BackgroundThe aim of our study was to evaluate factors affecting cystic fibrosis (CF) patients’ health-related quality of life (HRQoL) and to assess the level of agreement on HRQol between children and their parents.MethodsFifty-nine patients (mean age: 14.03 ± 4.81 years) from 5 Hungarian CF centres completed the survey. HRQoL was measured using The Cystic Fibrosis Questionnaire-Revised (CFQ-R). Parents were asked to fill out a questionnaire about their smoking habits, educational level and history of chronic illness. Disease severity was assessed using the physician-reported Shwachman-Kulczycki (SK) score system. Spirometry, Body Mass Index (BMI) percentile (pc), hospitalisation and Pseudomonas aeruginosa (PA) infection were examined as physiologic parameters of CF, and the impact of these factors on HRQoL was assessed. A multivariate regression analysis was performed to identify the most important factors affecting HRQoL. The level of significance was set to 0.05.ResultsPassive smoking and parental educational level and chronic diseases status did not have a significant impact on the patients’ HRQoL (p > 0.05). Significantly lower SK scores and spirometry values were found in low BMI pc patients (p < 0.001), in hospitalised (p < 0.01) and in PA-infected patients (p < 0.01), than in the adequate-weight, non-hospitalised and PA culture-negative subgroup. Lower CFQ-R scores were detected in hospitalised patients than in non-hospitalised patients in their Physical functioning domain. PA-infected patients had HRQoL scores that were significantly worse in the Body image (p < 0.01) and Respiratory symptoms (p < 0.05) domains than the PA culture-negative patients. Patients with a low BMI pc (<25th BMI pc) had significantly lower scores in the Eating, Body image and Treatment burden domains, than the adequate-weight patients (>25th BMI pc) (p < 0.01). A strong child–parent agreement was found in the Physical functioning domain (r = 0.77, p < 0.01).ConclusionsPassive smoking, parental educational level and chronic diseases of parents do not affect the HRQoL of CF patients. In contrast, hospitalisation, PA infection and malnutrition have a significant and negative impact on patients’ HRQoL and the clinical severity of the disease. Parents and children were consistent in their scoring of symptoms and behaviours that were observable.
Bone deficit is present in patients with CF who have never received steroid treatment. Delay of puberty, chronic inflammation, or genetic susceptibility might be responsible for this phenomenon which was found in patients who had never received steroids and who were in relatively good clinical state.
Background: The aim of the study was to assess the antioxidant status in cystic fibrosis (CF) patients compared to healthy controls. In order to determine the influence of nutrition on the level of the antioxidants, nutrient intake was also monitored in both groups at the time of the antioxidant assessment. Subjects and Methods: The authors measured the serum malondialdehyde levels in children with CF, n = 21; 9 females and 12 males, mean age: 8.71 years (6–12 years) and compared these values to the levels found in age-matched healthy control subjects, n = 24; 13 females and 11 males, mean age: 8.33 years (6–12 years). In order to assess the antioxidant status, catalase and superoxide dismutase activities in washed erythrocytes, glutathione peroxidase activity of heparinized whole blood and serum ascorbic acid, α-tocopherol and retinol levels were measured. Total antioxidant status (TAS) was also tested. The patients with CF received vitamin supplementation in doses prescribed in international guidelines (α-tocopherol: <10 years 100 mg daily, >10 years 200 mg daily, retinol: 2.5 mg daily, ascorbic acid: 100–200 mg daily). Results: Plasma levels of malondialdehyde were significantly higher (p < 0.05), superoxide dismutase activities were significantly lower (p < 0.05) in patients with cystic fibrosis. There were no significant differences in catalase, glutathione peroxidase activities and TAS levels between CF patients and control group. Plasma ascorbic acid, α-tocopherol and retinol levels were within normal limits in both groups. Conclusion: On the basis of the present results this regime failed to provide sufficient antioxidant protection. Therefore, the authors suggest that the daily dose of these antioxidants should be either increased or to administer in parenteral route to patients with severe form of the disease.
Patients with cystic fibrosis (CF) exhibit normal concentrations of sodium and chloride in spite of the disturbance of Cl- and Na+ transport in epithelial cells. To characterize compensatory mechanisms in the regulation of sodium homeostasis, erythrocytes of 13 CF patients were analysed for sodium-lithium counter-transport (SLC), Na+/K(+)-ATPase activity and intracellular sodium content. Values were compared to those of healthy controls. Patients with CF had normal serum sodium and chloride concentrations and renal excretions of these ions were within the physiological range. Intracellular sodium concentration was similar in the CF and the control group (6.8 +/- 2.2 vs 5.7 +/- 1.0 mmol/l RBCs). Red blood cells' SLC and Na+/ K(+)-ATPase activity were elevated in CF patients (381 +/- 106 mumol/h/l RBCs vs 281 +/- 64; p < 0.01) and (445 +/- 129 mumol ATP mg prot/h vs 322 +/- 84, p < 0.01). Our study demonstrates that transmembrane cation transport systems are highly activated in CF. The increased sodium transport may be part of a compensatory mechanism of sodium homeostasis in children with CF.
Bevezetés: A cystás fi brosis progresszív genetikai betegség, amely korlátozhatja a betegek mindennapi életét, befolyá-solja életminőségüket. Célkitűzés: A szerzők célul tűzték ki a hazai cystás fi brosisban szenvedő betegek életminőségé-nek felmérését. Módszer: Az életminőség értékelésére betegségspecifi kus kérdőív (The Cystic Fibrosis Questionnaire -Revised) magyar nyelvre validált változatát alkalmazták. A betegség súlyossági állapotát Shwachman-Kulczyckipontszám segítségével határozták meg. Spirometriai vizsgálat is történt. Eredmények: A vizsgálatban 59 beteg (átlag-életkor 14,03±4,8 év) vett részt öt magyarországi centrumból. A 8-13 éves korosztályban a gyermekek és szüleik válaszai között a következő korrelációkat állapították meg: fi zikai aktivitás = 0,77 (p<0,001); érzelmi állapot = 0,07 (p<0,001); étkezési zavarok = 0,51 (p<0,001); kezelés terhe = 0,21 (p<0,001); testkép = 0,54 (p<0,001); légúti tünetek = 0,49 (p<0,001); emésztési tünetek = 0,40 (p<0,001). Következtetések: A gyermekkori életminőség felmé-rése során a gyermekek és szüleik véleménye szorosan megegyezett azokban a dimenziókban, amelyek a fi zikális területre vonatkoztak, azonban a pszichoszociális doménekben lényeges különbségek voltak mérhetők. Cystás fi brosisos gyermekek életminőség-vizsgálata során mind a gyermekek, mind a szülők véleményének fi gyelembevétele ajánlott. Orv. Hetil., 2013, 154, 784-791. Kulcsszavak: cystás fi brosis, életminőség, gyermekgyógyászat Quality of life in Hungarian patients with cystic fi brosisIntroduction: Cystic fi brosis is a progressive multisystemic disease which affects the quality of life of patients. Aim: The aim of the study was to evaluate quality of life in Hungarian patients with cystic fi brosis. Methods: Validated Hungarian translation of The Cystic Fibrosis Questionnaire -Revised was used to measure quality of life. Clinical severity was determined on the basis of Shwachman-Kulczycki score. Lung function was measured using spirometry. Results: 59 patients were included from fi ve centres in Hungary. The relationships between 8-13 year-old children self-report and parent proxy report was 0.77 (p<0.001) in physical functioning, 0.07 (p<0.001) in emotional functioning, 0.51 (p<0.001) in eating, 0.21 (p<0.001) in treatment burden, 0.54 (p<0.001) in body image, 0.49 (p<0.001) in respiratory symptoms and 0.40 (p<0.001) in digestive symptoms domains. Conclusions: In contrast to physical domains weak correlations were observed between answers obtained from children and their parents in psychosocial domains. The perception of both patients and their parents should be assessed when measuring quality of life in paediatric patients with cystic fi brosis. Orv. Hetil., 2013, 154, 784-791.
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