a group of 15 CF children (4 F, 11 M), aged 12.6 ±5.4 years. The serum for a control group was collected from 32 healthy children. Urine samples for control group was collected from 62 health children. Free carnitine and total carnitine was assessed using spectrophotomeric method in which acyl group is transferred from acetyl-CoA to carnitine by carnitine acetyltransferase (CAT). Acyl carnitine concentration and acylcarnitine/free carnitine ratio was counted using Schmidt- Sommerfeld and Seccombe equation. Results: In 12 CF patients (80%) free carnitine and total carnitine was below lower limit of normal (p<0.001). In 9 patients (60%) free carnitine level was ≤ 20 µmol/L, which can be clinically diagnosed. Acylcarnitine levels were also statistically lower in CF group (p<0.01). Acylcarinitie/free carnitine ratio did not differ between the groups (p=0.05). Urine excretion of free carnitine, total carnitine and acylcarnitine was lower in CF group (p<0.001). Conclusions: In CF pediatric patients statistically significant lower levels of free carnitine, total carnitine and acylcarnitine were observed in comparison to controls. Low urine excretion of free carnitine, total carnitine and acylcarnitine was lower in CF group. No correlation between serum and urine levels of free carnitine, total carnitine and acylcarnitine were observed.
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