116 research outputs found

    Adherence to nebulised antibiotics in cystic fibrosis

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    Decision Making about Risk of Infection by Young Adults with CF

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    Young people with cystic fibrosis (CF) are asked to avoid a number of environments associated with increased infection risk, but in practice they need to balance this with competing priorities such as building and sustaining relationships with friends and family. This study explored the process by which young people make these decisions. Mixed methods were used: a vignette study presenting choices around engaging in activities involving a degree of infection risk and a thematic analysis of participant's accounts of their decision making. The eight participants chose to engage in high risk behaviours in 59% of the choices. All participants chose to engage in at least one risky behavior, though this was less likely when the risk was significant. Thematic analysis revealed large areas of misunderstanding and lack of knowledge, leading to some potentially worrying misconceptions about the nature of infections and risk. Young people with CF are not currently making informed decisions around activities that involve increased risk of infection, and there is an urgent need for CF teams to address this in information provision

    327 Employment and education in adults with cystic fibrosis: employment experiences

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    Endoscopic ultrasound in the assessment of advanced duodenal adenomatosis in familial adenomatous polyposis

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    Objective Current surveillance strategies for duodenal adenomatosis in familial adenomatous polyposis (FAP) miss malignancies and underestimate cancer risk in ampullary disease. This study aimed to evaluate the utility of endoscopic ultrasound (EUS) in the assessment of FAP patients with duodenal and/or ampullary polyposis referred for surgical intervention. Design A retrospective analysis of FAP patients undergoing index EUS between December 2006 and May 2015 was performed. Follow-up was completed in January 2018, including review of all EUS procedures and surgical interventions (median follow-up 6 years). Results Fifty-five patients underwent 188 EUS procedures. Six patients (11%) developed malignancy (three duodenal, three ampullary). Ampullary cancer risk was underestimated by Spigelman stage and overestimated by Kashiwagi classification. Ultrasound findings were poor predictors of malignancy, with common bile duct dilatation being the only finding present in one EUS prior to a diagnosis of ampullary cancer. The best predictors of ampullary malignancy were an ampullary polyp size >3 cm and an increase >1 cm in ampullary polyp size. Ampullary polyp size >3 cm provided the best predictive value, correctly identifying two of the three cases of ampullary cancer and both patients with high-grade dysplasia. EUS biopsy failed to detect malignancy later confirmed by surgical histology in two patients. Conclusion EUS surveillance confers little additional benefit to standard endoscopic surveillance in FAP patients. The best predictor of ampullary malignancy is an ampullary polyp >3 cm; this could be regarded as a relative indication for surgery

    Endoscopic ultrasound in the assessment of advanced duodenal adenomatosis in familial adenomatous polyposis

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    Objective: Current surveillance strategies for duodenal adenomatosis in familial adenomatous polyposis (FAP) miss malignancies and underestimate cancer risk in ampullary disease. This study aimed to evaluate the utility of endoscopic ultrasound (EUS) in the assessment of FAP patients with duodenal and/or ampullary polyposis referred for surgical intervention. Design: A retrospective analysis of FAP patients undergoing index EUS between December 2006 and May 2015 was performed. Follow-up was completed in January 2018, including review of all EUS procedures and surgical interventions (median follow-up 6 years). Results: Fifty-five patients underwent 188 EUS procedures. Six patients (11%) developed malignancy (three duodenal, three ampullary). Ampullary cancer risk was underestimated by Spigelman stage and overestimated by Kashiwagi classification. Ultrasound findings were poor predictors of malignancy, with common bile duct dilatation being the only finding present in one EUS prior to a diagnosis of ampullary cancer. The best predictors of ampullary malignancy were an ampullary polyp size >3 cm and an increase >1 cm in ampullary polyp size. Ampullary polyp size >3 cm provided the best predictive value, correctly identifying two of the three cases of ampullary cancer and both patients with high-grade dysplasia. EUS biopsy failed to detect malignancy later confirmed by surgical histology in two patients. Conclusion: EUS surveillance confers little additional benefit to standard endoscopic surveillance in FAP patients. The best predictor of ampullary malignancy is an ampullary polyp >3 cm; this could be regarded as a relative indication for surgery

    European guidelines from the EHTG and ESCP for Lynch syndrome: an updated third edition of the Mallorca guidelines based on gene and gender

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    Background: Lynch syndrome is the most common genetic predisposition for hereditary cancer but remains underdiagnosed. Large prospective observational studies have recently increased understanding of the effectiveness of colonoscopic surveillance and the heterogeneity of cancer risk between genotypes. The need for gene- and gender-specific guidelines has been acknowledged. Methods: The European Hereditary Tumour Group (EHTG) and European Society of Coloproctology (ESCP) developed a multidiscipli nary working group consisting of surgeons, clinical and molecular geneticists, pathologists, epidemiologists, gastroenterologists, and patient representation to conduct a graded evidence review. The previous Mallorca guideline format was used to revise the clinical guidance. Consensus for the guidance statements was acquired by three Delphi voting rounds. Results: Recommendations for clinical and molecular identification of Lynch syndrome, surgical and endoscopic management of Lynch syndrome-associated colorectal cancer, and preventive measures for cancer were produced. The emphasis was on surgical and gastroenterological aspects of the cancer spectrum. Manchester consensus guidelines for gynaecological management were en dorsed. Executive and layperson summaries were provided. Conclusion: The recommendations from the EHTG and ESCP for identification of patients with Lynch syndrome, colorectal surveil lance, surgical management of colorectal cancer, lifestyle and chemoprevention in Lynch syndrome that reached a consensus (at least 80 per cent) are presented

    The (ir)relevance of the abandoned criterion II for the diagnosis of serrated polyposis syndrome:a retrospective cohort study

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    The World Health Organization (WHO) recently updated the diagnostic criteria for serrated polyposis syndrome (SPS). One of the three previous diagnostic criteria (criterion II 2010) is now abandoned: ≥ 1 serrated polyp (SP) proximal to the sigmoid in a first-degree relative (FDR) of a patient with SPS. Individuals fulfilling this abandoned criterion now receive the same surveillance recommendations as all FDRs of patients with SPS. We aimed to compare the incidence of advanced neoplasia (AN) in FDRs with vs. without fulfillment of the abandoned criterion II 2010. We retrospectively recruited FDRs of patients with SPS who underwent a colonoscopy, and stratified them according to fulfilment of criterion II 2010 at baseline. Our primary and secondary outcomes were AN incidence during surveillance and at baseline, respectively. We included 224 FDRs of patients with SPS, of whom 36 (16%) fulfilled criterion II 2010 at baseline. One hundred and five underwent surveillance after baseline. Criterion II 2010-positive FDRs were at increased risk of AN, both during surveillance (hazard ratio 8.94, 95% CI 2.15–37.1, p =.003) as well as at baseline (adjusted odds-ratio 9.30, 95% CI 3.7–23.3, p <.001). FDRs of patients with SPS that underwent colonoscopy and fulfilled the abandoned criterion II 2010 for SPS diagnosis were at increased risk of AN at baseline and during surveillance in this small, retrospective cohort study. Our results should be interpreted with caution but suggest that adherence to surveillance recommendations for all FDRs of patients with SPS is important, especially for those that would have fulfilled the now abandoned criterion II 2010

    Evidence of objective sleep impairment in nonepileptic attack disorder : a naturalistic prospective controlled study using actigraphy and daily sleep diaries over six nights

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    Poor sleep is reported by many with nonepileptic attack disorder (NEAD) with correlations evident between self-reported sleep quality and mood and functional impairment. However, it is contended that self-reported sleep impairment in NEAD is a subjective phenomenon, which represents a general tendency to over-report symptoms or misinterpret bodily states in those with NEAD. The present study was therefore designed to investigate the extent of subjective and objective sleep impairments in those with NEAD. Over six nights we prospectively recorded comparable nightly objective (actigraphy) and subjective (consensus sleep diary) sleep parameters in a sample of 17 people with NEAD, and an age- and gender-matched normative control group (N = 20). Participants recorded daily measures of attacks, dissociation, and mood. Alongside higher subjective sleep impairment, the NEAD group had significantly worse objective sleep on several metrics compared to the normative controls, characterized by disrupted sleep (frequent awakenings and wake after sleep onset, low efficiency). Exploratory analyses using mixed effects models showed that attacks were more likely to occur on days preceded by longer, more restful sleep. This study, which had good ecological validity, evidences the presence of objective sleep impairment in NEAD, suggesting that in patient reports of problems with sleep should be given careful consideration in clinical practice

    Motivational Interviewing Post-Stroke: An Analysis of Stroke Survivors' Concerns and Adjustment

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    Our earlier research demonstrated that participation in four sessions of motivational interviewing (MI) early post-stroke has a positive impact on stroke survivors' mood. However, the theoretical underpinnings of MI in supporting adjustment (rather than its traditional use in supporting behavior change) require clarification. This article describes a content analysis of MI transcripts for 10 participants in our previous study, to identify the focus of discussions (patient "concerns") and potential effective components of our MI approach. Patients' post-stroke concerns were shown in 16 categories, including frustration, family impact, and getting well. There was a pattern of change discourse across sessions: "Sustain talk" (reasons for not changing) reduced from Session 1 onward, "change talk" (intent to change) increased then reduced, and "change expressed" (changes achieved) increased from Sessions 1 to 4. MI facilitates healthy adjustment post-stroke in some patients, in turn affecting mood, but clarification of how this effect is achieved requires further exploration
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