Background
A comparison of the longitudinal progression of lung disease in cystic fibrosis patients identified through newborn screening (NBS) in cohorts located in two different countries has never been performed and was the primary objective of this study.
Methods
The study included 56 patients in Brittany diagnosed through NBS between 1989 and 1994 and 69 similar patients in Wisconsin between 1985 and 1994. The onset and progression of lung disease was radiographically quantified using the Wisconsin Chest X-ray (WCXR) scoring system. A single pediatric pulmonologist blinded to all identifiers scored the films.
Results
Generalized estimating equation analyses adjusted for age, genotype, sex, pancreatic insufficiency, and meconium ileus showed worse WCXR scores in Brittany patients compared to Wisconsin patients (average score difference=4.48; p<0.001). Percent predicted FEV1 was also worse among Brittany patients (p<0.001).
Conclusions
The finding of milder radiographically-quantified lung disease using the WCXR scoring system, as well as better FEV1 values, may be explained by variations in nutrition, environmental exposures, or healthcare delivery.
between subjects with UC (n=41), CD (n=32) and their healthy twin at time of symptom onset. Medication and Stress: On review of all IBD sufferers, 7.1% (8/112) and 13.4% (15/112) used NSAIDS and antibiotics within 3 months preceding onset. 48.2% (54/112) reported significant stress within the year before diagnosis. Time of onset in concordant pairs: The mean lag between diagnosis of concordant pairs was 7 years 5 months. Conclusions: Concordance of twin pairs with CD is in keeping with the literature. UC concordance is greater than previously quoted. However DZ and MZ concordance of UC twins is similar inferring low heritability. DZ UC pair concordance is 4 fold expected non twin sibling concordance [2], suggesting early environment to be important. This study supports an associaton between diet, stress and gastrointestinal infection with IBD onset. The lack of associatoin with smoking at incidence may reflect sample size.
References:[1] Brant S, (2012) Background: An aberrant microbiota has been implicated in the pathophysiology of ulcerative colitis (UC) and several case series reported favorable effects of faecal microbiota transplantation (FMT). We aimed to assess the efficacy FMT in active UC patients in a randomized parallel group study. Methods: Patients with mild-moderate UC (Simple Clinical Colitis Activity Index [SCCAI] score of 4-11 and a Mayo endoscopic score of at least 1) were randomly assigned (1:1) to FMT derived from faeces from a healthy donor: FMT-D or their own faeces used as a placebo: FMT-P. FMT was administered via a naso-duodenal tube at week 0 and 3. Patients, physicians and endoscopists were blinded, with exception of the nurse who performed randomisation and prepared the faeces filtered with normal saline. The composite primary endpoint was clinical remission (SCCAI score 2 or lower) and endoscopic response (at least 1 point decrement on the Mayo endoscopic score) at week 12. Main secondary endpoints were microbiota composition in faecal samples and safety. Results: Of 50 patients who were screened, 48 were randomised (23 to FMT-D and 25 to FMT-P. In the ITT analysis, seven out of 23 patients (30.4%) in the donor arm versus five out of 25 patients in the placebo arm (20.0%) achieved the primary endpoint, P= .51. In the per protocol analysis 37 patients completed endoscopic follow-up; seven out of 17 patients in the donor arm (41.2%) versus five out of 20 (25.0%) in the placebo arm achieved the primary endpoint, P= .29. The majority of patients experienced mild adverse events with spontaneous recovery. Serious adverse events occurred in four patients and were considered not related to FMT. The trial was terminated after 50 inclusions due to futility. Redundancy analysis showed that at 12 weeks faecal microbiota of responders in the FMT-D group overlapped with healthy donors, which was associated with occurrence of Clostridium cluster XIVa. This was not seen in responders from the FMT-P group. Conclusions: FMT with faeces of a healthy donor did not result in statistically higher clinical...
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