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- 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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| 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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