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Background and Objectives: Cystic fibrosis (CF) screen-positive infants with an inconclusive diagnosis (CFSPID) are infants in whom sweat testing and genetic analysis does not resolve a CF diagnosis. Lack of knowledge about the health outcome of these children who require clinical follow-up challenges effective consultation. Early predictive biomarkers to delineate the CF risk would allow a more targeted approach to these children., Methods: Prospective, longitudinal, multicenter, Canada-wide cohort study of CF positive-screened newborns with 1 to 2 cystic fibrosis transmembrane conductance regulator gene variants, of which at least 1 is not known to be CF-causing and/or a sweat chloride between 30 and 59 mmol/L. These were monitored for conversion to a CF diagnosis, pulmonary, and nutritional outcomes., Results: The mean observation period was 7.7 (95% confidence interval 7.1 to 8.4) years. A CF diagnosis was established for 24 of the 115 children with CFSPID (21%) either because of reinterpretation of the cystic fibrosis transmembrane conductance regulator genotype or because of increase in sweat chloride concentration ≥60 mmol/L. An initial sweat chloride of ≥40 mmol/l predicted conversion to CF on the basis of sweat testing. The 91 remaining children with CFSPID were pancreatic sufficient and showed normal growth until school age. Pulmonary function as well as lung clearance index in a subgroup of children with CFSPID were similar to that of healthy controls., Conclusions: Children with CFSPID have good nutritional and pulmonary outcomes at school age, but rates of reclassifying the diagnosis are high. The initial sweat chloride test can be used as a biomarker to predict the risk for CF in CFSPID., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Purpose: Cystic fibrosis (CF), caused by pathogenic variants in the CF transmembrane conductance regulator (CFTR), affects multiple organs including the exocrine pancreas, which is a causal contributor to cystic fibrosis-related diabetes (CFRD). Untreated CFRD causes increased CF-related mortality whereas early detection can improve outcomes., Methods: Using genetic and easily accessible clinical measures available at birth, we constructed a CFRD prediction model using the Canadian CF Gene Modifier Study (CGS; n = 1,958) and validated it in the French CF Gene Modifier Study (FGMS; n = 1,003). We investigated genetic variants shown to associate with CF disease severity across multiple organs in genome-wide association studies., Results: The strongest predictors included sex, CFTR severity score, and several genetic variants including one annotated to PRSS1, which encodes cationic trypsinogen. The final model defined in the CGS shows excellent agreement when validated on the FGMS, and the risk classifier shows slightly better performance at predicting CFRD risk later in life in both studies., Conclusion: We demonstrated clinical utility by comparing CFRD prevalence rates between the top 10% of individuals with the highest risk and the bottom 10% with the lowest risk. A web-based application was developed to provide practitioners with patient-specific CFRD risk to guide CFRD monitoring and treatment.

In view of market-driven health-care policies and the move to greater efficiencies within the health-care system, the cost of nursing care is being increasingly scrutinised. Different overseas practices are commonly cited as justification for changing practices within Australia. This study is based on a review of the literature on intensive care nurse staffing requirements in Australasia; specifically, New South Wales, the United States (US) and, to a lesser extent, Europe. It was found that looking to the US for cost-cutting strategies in intensive care units (ICUs) is based on a false premise: that we are comparing like with like. ICUs in the US have a different historical trajectory and culture, service wider constituencies, have technicians and unregistered personnel providing nursing care and do not provide demonstrably better outcomes or significant cost savings. Research indicates that continuous nursing care by trained professionals provides the best outcomes. If costs must be cut, technology, pharmaceuticals and laboratory tests should be targeted. Further, a greater commitment to the development of a 'progressive patient care' model in hospital planning is required, in order to establish or consolidate an intermediate level of nursing care between the ward and the ICU. Programs aiming to improve and continuously monitor patient care, such as adverse event monitoring, the prevention of unplanned extubation and facilitation of early extubation, should be instituted, as these have been shown to not only reduce ICU costs but also improve patient outcomes. (c) 2000 Harcourt Publishers Ltd.

In view of market-driven health-care policies and the move to greater efficiencies within the health-care system, the cost of nursing care is being increasingly scrutinised. Different overseas practices are commonly cited as justification for changing practices within Australia. This study is based on a review of the literature on intensive care nurse staffing requirements in Australasia; specifically, New South Wales, the United States (US) and, to a lesser extent, Europe. It was found that looking to the US for cost-cutting strategies in intensive care units (ICUs) is based on a false premise: that we are comparing like with like. ICUs in the US have a different historical trajectory and culture, service wider constituencies, have technicians and unregistered personnel providing nursing care and do not provide demonstrably better outcomes or significant cost savings. Research indicates that continuous nursing care by trained professionals provides the best outcomes. If costs must be cut, technology, pharmaceuticals and laboratory tests should be targeted. Further, a greater commitment to the development of a 'progressive patient care' model in hospital planning is required, in order to establish or consolidate an intermediate level of nursing care between the ward and the ICU. Programs aiming to improve and continuously monitor patient care, such as adverse event monitoring, the prevention of unplanned extubation and facilitation of early extubation, should be instituted, as these have been shown to not only reduce ICU costs but also improve patient outcomes.