ABSTRACT
A multidisciplinary committee developed evidence-based guidelines for the management of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen-positive, inconclusive diagnosis (CRMS/CFSPID). A total of 24 patient, intervention, comparison, and outcome questions were generated based on surveys sent to people with CRMS/CFSPID and clinicians caring for these individuals, previous recommendations, and expert committee input. Four a priori working groups (genetic testing, monitoring, treatment, and psychosocial/communication issues) were used to provide structure to the committee. A systematic review of the evidence was conducted, and found numerous case series and cohort studies, but no randomized clinical trials. A total of 30 recommendations were graded using the US Preventive Services Task Force methodology. Recommendations that received ≥80% consensus among the entire committee were approved. The resulting recommendations were of moderate to low certainty for the majority of the statements because of the low quality of the evidence. Highlights of the recommendations include thorough evaluation with genetic sequencing, deletion/duplication analysis if <2 disease-causing variants were noted in newborn screening; repeat sweat testing until at least age 8 but limiting further laboratory testing, including microbiology, radiology, and pulmonary function testing; minimal use of medications, which when suggested, should lead to shared decision-making with families; and providing communication with emphasis on social determinants of health and shared decision-making to minimize barriers which may affect processing and understanding of this complex designation. Future research will be needed regarding medication use, antibiotic therapy, and the use of chest imaging for monitoring the development of lung disease.
Subject(s)
Cystic Fibrosis , Evidence-Based Medicine , Humans , Cystic Fibrosis/therapy , Cystic Fibrosis/genetics , Cystic Fibrosis/diagnosis , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Infant, Newborn , Neonatal Screening/methods , Genetic Testing , ChildABSTRACT
PURPOSE: The benefits of prophylactic coronary revascularization for patients undergoing noncardiac surgery are uncertain. The purpose of this study was to systematically evaluate the effect of coronary revascularization and medical management on short- and long-term outcomes after noncardiac surgery. METHOD: Ten electronic databases including MEDLINE and EMBASE (1980 to February 2006), and bibliographies of included articles were searched without language restrictions. Studies comparing effects of coronary revascularization and medical management before noncardiac surgery were included. Patient outcome data including perioperative mortality, myocardial infarction, long-term mortality, or late adverse cardiac events were extracted and entered into a meta-analysis. RESULTS: The quality of published evidence was modest, comprising one randomized controlled trial and six retrospective studies. A total of 3,949 patients undergoing high-risk noncardiac surgery were included in the quantitative analysis. There was no significant difference between coronary revascularization and medical management groups with regards to postoperative mortality and myocardial infarction; the odds ratios (95% confidence intervals) were 0.85 (0.48-1.50) and 0.95 (0.44-2.08), respectively. There were no long-term outcome benefits associated with prophylactic coronary revascularization; the odds ratios (95% confidence intervals) were 0.81 (0.40-1.63) and 1.65 (0.70-3.86) for long-term mortality and late adverse cardiac events, respectively. CONCLUSION: In patients with stable coronary artery disease, prophylactic coronary revascularization before high-risk noncardiac surgery does not confer any beneficial effects, when compared with optimized medical management, in terms of perioperative mortality, myocardial infarction, long-term mortality, or adverse cardiac events.
