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- Davies, Julia R., et al.
(author)
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Identification of MUC5B, MUC5AC and small amounts of MUC2 mucins in cystic fibrosis airway secretions
- 1999
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In: Biochemical Journal. - 0264-6021. ; 344:2, s. 321-330
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Journal article (peer-reviewed)abstract
- To investigate the genetic identities of the mucins secreted in cystic fibrosis (CF) airways, sputum was collected from five individuals. Samples were separated into gel and sol phases by high-speed centrifugation and the gel phase was extracted in 6 M guanidinium chloride. The 'insoluble' residue remaining after extraction of the gel phase was brought into solution by reduction/alkylation. Density-gradient centrifugation in CsCl revealed polydisperse distributions of sialic acid-containing mucins in the gel phase, insoluble residue and sol phase fractions and the degree of variation between the different individuals was low. Antibodies recognizing MUC5AC and MUC5B identified these mucins in each of the fractions. MUC2, however, was present only as a component of the insoluble residue from the gel which accounted for less than 4% by mass of the total mucins, MUC5B and MUC5AC from the gel phase were large oligomeric species composed of disulphide-bond linked subunits and MUC5B was present as two populations with different charge densities which are likely to correspond to MUC5B 'glycoforms'. The sol phase contained, in addition to MUC5AC and MUC5B mainly smaller mucins which did not react with the antisera and which were probably degraded. MUC5AC appeared to be enriched in the sol, suggesting that this mucin may be more susceptible to proteolytic degradation than MUC5B. The mucins present in sputum remained broadly similar during acute exacerbation and following antibiotic treatment, although the relative amount of an acidic MUC5B glycoform was decreased during infection.
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- Magnusson, Gunilla, 1968, et al.
(author)
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Evaluation of screening procedures for congenital cataracts.
- 2003
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In: Acta paediatrica (Oslo, Norway : 1992). - : Wiley. - 0803-5253 .- 1651-2227. ; 92:12, s. 1468-73
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Journal article (peer-reviewed)abstract
- AIM: To evaluate the efficacy of two different Swedish screening procedures for early detection of congenital cataracts in comparison with no screening. METHODS: Children born between January 1992 and December 1998 in Swedish regions with an established eye-screening routine procedure, diagnosed with congenital cataract, and operated on before 1 y of age, were included in a retrospective study. Age at referral and age at time of the operation were compared between regions using different screening procedures: screening in the maternity wards (Region 1), at the well-baby clinics (Region 2) and one region without any screening (Region 3). RESULTS: Seventy-two children were included in the study. Concerning early diagnosis and surgery, Region 1 differed significantly from Regions 2 and 3, which were more similar and were combined for further analysis. The difference in detected cases was greatest at 21 d of age (55% vs 18%; p < 0.001), but persisted even at 100 d of age (78% vs 64%; p < 0.02). Region 1 screening resulted in more and earlier cases detected than the other two regions (22 vs 15 per 100,000 births). In 72% of all cases, surgery was performed in response to referrals from either the maternity wards (36%), or the well-baby clinics (36%). However, half of the cases from the well-baby clinics were detected too late, i.e. at > 100 d. CONCLUSION: Eye screening in the maternity ward is preferable to well-baby clinic screening and to no screening at all, since it leads to early detection. Screening should also be performed routinely at well-baby clinics within the period when successful treatment is possible.
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- Schaedel, Charlotta, et al.
(author)
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Predictors of deterioration of lung function in cystic fibrosis.
- 2002
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In: Pediatric Pulmonology. - : Wiley. - 8755-6863 .- 1099-0496. ; 33:6, s. 483-491
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Journal article (peer-reviewed)abstract
- The severity of lung disease in cystic fibrosis (CF) may be related to the type of mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, and to environmental and immunological factors. Since pulmonary disease is the main determinant of morbidity and mortality in CF, it is important to identify factors that can explain and predict this variation. The aim of this longitudinal study of the whole Swedish CF population over age 7 years was to correlate genetic and clinical data with the rate of decline in pulmonary function. The statistical analysis was performed using the mixed model regression method, supplemented with calculation of relative risks for severe lung disease in age cohorts.The severity of pulmonary disease was to some extent predicted by CFTR genotype. Furthermore, the present investigation is the first long-term study showing a significantly more rapid deterioration of lung function in patients with concomitant diabetes mellitus. Besides diabetes mellitus, pancreatic insufficiency and chronic Pseudomonas colonization were found to be negative predictors of pulmonary function. In contrast to several other reports, we found no significant differences in lung function between genders. Patients with pancreatic sufficiency have no or only a slight decline of lung function with age once treatment is started, but an early diagnosis in this group is desirable.
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- Wennstrom, Inga-Lill, et al.
(author)
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Gender affects self-evaluation in children with cystic fibrosis and their healthy siblings.
- 2005
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In: Acta Pædiatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 94:9, s. 1320-1326
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Journal article (peer-reviewed)abstract
- Aim: To determine whether self-esteem among children with cystic fibrosis (CF) and their healthy siblings differs from that of a healthy reference group and whether there are differences within and between sibling pairs. Methods: All Swedish CF children 6–14 y old with a healthy sibling in the same age range (n=65) were invited to participate, 55 sibling pairs and their parents taking part in the study. Five aspects of the children's self-concept—physical characteristics, skills and talents, mental well-being, relations to parents and family, and relations to others—were assessed by the "I think I am" self-evaluation questionnaire. Severity of illness was assessed by means of the Shwachman Clinical Evaluation System. Results: Whereas self-evaluation did not differ between groups at a general level, healthy girls as well as those with CF scored lower than girls in the reference group on the "mental well-being" and "relations to parents and family" subscales. Comparison of gender combinations (sick girl/healthy boy, sick girl/healthy girl, sick boy/healthy boy, sick boy/healthy girl) suggested that girls pay a cost of a lesser sense of psychological well-being and feelings of inadequacy in relation to their parents and family. The Shwachman score of the sick child was not related to the level of self-esteem. Conclusion: When CF is present among siblings, girls seem to carry more of the family pain than boys, a finding that calls for an increased awareness of the girls' situation by members of care teams.
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- Westbom, Lena, et al.
(author)
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Chronic illness among children in a total population. An epidemiological study in a Swedish primary health care district
- 1987
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In: Scandinavian Journal of Social Medicine. - 0300-8037. ; 15:2, s. 87-97
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Journal article (peer-reviewed)abstract
- The prevalence of chronic illness of all 6,080 0-15-year-old children in a defined geographical area in southern Sweden was studied. Information on the health status of the children was obtained from health and medical records, interviews with the district and school nurses and questionnaires to the parents. Chronic illness was defined as a disability interfering with normal life and/or demanding treatment for at least three months during the year 1981 and was revealed in 510 children corresponding to the period prevalence 84/1,000 with 95% confidence interval (CI) 60-108/1,000. Boys predominated. 131 children suffered from more than one disease. Chronic illness caused severe disability in 40 children, moderate in 113 and mild in 357 children. Atopic disorders were the leading cause of chronic illness (34/1,000 with 95% CI 29-39/1,000). Mental and nervous system disorders and congenital malformations were the most frequent causes of severe disability. Prevalence figures for the different diagnoses in relation to disability level, sex and age are presented.
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