| 1. |
- Moen, Inger E, et al.
(författare)
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Dietary intake and nutritional status in a Scandinavian adult cystic fibrosis-population compared with recommendations
- 2011
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Ingår i: Food & Nutrition Research. - : SNF Swedish Nutrition Foundation. - 1654-6628 .- 1654-661X. ; 55, s. 7561-
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Malnutrition is a well-known complication in cystic fibrosis (CF). There is good evidence that maintaining a normal body-weight correlates well with improved survival in CF. Energy intake in excess of 120% of the estimated average requirement (EAR) has been advised since 1980s. OBJECTIVES: To investigate the nutritional intake and status in the adult Scandinavian CF-population. SUBJECTS/METHODS: A cross-sectional multi-centre study was used to investigate the nutritional status of 456 adult CF-patients (2003 2006). Height and weight were measured and body mass index (BMI) and z-scores were calculated. Pulmonary function was examined by dynamic spirometry. A 7-day pre-coded food record (FR) obtained energy and nutrient intake data in 180 patients. RESULTS: The mean energy intake was 114 (SD 30.0)% of EAR and thus significantly lower than the target of 120% EAR (p< 0.001) for patients with pancreatic insufficiency (PI) (n=136). Mean BMI was 22.0 (SD 2.9), the prevalence of BMI <18 was 13% and the prevalence of BMI ≥25 was 15% (n=136). Mean BMI was 20.8 (SD 2.4) in PI-patients with FEV(1) <70% and 23.2% (SD 3.0), in PI-patients with FEV(1) ≥70%, mean difference 2.4, (95% CI: 1.5, 3.3) (p<0.001), but there was no difference in energy intake. BMI ≥18.5 and a reported energy intake <120% were revealed in 54% of the PI-patients. CONCLUSIONS: The energy intake did not reach the recommended 120% EAR, but the prevalence of underweight was lower than reported in other studies. The recommendation may exceed the requirement for a number of CF-patients. The nutritional status must still be closely monitored and nutritional advice and intervention should be individualised and adjusted to actual needs.
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| 2. |
- Ohlsson, Lena, et al.
(författare)
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Expression of intestinal and lung alkaline sphingomyelinase and neutral ceramidase in cystic fibrosis f508del transgenic mice.
- 2008
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Ingår i: Journal of Pediatric Gastroenterology and Nutrition - Jpgn. - 1536-4801. ; 47:5, s. 547-554
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVES: The intestinal brush border enzymes alkaline sphingomyelinase (alk-SMase) and neutral ceramidase (CDase) digest milk sphingomyelin in suckling neonates. In addition, alk-SMase, CDase, and acid sphingomyelinase (acid-SMase) have been implicated in sphingolipid signaling, which exhibits abnormalities in cystic fibrosis (CF). In this study, we tested the hypothesis that the expression of these enzymes is different in CF. MATERIALS AND METHODS: We used mice with F508del (Cftr) mutation, a CF mouse model with well-characterized intestinal pathology. Enzyme activities were measured using radiolabeled sphingolipid substrates incubated with tissue homogenates from different organs and intestinal contents of wild-type mice, homozygous, and heterozygous F508del mice. RESULTS: No difference was found in levels of CDase and alk-SMase in the small intestinal mucosa or in their longitudinal distribution. Acid-SMase activity was significantly lower in the mucosa of the distal half of the small intestine of F508del compared with wild-type mice. Despite a lower body weight of F508del mice, length and weight of the small intestine and weight per centimeter of colon were larger than in wild-type. Neutral CDase and alk-SMase activities in lungs were lower than in the gut, whereas acid-SMase activity was comparable in both organs. CDase activity in the spleen was significantly higher in F508del than in wild-type mice. CONCLUSIONS: Alk-SMase and neutral CDase are normally expressed in F508del CF mice, whereas activity of acid-SMase in the distal small intestine is decreased. We found no differences in activity of these enzymes in lungs in this mouse model.
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| 3. |
- Olesen, Hanne Vebert, et al.
(författare)
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Gender differences in the Scandinavian cystic fibrosis population.
- 2010
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Ingår i: Pediatric pulmonology. - : Wiley. - 1099-0496 .- 8755-6863. ; 45:10, s. 959-65
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Tidskriftsartikel (refereegranskat)abstract
- To explore whether gender differences in the Scandinavian Cystic Fibrosis (CF) patients exist in the areas of key clinical parameters, complications, and medication.
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| 4. |
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| 5. |
- Bäcke, Pyrola, et al.
(författare)
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When all I wanted was to hold my baby-The experiences of parents of infants who received therapeutic hypothermia
- 2021
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Ingår i: Acta Paediatrica. - : John Wiley & Sons. - 0803-5253 .- 1651-2227. ; 110:2, s. 480-486
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Tidskriftsartikel (refereegranskat)abstract
- Aim The knowledge is limited about how parents experience the time when their infant is receiving therapeutic hypothermia (TH) after severe perinatal asphyxia. The aim of this study was to explore parents' experience of closeness and involvement in their infant's care while in the neonatal intensive care unit (NICU) with their newborn undergoing TH. Methods Face-to-face, semi-structured interviews were conducted with parents (n = 11) whose infants (n = 8, aged 3-5 years at the time of the study) underwent TH at a level III Swedish NICU during 2013-2016. The interviews were analysed using qualitative content analysis. Results All the parents shared the trauma of being both physically and psychologically separated from their infant. They all described a need for information and emotional support, and reported that the NICU staff had influenced the extent to which they as parents had been able to be near and actively participate in the care. Parents described the wish to be closer to their infant and to be more actively involved in their infant's care. Conclusion Strategies to enable parent-infant closeness and active guidance from staff might help alleviate the emotional stress of parents and promote their participation during TH.
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| 6. |
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| 7. |
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| 8. |
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| 9. |
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| 10. |
- Hochwälder, Jacek, et al.
(författare)
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Psychometric evaluation of the Swedish translation of the revised Cystic Fibrosis Questionnaire in adults
- 2017
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Ingår i: Upsala Journal of Medical Sciences. - : TAYLOR & FRANCIS LTD. - 0300-9734 .- 2000-1967. ; 122:1, s. 61-66
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Tidskriftsartikel (refereegranskat)abstract
- Aim: The CFQ-R is one of the most established disease-specific, health-related quality of life (HRQOL) measurements for patients with cystic fibrosis (CF). The aim was to evaluate the psychometric properties of the Swedish translation of CFQ-R in adults. Method: A total of 173 CF patients answered the CFQ-R. The CFQ-R was evaluated with regard to: (1) distributional properties; (2) reliability; and (3) construct validity. Results: The majority of scales were negatively skewed with ceiling effects. Eight of the 12 scales had satisfactory homogeneity; 10 of the 12 scales had satisfactory test-retest reliability. On many of the CFQ-R scales expected differences were observed when patients were divided regarding disease severity, nutritional status, age, and gender. Conclusion: Some weaknesses were detected, but overall the instrument has satisfactory psychometric properties.
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| 11. |
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| 12. |
- Nilsson, Åke, et al.
(författare)
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Adaptive regulation of lipoprotein lipase and salt-resistant lipase activities in essential fatty acid deficiency. An experimental study in the rat.
- 1990
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Ingår i: Metabolism. ; 39:12, s. 1305-1308
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Tidskriftsartikel (refereegranskat)abstract
- Lipoprotein lipase (LPL) activities of postheparin plasma, heart, lungs, and adipose tissue, and salt-resistant lipase (hepatic lipase, SRL) activities of postheparin plasma, liver, and adrenals were examined in essential fatty acid deficient (EFAD) rats and in age-matched controls. The LPL activity of heart was higher in the deficient than in the control rats, but did not differ in the other tissues. The SRL activity of postheparin plasma was twofold higher, and that of liver and adrenals approximately 50% higher in the group with EFA deficiency. It is suggested that SRL exhibits an adaptive up-regulation in EFA deficiency. This up-regulation may be linked to a role for the enzyme in the transport of polyenoic fatty acids.
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| 13. |
- Nilsson, Åke, et al.
(författare)
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Incorporation of dietary 14Carachidonic and 3Heicosapentaenoic acid into tissue lipids during absorption of a fisk oil emulsion
- 1992
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Ingår i: Journal of Lipid Research. - 1539-7262. ; 33:9, s. 1295-1305
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Tidskriftsartikel (refereegranskat)abstract
- A preferential incorporation of dietary arachidonic acid (20:4, n-6) into chyle lipoprotein phospholipids, a relative resistance of 20:4 esters of chyle triacylglycerol (TG) to hydrolysis by lipoprotein lipase, a preferential utilization of 20:4 for phospholipid acylation, and a low rate of oxidation of 20:4 are factors that may contribute to the differences seen in the incorporation into tissue lipids between absorbed 20:4 and the predominant dietary 16-18 carbon fatty acids. In this study we fed [14C]20:4 and [3H]eicosapentaenoic acid (20:5, n-3) as free fatty acids in a fish oil emulsion to rats and analyzed the radioactivity in different tissue lipids after 1, 2, and 4 h. The purpose was to examine the degree of similarity in the fate of the two major eicosanoid precursors during the absorption of a fish oil meal. The recovery after 2 and 4 h of 14C exceeded that of 3H in lipids of small intestine, serum, liver, heart, kidneys, and spleen. The differences increased with time, e.g., the liver contained 9.7 (+/- 0.7)% 3H and 17.9 (+/- 1.4)% of the 14C (P less than 0.001), and the upper half of the small intestine 10.0 (+/- 0.8)% of the 3H and 22.8 (+/- 1.1)% of the 14C (P less than 0.001) after 4 h. The 14C and 3H radioactivity per g tissue after 4 h ranked as follows: liver and brown adipose tissue greater than kidneys greater than heart, lungs, spleen, and serum greater than colon greater than white adipose tissue and testes, the differences between tissues being up to 50-fold. There were up to fourfold variations in the 14C/3H ratios between tissues after 4 h, the highest value being observed in the heart and the lowest in white adipose tissue. Of the radioactivity retained in liver and intestine, more 14C and 3H was in phospholipids and less in triacylglycerol (TG), the differences being largest in the liver, e.g., after 4 h 57.6 (+/- 0.8)% of the 14C and 29.9 (+/- 0.9)% of the 3H (P less than 0.001) in the liver was in phosphatidylcholine (PC). In both intestine and liver the highest 14C/3H ratios were found in phosphatidylinositiol (PI). Also phosphatidylethanolamine (PE) contained more 14C than 3H but the quantitative differences were relatively small after 4 h. In heart the proportions of 3H and 14C found in PE and PI did not differ, whereas more of the 14C was in PC and more of the 3H was in cardiolipin and phosphatidylserine.(ABSTRACT TRUNCATED AT 400 WORDS).
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| 14. |
- Nydert, Per, et al.
(författare)
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Chitosan as a carrier for non-viral gene transfer in a cystic-fibrosis cell line
- 2008
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Ingår i: Biotechnology and applied biochemistry. - 0885-4513 .- 1470-8744. ; 51:Pt 4, s. 153-7
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Tidskriftsartikel (refereegranskat)abstract
- The gene transfer mediated by chitosan in CFBE41o(-) (a cystic-fibrosis bronchial epithelial cell line) and HEK (a human embryonic kidney cell line) has been evaluated. Polyplexes based on chitosan and PEI (polyethyleneimine) using a luciferase and enhanced green fluorescent protein reporter plasmid showed that the transfection efficacy of polyplexes in the CFBE41o(-) cell line was poor compared with that in HEK cells. In the highly differentiated cystic-fibrosis bronchial epithelial cell line the narrow-size-distributed chitosan shows enhanced transfection at a low pH compared with PEI. The enhanced transfection at lower pH could be a result of damage to the cell surface or changes in the cell-surface charge, leading to better penetration of the cell membrane.
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| 15. |
- Pincikova, T, et al.
(författare)
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Inverse relation between vitamin D and serum total immunoglobulin G in the Scandinavian Cystic Fibrosis Nutritional Study.
- 2011
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Ingår i: European journal of clinical nutrition. - : Springer Science and Business Media LLC. - 1476-5640 .- 0954-3007. ; 65:1, s. 102-9
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Tidskriftsartikel (refereegranskat)abstract
- The hallmark of cystic fibrosis (CF) is chronic lung inflammation. The severity of lung disease is closely correlated with immunoglobulin G (IgG) levels. Beyond its contribution to the bone health, the importance of vitamin D has not been fully recognized owing to the lack of human studies providing evidence of its benefit. In the context of the recently described immunomodulatory functions of vitamin D, we aimed to assess the relationship between vitamin D and IgG levels.
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| 16. |
- Pincikova, Terezia, et al.
(författare)
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MAIT cell counts are associated with the risk of hospitalization in COPD
- 2022
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Ingår i: Respiratory Research. - : BioMed Central (BMC). - 1465-9921 .- 1465-993X. ; 23:1
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundChronic obstructive pulmonary disease (COPD) is characterized by persistent airflow limitation associated with chronic inflammation in the airways. Mucosal-associated invariant T (MAIT) cells are unconventional, innate-like T cells highly abundant in mucosal tissues including the lung. We hypothesized that the characteristics of MAIT cells in circulation may be prospectively associated with COPD morbidity.MethodsCOPD subjects (n = 61) from the Tools for Identifying Exacerbations (TIE) study were recruited when in stable condition. At study entry, forced expiratory volume in 1 s (FEV1) was measured and peripheral blood mononuclear cells were cryopreserved for later analysis by flow cytometry. Patients were followed for 3 years to record clinically meaningful outcomes.ResultsPatients who required hospitalization at one or more occasions during the 3-year follow-up (n = 21) had lower MAIT cell counts in peripheral blood at study inclusion, compared with patients who did not get hospitalized (p = 0.036). In contrast, hospitalized and never hospitalized patients did not differ in CD8 or CD4 T cell counts (p = 0.482 and p = 0.221, respectively). Moreover, MAIT cells in hospitalized subjects showed a more activated phenotype with higher CD38 expression (p = 0.014), and there was a trend towards higher LAG-3 expression (p = 0.052). Conventional CD4 and CD8 T cells were similar between the groups. Next we performed multi-variable logistic regression analysis with hospitalizations as dependent variable, and FEV1, GOLD 2017 group, and quantity or activation of MAIT and conventional T cells as independent variables. MAIT cell count, CD38 expression on MAIT cells, and LAG-3 expression on both MAIT and CD8 T cells were all independently associated with the risk of hospitalization.ConclusionsThese findings suggest that MAIT cells might reflect a novel, FEV1-independent immunological dimension in the complexity of COPD. The potential implication of MAIT cells in COPD pathogenesis and MAIT cells’ prognostic potential deserve further investigation.
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| 17. |
- Pincikova, Terezia, et al.
(författare)
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Severely Impaired Control of Bacterial Infections in a Patient With Cystic Fibrosis Defective in Mucosal-Associated Invariant T Cells
- 2018
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Ingår i: Chest. - : ELSEVIER SCIENCE BV. - 0012-3692 .- 1931-3543. ; 153:5, s. E93-E96
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Tidskriftsartikel (refereegranskat)abstract
- Here we report a unique case of a patient with cystic fibrosis characterized by severely impaired control of bacterial respiratory infections. This patient's susceptibility to such infections was much worse than expected from a cystic fibrosis clinical perspective, and he died at age 22 years despite extensive efforts and massive use of antibiotics. We found that this severe condition was associated with a near-complete deficiency in circulating mucosal-associated invariant T (MAIT) cells as measured at several time points. MAIT cells are a large, recently described subset of T cells that recognize microbial riboflavin metabolites presented by the highly evolutionarily conserved MR1 molecules. The MAIT cell deficiency was specific; other T-cell subsets were intact. Even though this is only one unique case, the findings lend significant support to the emerging role of MAIT cells in mucosal immune defense and suggest that MAIT cells may significantly modify the clinical phenotype of respiratory diseases.
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| 18. |
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| 19. |
- Pincikova, T., et al.
(författare)
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Vitamin D deficiency as a risk factor for cystic fibrosis-related diabetes in the Scandinavian Cystic Fibrosis Nutritional Study
- 2011
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Ingår i: Diabetologia. - : Springer Science and Business Media LLC. - 1432-0428 .- 0012-186X. ; 54:12, s. 3007-3015
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Tidskriftsartikel (refereegranskat)abstract
- Aims/hypothesis Many cystic fibrosis patients are vitamin D-insufficient. Cystic fibrosis-related diabetes is a major complication of cystic fibrosis. The literature suggests that vitamin D might possess certain glucose-lowering properties. We aimed to assess the relationship between vitamin D and cystic fibrosis-related glucose intolerance. Methods We enrolled 898 cystic fibrosis patients from Sweden, Norway and Denmark. Vitamin D intake was assessed using a seven-day food record. Serum 25-hydroxyvitamin D (s25OHD) and HbA(1c) were measured, and an OGTT was carried out. Multiple linear and logistic regressions were used for HbA(1c) and cystic fibrosis-related diabetes/OGTT result as outcome variables, respectively. Each model was controlled for country, and for known cystic fibrosis-related diabetes risk factors: age, sex, genotype, liver dysfunction, long-term corticosteroid treatment, and lung and pancreatic function. Results Degree of vitamin D insufficiency (OR 1.36; p=0.032) and s25OHD<30 nmol/l (OR 1.79; p=0.042) were significant risk factors for cystic fibrosis-related diabetes. Accordingly, HbA(1c) value was positively associated with s25OHD<30 nmol/l and<50 nmol/l, as well as with degree of vitamin D insufficiency (adjusted R-2=20.5% and p<0.05 in all). In subgroup analyses, s25OHD<30 nmol/l determined the HbA(1c) value in paediatric patients (adjusted R-2=20.2%; p=0.017), but not in adults. Conclusions/interpretation Vitamin D status is associated with HbA(1c) and diabetes in cystic fibrosis, particularly in children. The study justifies prospective studies on the proposed role of vitamin D deficiency in the pathophysiology of diabetes mellitus.
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| 20. |
- Pressler, Tacjana, et al.
(författare)
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Diagnostic significance of measurements of specific IgG antibodies to Pseudomonas aeruginosa by three different serological methods
- 2009
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1873-5010 .- 1569-1993. ; 8:1, s. 37-42
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Tidskriftsartikel (refereegranskat)abstract
- The aim of the study was to evaluate three serological methods for their ability to identify CF patients in different infection status especially those at risk of developing chronic Pseudomonas aeruginosa (Pa) infection. Methods: Two ELISA methods: exotoxin A (ExoA) and CF-IgG-ELISA (CF-IgG) and Crossed Immunoelectrophoresis (CIE) were used for measurement of Pa-antibodies in sera from 791 Scandinavian CF patients. Results: 381 patients were cultured negative for Pa in the year before study start, 129 patients were intermittently colonized and 281 patients were chronically infected. The sensitivity of the investigated assays was 96%, 93% and 97%, specificity 89%, 89% and 83% for CIE, ExoA and CF-IgG respectively. The negative predictive value was for CIE 97%, for ExoA 95% and for CF-IgG 98% and positive predictive values 87%, 86% and 80%. Out of the 381 patients cultured negative for Pa, 11 changed status to chronically infected. Twenty-four out of the 129 patients intermittently colonized became chronically infected. The antibody levels in this latter group of patients were significantly higher already at the study start and increased significantly during the study period (p < 0.05). Elevated levels of specific anti-Pseudomonal antibodies showed to be the risk factor for developing chronic P. aeruginosa infection (OR 4.9 and OR 2.7, p < 0.05 for CF-IgG and ExoA). Conclusion: All three serological assays were equally informative. The very high sensitivity of the assays made it possible to characterize patients with different infection status. Elevated levels of specific anti-Pseudomonas antibodies showed to be the risk factor for developing chronic Pa infection. Due to the specificity of the tests, antibiotic treatment based on serology might be considered in selected cases. There is a window of opportunity for suppression and eradication of initial P aeruginosa infection making measurement of specific anti-Pseudomonas antibodies helpful. (c) 2008 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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| 21. |
- Servetnyk, Zhanna, et al.
(författare)
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Activation of chloride transport in CF airway epithelial cell lines and primary CF nasal epithelial cells by S-nitrosoglutathione
- 2006
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Ingår i: Respiratory Research. - : Springer Science and Business Media LLC. - 1465-9921 .- 1465-993X. ; 7, s. 124-
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Tidskriftsartikel (refereegranskat)abstract
- Background: It has been suggested that low mu M concentrations of S-nitrosoglutathione (GSNO), an endogenous bronchodilator, may promote maturation of the defective cystic fibrosis (CF) transmembrane conductance regulator ( CFTR). Because nitric oxide ( NO) and GSNO levels appear to be low in the CF airway, there is an interest in the possibility that GSNO replacement could be of therapeutic benefit in CF.Methods: The effect of GSNO on chloride (Cl-) transport was investigated in primary nasal epithelial cells obtained from CF patients homozygous for the delF508 mutation, as well as in two CF cell lines (CFBE and CFSME), using both a fluorescent Cl- indicator and X-ray microanalysis. Maturation of delF508 CFTR was determined by immunoblotting.Results: Treatment with 60 mu M GSNO for 4 hours increased cAMP-induced chloride efflux in nasal epithelial cells from 18 out of 21 CF patients, but did not significantly affect Cl- efflux in cells from healthy controls. This Cl- efflux was confirmed by measurements with a fluorescent Cl- indicator in the CFBE and CFSME cell lines. The effect of GSNO on Cl- efflux in CFBE cells could be inhibited both by a specific thiazolidinone CFTR inhibitor (CFTRinh-172) and by 4,4'-diisothiocyanatodihydrostilbene- 2,2'-disulfonic acid (H2DIDS). X-ray microanalysis showed that, following 4 hours incubation with 60 mu M GSNO, cAMP agonists caused a decrease in the cellular Cl- concentration in CFBE cells, corresponding to Cl- efflux. GSNO exposure resulted in an increase in the protein expression and maturation, as shown by immunoblot analysis. GSNO did not increase the cytosolic Ca2+ concentration in cultured airway epithelial cells.Conclusion: Previous studies have suggested that treatment with GSNO promotes maturation of delF508-CFTR, consistent with our results in this study. Here we show that GSNO increases chloride efflux, both in the two CF cell lines and in primary nasal epithelial cells from delF508-CF patients. This effect is at least in part mediated by CFTR. GSNO may be a candidate for pharmacological treatment of the defective chloride transport in CF epithelial cells.
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| 22. |
- Servetnyk, Zhanna, et al.
(författare)
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The effect of S-nitrosoglutathione and L-cysteine on chloride efflux from cystic fibrosis airway epithelial cells
- 2011
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Ingår i: Experimental and molecular pathology (Print). - : Elsevier BV. - 0014-4800 .- 1096-0945. ; 90:1, s. 79-83
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Tidskriftsartikel (refereegranskat)abstract
- The endogenous bronchodilator, S-nitrosoglutathione (GSNO), has been proposed as a possible pharmacological remedy that reverses the Delta F508-CFTR (cystic fibrosis transmembrane conductance regulator) maturation defect and increases CFTR-mediated chloride efflux in cultured cystic fibrosis airway epithelial cells (CFBE41o(-)). It has also been reported that L-cysteine enhanced S-nitrosothiol uptake and increased the intracellular S-nitrosothiol levels, likely through transnitrosation chemistry. The present study investigated whether L-cysteine augmented the effect of GSNO on chloride efflux from CF airway epithelial cells. Treatment with 10 mu M GSNO combined with 20 mu M L-cysteine resulted in increased chloride efflux from CFBE41o(-) cells after 5 minutes exposure compared to the control efflux rate and to the efflux rate in the presence of L-cysteine alone as measured using the fluorescent dye N-(ethoxycarbonylmethyl)-6-methoxyquinolinium bromide (MQAE). Chloride efflux rates from these cells after 4 h exposure to GSNO and L-cysteine were not different from control. Treatment with 10 mu M GSNO alone increased chloride efflux from CFBE41o(-) cells after 4 h but not at shorter incubation times. GSNO with or without L-cysteine did not alter epithelial tight junction integrity. In conclusion, a combination of GSNO with L-cysteine led to significant increase in chloride efflux in CFBE41o(-) cells but the effect was transient and not sustained beyond minutes.
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