| 1. |
- Gilljam, Marita, et al.
(författare)
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Lung transplantation in patients with cystic fibrosis and Mycobacterium abscessus infection
- 2010
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 9:4, s. 272-276
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Tidskriftsartikel (refereegranskat)abstract
- Mycobacterium abscessus lung disease is difficult to treat and has been considered a strong relative contraindication to lung transplantation. We performed double lung transplantation in three cystic fibrosis patients with ongoing, and a fourth with recent treatment for Mycobacterium abscessus lung infection. Despite prolonged antibiotic courses and adjustment of immunosuppressive therapy the first three patients developed skin infection and abscesses. At follow-up after 1, 3, 5 and 7 years respectively no patient had evidence of M abscessus infection and all had stable lung function. Lung transplantation in patients with M abscessus lung infection is feasible but may involve severe complications.
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| 2. |
- Gronowitz, Eva, 1956, et al.
(författare)
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Ultraviolet B radiation improves serum levels of vitamin D in patients with cystic fibrosis
- 2005
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Ingår i: Acta Paediatr. - : Wiley. - 0803-5253. ; 94:5, s. 547-52
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Ultraviolet B (UVB) radiation can be used in the prevention and treatment of vitamin D deficiency. AIM: To investigate, in a controlled study of patients with cystic fibrosis (CF), whether regular UVB radiation would improve serum levels of calcidiol during the dark season (October-April). METHODS: Thirty patients with mild to moderate disease were included (aged 9-40 y). All patients had cholecalciferol supplementation. One group (15 patients) was given UVB one to three times a week for 6 mo and one group (15 sex- and age-matched patients) served as controls. The radiation source consisted of three TL 12/40W UVB fluorescent lamps. Initial treatment duration was 1 min, subsequently increased by 0.5-1 min/treatment to a maximum of 10 min. RESULTS: The mean initial serum calcidiol levels were 21 ng/ml in the controls and 22 ng/ml in the intervention group. Serum calcidiol levels increased to 44 ng/ml after 8 wk and to 50 ng/ml after 24 wk of UVB radiation; the corresponding serum levels in the controls were 19 and 25 ng/ml, respectively. The mean serum calcitriol levels increased in the treated group and were unaltered in the control group. CONCLUSIONS: UVB radiation was effective in increasing vitamin D levels in patients with CF. The study results imply that UVB radiation is valuable in chronic conditions associated with vitamin D deficiency.
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| 3. |
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| 4. |
- Krantz, Christina, et al.
(författare)
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Exhaled and nasal nitric oxide in relation to lung function, blood cell counts and disease characteristics in cystic fibrosis
- 2017
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Ingår i: Journal of Breath Research. - : IOP Publishing. - 1752-7155 .- 1752-7163. ; 11:2
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Patients with cystic fibrosis (CF) have similar or lower exhaled nitric oxide (FeNO) and lower nasal nitric oxide (nNO) levels than controls. There are divergent results on alveolar NO (CalvNO) concentrations in relation to CF. There are inconsistent results on correlation between different nitric oxide parameters and lung function and inflammation in CF.AIM: To compare FeNO, CalvNO and nNO levels between subjects with CF, asthma and healthy controls and to study whether these parameters are related to lung function, blood cell counts or clinical characteristics in CF patients.MATERIAL AND METHODS: Measurements of FeNO at multiple exhalation flow rates, nNO and spirometry were done in 38 patients (18 adults) with CF. Blood cell counts and CF clinical characteristics were recorded. Thirty-eight healthy controls and 38 asthma patients, gender- and age-matched, were included as reference groups.RESULTS: FeNO levels were lower in CF patients (7.2 [4.7-11.2] ppb) than in healthy controls (11.4 [8.3-14.6] ppb) and asthma patients (14.7 [8.7-24.7] ppb) (both p < 0.005). These differences were consistent in adults. No difference in CalvNO was seen between the groups. nNO levels in CF patients (319 [193-447] ppb) were lower than in healthy controls (797 [664-984] ppb) and asthma patients (780 [619-961] ppb) (both p < 0.001). FeNO positively related to FEV1 (rho = 0.51, p = 0.001) in CF patients and this was consistent in both adults and children. A negative correlation was found between FeNO and blood neutrophil counts (rho = -0.37, p = 0.03) in CF patients.CONCLUSION: CF patients have lower FeNO and nNO and similar CalvNO levels as healthy controls and asthma patients. Lower FeNO related to lower lung function in both adults and children with CF. Furthermore, in CF, lower FeNO also related to higher blood neutrophil counts.
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| 5. |
- 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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| 6. |
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| 7. |
- 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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| 8. |
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| 9. |
- 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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| 10. |
- Qvist, Tavs, et al.
(författare)
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Epidemiology of nontuberculous mycobacteria among patients with cystic fibrosis in Scandinavia
- 2015
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Ingår i: Journal of Cystic Fibrosis. - : Elsevier BV. - 1569-1993 .- 1873-5010. ; 14:1, s. 46-52
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Tidskriftsartikel (refereegranskat)abstract
- Background: Nontuberculous mycobacteria (NTM) are an emerging threat to cystic fibrosis (CF) patients but their epidemiology is not well described. Methods: In this retrospective observational study we identified all Scandinavian CF patients with a positive NTM culture from airway secretions from 2000 to the end of 2012 and used national CF databases to describe microbiological and clinical characteristics. Results: During the 13-year period 157 (11%) CF patients were culture positive for NTM at least once. Mycobacterium abscessus complex (MABSC) (45%) and Mycobacterium avium complex (MAC) (32%) were the predominant species with geographical differences in distribution. Younger patients were more prone to MABSC (p < 0.01). Despite treatment, less than one-third of MABSC patients with repeated positive cultures cleared their infection and a quarter had a lung transplant or died. Conclusion: NTM are significant CF pathogens and are becoming. more prevalent in Scandinavia. MABSC and MAC appear to target distinct patient groups. Having multiple positive cultures despite treatment conveys a poor outcome. (C) 2014 The Authors. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.
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