Malnutrition defined by GLIM criteria identifies a higher incidence of malnutrition and is associated with pulmonary complications after oesophagogastric cancer surgery, compared to ICD-10-defined malnutrition.

BACKGROUND & OBJECTIVES: Low muscle mass, measured using computed tomography (CT), is associated with poor surgical outcomes. We aimed to include CT-muscle mass in malnutrition diagnosis using the Global Leadership Initiative on Malnutrition (GLIM) criteria, compare it to the International Classification of Diseases 10th Revision (ICD-10) criteria, and assess the impact on postoperative outcomes after oesophagogastric (OG) cancer surgery. METHODS: One hundred and eight patients who underwent radical OG cancer surgery and had preoperative abdominal CT imaging were included. GLIM and ICD-10 malnutrition data were assessed against complication and survival outcomes. Low CT-muscle mass was determined using predefined cut-points. RESULTS: GLIM-defined malnutrition prevalence was significantly higher than ICD-10-malnutrition (72.2% vs. 40.7%, p < 0.001). Of the 78 patients with GLIM-defined malnutrition, low muscle mass (84.6%) was the predominant phenotypic criterion. GLIM-defined malnutrition was associated with pneumonia (26.9% vs. 6.7%, p = 0.010) and pleural effusions (12.8% vs. 0%, p = 0.029). Postoperative complications did not correlate with ICD-10 malnutrition. Severe GLIM (HR: 2.51, p = 0.014) and ICD-10 (HR: 2.15, p = 0.039) malnutrition were independently associated with poorer 5-year survival. CONCLUSIONS: GLIM criteria appear to identify more malnourished patients and more closely relate to surgical risk than ICD-10 malnutrition, likely due to incorporating objective muscle mass assessment.

Can we HALT obesity following lung transplant? A Dietitian- and Physiotherapy-directed pilot intervention.

INTRODUCTION: Unintentional weight gain, overweight and obesity following solid organ transplantation (SOT) are well-established and linked to morbidity and mortality risk factors. No interventional studies aimed at prevention have been undertaken among lung transplant (LTx) recipients. The combination of group education and telephone coaching is effective in the general population but is untested among SOT cohorts. METHODS: A non-randomized, interventional pilot study was conducted among new LTx recipients. The control group received standard care. In addition to standard care, the intervention involved four group education and four individual, telephone coaching sessions over 12-months. Data collection occurred at 2 weeks, 3- and 12 months post-LTx. Measurements included weight, BMI, fat mass (FM), fat mass index (FMI), fat-free mass (FFM), fat-free mass index (FFMI), waist circumference (WC), visceral adipose tissue (VAT), nutrition knowledge, diet, physical activity, lipid profile, HbA1C , FEV1 , six-minute walk distance and patient satisfaction. RESULTS: Fifteen LTx recipients were recruited into each group. One control participant died 120 days post-LTx, unrelated to the study. There were trends towards lower increases in weight (6.7±7.2 kg vs. 9.8±11.3 kg), BMI (9.6% of baseline vs. 13%), FM (19.7% vs. 40%), FMI, VAT (7.1% vs. 30.8%) and WC (5.5% vs. 9.5%), and greater increases in FFM and FFMI (all P > .05), among the intervention group by 12 months. The intervention was well-accepted by participants. CONCLUSION: This feasible intervention demonstrated non-significant, but clinically meaningful, favorable weight and body composition trends among LTx recipients over 12 months compared to standard care.

Probiotic use in adults with cystic fibrosis is common and influenced by gastrointestinal health needs: A cross-sectional survey study.

BACKGROUND: Cystic fibrosis (CF) primarily affects the lung, however, gastrointestinal disorders and symptoms, including dysbiosis, also impact on morbidity and quality of life. There is interest in strategies to modulate the gastrointestinal microbiota, including probiotics, although the evidence remains inadequate to guide practice, and information on use is limited. The present study aimed to characterise probiotic use, beliefs and experiences of adults with CF. METHODS: A cross-sectional questionnaire study was conducted in adults with CF (n = 205) and a general population Control group (n = 158), recruited from Victoria, Australia. Participants were classified as probiotic 'Ever Users' or 'Never Users'. Outcomes included self-reported probiotic use and factors associated with probiotic use, which were analysed using logistic regression analysis. Open-ended questionnaire responses were thematically analysed. RESULTS: In total, 70% of adults with CF had ever used probiotics (supplements and/or foods), comparable to Controls (80%) (p = 0.03). Key reasons for CF probiotic use were gastrointestinal- and antibiotic-related (75%). Most CF Ever Users (73%) did not discuss probiotic use with CF clinicians and 33% were uncertain if probiotics had been helpful. Female gender (odds ratio [OR] = 2.82; 95% confidence interval [CI] = 1.36-5.87; p = 0.005), university-level education (OR = 2.73; 95% CI = 1.24-6.01; p = 0.01) and bloating on antibiotics (OR = 2.14; 95% CI = 1.04-4.40; p = 0.04) were independently associated with probiotic use in CF; as was female gender in Controls (OR = 2.84; 95% CI = 1.20-6.71; p = 0.02). CONCLUSIONS: Probiotics were used by adults with CF for gastrointestinal- and antibiotic-related reasons often without informing clinicians and despite uncertainty about perceived helpfulness. Further research investigating gastrointestinal outcomes of probiotics will inform practice recommendations guiding their use in CF and other chronic diseases.

Probiotic knowledge of adults with cystic fibrosis is limited but is associated with probiotic use: A cross-sectional survey study.

Background: Probiotics are used by people with cystic fibrosis (CF) and other chronic diseases to manage gastrointestinal symptoms. Aim: To describe probiotic knowledge; its relationship with probiotic use, probiotic information sources and factors influencing choice in adults with CF and a general population control group. Methods: A cross-sectional questionnaire study was conducted in adults with CF (n = 205) and Controls (n = 158). Probiotic knowledge was compared between CF and Controls using a knowledge score (maximum 5) based on predefined criteria: (1a) bacteria/microorganism; (1b) live; (2a) administered; (2b) adequate dose and (3) health benefit, using independent samples t-test. Two-way analysis of variance explored knowledge scores between CF and Control and between Ever User and Never User groups. Chi-square and Fisher's exact tests compared knowledge criterion, probiotic sources and influences on probiotic choice between groups. Thematic analysis of open-text responses explored probiotic-related knowledge and influences on probiotic decision making. Results: Knowledge scores (mean ± SD) did not differ between CF (1.70 ± 1.12) and Controls (1.89 ± 0.99), p = 0.13. Probiotic use was associated with knowledge score (p < 0.001). More CF Ever Users than Never Users correctly identified criteria 1a (65% vs. 38%), 1b (16% vs. 0%), 2a (45% vs. 22%) and 3 (73% vs. 42%) (all p < 0.005). CF participants considered 'dairy yoghurt' (69%), 'live cultures' (64%) and 'fermented foods' (37%) as 'all/mostly' probiotic sources. The internet was the commonest source of probiotic-related information. Conclusion: Probiotic knowledge and use were associated in adults with CF. Understanding of probiotic characteristics and sources were limited. Education is needed to help guide patient probiotic decision making.

Redesign of the Australian Cystic Fibrosis Data Registry: A multidisciplinary collaboration.

Clinical registries that monitor and review outcomes for patients with cystic fibrosis have existed internationally for many decades. However, their purpose continues to evolve and now includes the capability to support clinical effectiveness research, clinical trials and Phase IV studies, and international data comparisons and projects. To achieve this, registries must regularly update the information that they collect and ensure design that is adaptable and flexible to changing needs. The Australian Cystic Fibrosis Data Registry commenced in 1998, and in 2018-19 undertook a transformation to enable it to meet the needs of multiple stakeholders into the future. This included a comprehensive, multidisciplinary review of the registry's data elements, and a redesign and rebuild of the registry's database. The data element review comprised the processes of alignment, comparison, selection, consolidation, revision and definition of finalised data elements. The database redesign included attention to each of the registry functions of data collection, storage and management, and reporting. The revision of a national data collection system is a time-intensive process, and requires significant clinical and other expert engagement. The resulting database, while being continually refined, is now fit for purpose to support Australian clinicians and patients with CF to receive best practice care.

Validity of multi-frequency bioelectric impedance methods to measure body composition in obese patients: a systematic review.

OBJECTIVE: Excessive lean tissue loss following bariatric surgery may pose serious metabolic consequences. Accurate methods to assess body composition following bariatric surgery are required. This review aimed to investigate if multi-frequency bioelectric impedance (MF-BI) is a valid tool to determine body composition in obese patients. METHODS: MEDLINE, EMBASE, CINAHL and CENTRAL databases were searched until March 2017. Included studies were published in English with obese (body mass index (BMI) ≥ 30 kg/m2) adults measuring body composition with MF-BI methods in comparison with reference methods. Exclusions were pregnancy, animal studies, non-English language studies, single frequency BI. A total of 6395 studies were retrieved. RESULTS: Sixteen studies were eligible for inclusion. Sample sizes ranged from 15 to 157, with BMI 26-48 kg/m2. MF-BI underestimated fat mass (FM) in 11 studies and overestimated fat-free mass (FFM) in nine studies in comparison with reference methods. Correlations of absolute values from MF-BI and reference methods for FM and FFM were high, however, agreement was lower at an individual level. When adjustments for BMI were made to machine algorithms, measurement accuracy improved. Significant heterogeneity was evident among included studies. CONCLUSIONS: This review found that MF-BI is reliable for use at a group level. Obese-specific adjustment of algorithms for MF-BI machines increases the accuracy of absolute measures of body composition in obese individuals, improving their utility in the clinical setting. Multiple variables contributed a lack of consistency among studies included, highlighting the need for more robust studies that control confounding variables to establish clear validity assessment.

The activin A antagonist follistatin inhibits cystic fibrosis-like lung inflammation and pathology.

Cystic fibrosis (CF) is the most common life-limiting genetically acquired respiratory disorder. Patients with CF have thick mucus obstructing the airways leading to recurrent infections, bronchiectasis and neutrophilic airway inflammation culminating in deteriorating lung function. Current management targets airway infection and mucus clearance, but despite recent advances in care, life expectancy is still only 40 years. We investigated whether activin A is elevated in CF lung disease and whether inhibiting activin A with its natural antagonist follistatin retards lung disease progression. We measured serum activin A levels, lung function and nutritional status in CF patients. We studied the effect of activin A on CF lung pathogenesis by treating newborn CF transgenic mice (ß-ENaC) intranasally with the natural activin A antagonist follistatin. Activin A levels were elevated in the serum of adult CF patients, and correlated inversely with lung function and body mass index. Follistatin treatment of newborn ß-ENaC mice, noted for respiratory pathology mimicking human CF, decreased the airway activin A levels and key features of CF lung disease including mucus hypersecretion, airway neutrophilia and levels of mediators that regulate inflammation and chemotaxis. Follistatin treatment also increased body weight and survival of ß-ENaC mice, with no evidence of local or systemic toxicity. Our findings demonstrate that activin A levels are elevated in CF and provide proof-of-concept for the use of the activin A antagonist, follistatin, as a therapeutic in the long-term management of lung disease in CF patients.

What do people with cystic fibrosis eat? Diet quality, macronutrient and micronutrient intakes (compared to recommended guidelines) in adults with cystic fibrosis-A systematic review.

BACKGROUND: Treatment advancements have improved life expectancy and nutritional status of people with cystic fibrosis (CF). Alongside reductions in malnutrition, incidences of overweight, obesity and risk factors for diet-related chronic diseases have increased in recent years. This study aimed to synthesise the available literature on diet quality, macronutrient and micronutrient intakes compared to the recommended guidelines in adults with CF, an essential step in deducing the optimal dietary pattern and intakes for CF adults. METHODS: A systematic search of five electronic databases from inception until April 2023 was conducted using keywords related to CF, diet quality and nutrient intakes. RESULTS: Twenty-one studies were included comprising 18 cross-sectional, one cohort and two case control studies, reporting data from 724 adults with CF. Energy and / or macronutrient intake data was reported across 17 cohorts, eight studies provided micronutrients data, and diet quality was determined for four CF cohorts by using a diet quality score, and / or categorising food intake into servings per day for food groups and comparing findings to national dietary guidelines. Although energy intake recommendations were met, and most micronutrient requirements were achieved through supplementation, total energy intake from fat was above recommendations and diet quality was poor. CONCLUSION: This is the first systematic review comprehensively evaluating literature on dietary intakes of adults with CF. Energy-dense, nutrient-poor foods contribute to intakes which pose risk in developing diet-related chronic diseases. Revision of dietary guidelines and practice change in CF nutritional therapy is warranted to optimise nutrition and health outcomes.

MERGANSER: an empirical model to predict fish and loon mercury in New England lakes.

MERGANSER (MERcury Geo-spatial AssessmeNtS for the New England Region) is an empirical least-squares multiple regression model using mercury (Hg) deposition and readily obtainable lake and watershed features to predict fish (fillet) and common loon (blood) Hg in New England lakes. We modeled lakes larger than 8 ha (4404 lakes), using 3470 fish (12 species) and 253 loon Hg concentrations from 420 lakes. MERGANSER predictor variables included Hg deposition, watershed alkalinity, percent wetlands, percent forest canopy, percent agriculture, drainage area, population density, mean annual air temperature, and watershed slope. The model returns fish or loon Hg for user-entered species and fish length. MERGANSER explained 63% of the variance in fish and loon Hg concentrations. MERGANSER predicted that 32-cm smallmouth bass had a median Hg concentration of 0.53 µg g(-1) (root-mean-square error 0.27 µg g(-1)) and exceeded EPA's recommended fish Hg criterion of 0.3 µg g(-1) in 90% of New England lakes. Common loon had a median Hg concentration of 1.07 µg g(-1) and was in the moderate or higher risk category of >1 µg g(-1) Hg in 58% of New England lakes. MERGANSER can be applied to target fish advisories to specific unmonitored lakes, and for scenario evaluation, such as the effect of changes in Hg deposition, land use, or warmer climate on fish and loon mercury.

Integrating mercury science and policy in the marine context: challenges and opportunities.

Mercury is a global pollutant and presents policy challenges at local, regional, and global scales. Mercury poses risks to the health of people, fish, and wildlife exposed to elevated levels of mercury, most commonly from the consumption of methylmercury in marine and estuarine fish. The patchwork of current mercury abatement efforts limits the effectiveness of national and multi-national policies. This paper provides an overview of the major policy challenges and opportunities related to mercury in coastal and marine environments, and highlights science and policy linkages of the past several decades. The U.S. policy examples explored here point to the need for a full life cycle approach to mercury policy with a focus on source reduction and increased attention to: (1) the transboundary movement of mercury in air, water, and biota; (2) the coordination of policy efforts across multiple environmental media; (3) the cross-cutting issues related to pollutant interactions, mitigation of legacy sources, and adaptation to elevated mercury via improved communication efforts; and (4) the integration of recent research on human and ecological health effects into benefits analyses for regulatory purposes. Stronger science and policy integration will benefit national and international efforts to prevent, control, and minimize exposure to methylmercury.

Survival of people with cystic fibrosis in Australia.

Survival statistics, estimated using data from national cystic fibrosis (CF) registries, inform the CF community and monitor disease progression. This study aimed to estimate survival among people with CF in Australia and to identify factors associated with survival. This population-based cohort study used prospectively collected data from 23 Australian CF centres participating in the Australian CF Data Registry (ACFDR) from 2005-2020. Period survival analysis was used to calculate median age of survival estimates for each 5-year window from 2005-2009 until 2016-2020. The overall median survival was estimated using the Kaplan-Meier method. Between 2005-2020 the ACFDR followed 4,601 people with CF, noting 516 (11.2%) deaths including 195 following lung transplantation. Out of the total sample, more than half (52.5%) were male and 395 (8.6%) had undergone lung transplantation. Two thirds of people with CF (66.1%) were diagnosed before six weeks of age or by newborn/prenatal screening. The overall median age of survival was estimated as 54.0 years (95% CI: 51.0-57.04). Estimated median survival increased from 48.9 years (95% CI: 44.7-53.5) for people with CF born in 2005-2009, to 56.3 years (95% CI: 51.2-60.4) for those born in 2016-2020. Factors independently associated with reduced survival include receiving a lung transplant, having low FEV1pp and BMI. Median survival estimates are increasing in CF in Australia. This likely reflects multiple factors, including newborn screening, improvement in diagnosis, refinements in CF management and centre-based multidisciplinary care.

A GLIMmer of insight into lung transplant nutrition: Enhanced detection of malnutrition in lung transplant patients using the GLIM criteria.

BACKGROUND & AIMS: The Global Leadership Initiative on Malnutrition (GLIM) is a novel framework for diagnosing malnutrition and requires evaluation in wide-ranging clinical settings. This study aimed to assess the prevalence of malnutrition and its phenotypic characteristics among lung transplantation (LTx) candidates comparing GLIM to International Classification of Diseases, 10th Revision (ICD-10) criteria. METHODS: A retrospective analysis was conducted of all adult patients assessed for LTx in a one-year period. Phenotypic criteria included body mass index (BMI), unintentional loss of weight (LOW) over a 12-month period and fat-free mass index (FFMI) using bioelectrical impedance analysis (BIA). Systemic inflammation associated with severe end-stage lung disease met GLIM's etiological criterion. Diagnosis of malnutrition, and its severity, were classified according to each of GLIM and ICD-10. RESULTS: Of 130 patients, 112 (86%) had all data to classify malnutrition. Malnutrition prevalence according to GLIM was 59%, which was markedly greater than using ICD-10 criteria (26%). Half of the LTx patients were moderately malnourished using GLIM, compared to 19% using ICD-10. A similar proportion were severely malnourished using GLIM (9%) and ICD-10 (7%). Fat-free mass (FFM) depletion (47% of all patients) was a major contributor to GLIM-malnutrition. Over 60% of LTx patients with GLIM-malnutrition were not detected as malnourished using ICD-10 criteria. CONCLUSION: Malnutrition diagnosis using GLIM was higher than using ICD-10 in LTx patients, primarily attributable to the incorporation of quantitative evaluation of FFM depletion. This highlights the utility of the GLIM framework and the importance of including body composition in malnutrition assessment.

Marked losses of computed tomography-derived skeletal muscle area and density over the first month of a critical illness are not associated with energy and protein delivery.

OBJECTIVES: Changes in muscularity during different phases of critical illness are not well described. This retrospective study aimed to describe changes in computed tomography (CT)-derived skeletal muscle area (SMA) and density (SMD) across different weeks of critical illness and investigate associations between changes in these parameters and energy and protein delivery. METHODS: Thirty-two adults admitted to the intensive care unit (ICU) who had ≥2 CT scans at the third lumbar area performed ≥7 d apart were included in the study. CT-derived SMA (cm2) and SMD (Hounsfield units) were determined using specialized software. A range of clinical and nutrition variables were collected for each day between comparator scans. Associations were assessed by Pearson or Spearman correlations. RESULTS: There was a significant decrease in SMA between the two comparator scans where the first CT scan was performed in ICU wk 1 (n = 20; P < .001), wk 2 (n = 11; P < .007), and wk 3 to 4 (n = 7; P = .012). There was no significant change in SMA beyond ICU wk 5 to 7 (P = .943). A significant decline in SMD was observed across the first 3 wk of ICU admission (P < .001). Overall, patients received a mean 24 ± 6 kcal energy/kg and 1.1 ± 0.4 g protein/kg per study day and 83% of energy and protein requirements according to dietitian estimates. No association between SMA or SMD changes and nutrition delivery were found. CONCLUSIONS: Critically ill patients experience marked losses of SMA over the first month of critical illness, attenuated after wk 5 to 7. Energy and protein delivery were not associated with degree of muscle loss.

Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label extension.

OBJECTIVES: In patients with cystic fibrosis (CF) who carry the G551D mutation, treatment with ivacaftor improves lung function and weight; however, short- and long-term impacts on body composition have not been well studied. METHODS: Twenty adults with CF carrying the G551D mutation (mean ± standard deviation body mass index [BMI] 23.3 ± 4.3 kg/m2) were recruited for a single-center, double-blind, placebo-controlled, 28-d, crossover study of ivacaftor, followed by an open-label extension (OLE) for 5 mo. Eleven patients underwent measurements 2 y later. The study variables included weight, BMI, and body composition (including fat-free mass [FFM] and fat mass). RESULTS: After 28 d of treatment with ivacaftor, weight increased by 1.1 ± 1.3 kg, BMI by 0.4 ± 0.5 kg/m2, and FFM by 1.1 ± 1.2 kg (all P < .005) with no change in fat mass. Differences between 28-d changes on ivacaftor and placebo were not statistically significant. In the following 5 mo of the OLE, there were significant increases in weight (1.2 ± 1.9 kg; P < .05) and fat mass (1.5 ± 1.9 kg; P < .01), but not in FFM. Between baseline and the end of the OLE, the total weight gain was 2.5 ± 2.4 kg (P < .005), comprised of 0.9 ± 1.5 kg FFM (P < .05) and 1.6 ± 1.8 kg fat mass (P < .005). For the 11 participants who were followed for a further 2 y, no further changes in mean weight, BMI, or body composition parameters between 6 mo and 2 y later were observed. CONCLUSIONS: Small gains were seen in FFM in the first month of ivacaftor treatment. Weight, BMI, and fat-mass gains in the first 6 mo on ivacaftor plateaued by 2.5 y. The metabolic and clinical consequences of weight and fat-mass gains remain to be determined.

Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis.

INTRODUCTION: Lumacaftor/ivacaftor (LUM/IVA) has been shown to improve clinical outcomes in cystic fibrosis (CF) patients homozygous for Phe508del with forced expiratory volume in 1 s (FEV1) % pred >40%. We assessed the clinical utility of LUM/IVA in all eligible adult CF patients with FEV1 % pred <40% treated for at least 1 year under a single-centre managed access programme. METHODS: Following clinical optimisation, eligible patients (n=40) with FEV1 % pred <40% were commenced on LUM/IVA and monitored for tolerance and clinical outcomes, including health service utilisation, pulmonary function, weight and body composition. 24 patients reached 1 year of treatment by the time of evaluation. Six patients discontinued due to adverse events (five for increased airways reactivity) and three underwent lung transplantation. RESULTS: In comparison with the year prior to LUM/IVA commencement, significant reductions (median per year) were observed in the treatment year in the number of pulmonary exacerbations requiring hospitalisation (from 3 to 1.5; p=0.0002), hospitalisation days (from 27 to 17; p=0.0002) and intravenous antibiotic (IVAB) usage days (from 45 to 27; p=0.0007). Mean±sd change in FEV1 % pred was -2.10±1.18% per year in the year prior, with the decline reversed in the year following (+1.45±1.13% per year; p=0.035), although there was significant heterogeneity in individual responses. Mean±sd weight gain at 1 year was 2.5±4.1 kg (p=0.0007), comprising mainly fat mass (mean 2.2 kg). The proportion of patients severely underweight (body mass index <18.5 kg·m-2) decreased from 33% at baseline to 13% at 1 year (p=0.003). CONCLUSION: This real-world evaluation study demonstrated benefits over several clinical domains (infective exacerbations requiring hospitalisation, IVABs, pulmonary function decline and nutritional parameters) in CF patients with severe lung disease.

The AWESCORE, a patient-reported outcome measure: development, feasibility, reliability, validity and responsiveness for adults with cystic fibrosis.

BACKGROUND: Quality of life has improved dramatically over the past two decades in people with cystic fibrosis (CF). Quantification has been enabled by patient-reported outcome measures (PROMs); however, many are lengthy and can be challenging to use in routine clinical practice. We propose a short-form PROM that correlates well with established quality-of-life measures. METHODS: We evaluated the utility of a 10-item score (AWESCORE) by measuring reliability, validity and responsiveness in adults with CF. The questions were developed by thematic analysis of survey questions to patients in a single adult CF centre. Each question was scored using a numerical rating scale 0 to 10. Total scores ranged from 0 to 100. Test-retest reliability was assessed over 24 h. To determine validity, comparisons were sought between stable subjects and those in pulmonary exacerbation, and between AWESCORE and Cystic Fibrosis Questionnaire - Revised (CFQ-R). Responsiveness to pulmonary exacerbation in individual subjects was evaluated. RESULTS: Five domains, each with two questions, were identified for respiratory, physical, nutritional, psychological and general health. A total of 246 consecutive adults attending the outpatient clinic completed the AWESCORE. Scores were higher during clinical stability compared to pulmonary exacerbation (mean± sd): 73±11 versus 48±11 (p<0.001). Each domain scored worse during an acute exacerbation (p<0.001). No differences in reliability were observed in scores on retesting using Bland-Altman comparison. The CFQ-R scores (mean±sd: 813±125) and AWESCORE (81±13) were moderately correlated (Pearson's r=0.649; p=0.002). CONCLUSIONS: The AWESCORE is valid, reliable and responsive to altered health status in CF.

Comparison of Ultrasound-Derived Muscle Thickness With Computed Tomography Muscle Cross-Sectional Area on Admission to the Intensive Care Unit: A Pilot Cross-Sectional Study.

INTRODUCTION: The development of bedside methods to assess muscularity is an essential critical care nutrition research priority. We aimed to compare ultrasound-derived muscle thickness at 5 landmarks with computed tomography (CT) muscle area at intensive care unit (ICU) admission. Secondary aims were to (1) combine muscle thicknesses and baseline covariates to evaluate correlation with CT muscle area and (2) assess the ability of the best-performing ultrasound model to identify patients with low CT muscle area. METHODS: Adult patients who underwent CT scanning at the third lumbar area <72 hours after ICU admission were prospectively recruited. Muscle thickness was measured at mid-upper arm, forearm, abdomen, and thighs. Low CT muscle area was determined using published cutoffs. Pearson correlation compared ultrasound-derived muscle thickness and CT muscle area. Linear regression was used to develop ultrasound prediction models. Bland-Altman analyses compared ultrasound-predicted and CT-measured muscle area. RESULTS: Fifty ICU patients were enrolled, aged 52 ± 20 years. Ultrasound-derived muscle thickness at each landmark correlated with CT muscle area (P < .001). The sum of muscle thickness at mid-upper arm and bilateral thighs, including age, sex, and the Charlson Comorbidity Index, improved the correlation with CT muscle area (r = 0.85; P < .001). Mean difference between ultrasound-predicted and CT-measured muscle area was -2 cm2 (95% limits of agreement, -40 cm2 to +36 cm2 ). The best-performing ultrasound model demonstrated good ability to identify 14 patients with low CT muscle area (area under curve = 0.79). CONCLUSION: Ultrasound shows potential for assessing muscularity at ICU admission (Clinicaltrials.gov NCT03019913).

Utility of bioimpedance methods for the assessment of fat-free mass in adult outpatients with inflammatory bowel disease.

OBJECTIVES: Clinically accessible body composition assessment is required to identify fat-free mass (FFM) depletion, which is common in inflammatory bowel disease (IBD) and poorly correlated with body mass index (BMI). The aim of this study was to compare FFM assessed using bioimpedance spectroscopy (BIS) and multifrequency bioelectrical impedance analysis (MFBIA) with dual energy x-ray absorptiometry (DXA) in adult outpatients with IBD. METHODS: FFM was measured using DXA, BIS, and MFBIA in 40 outpatients with IBD. Paired t tests, Lin and Pearson's correlations, and limits of agreement (LOA) analysis were used to compare FFMDXA with FFMBIS and FFMMFBIA. RESULTS: Participants (26 men, 24 with Crohn's disease) had a median (IQR) age 39 y (32-50 y) and median (IQR) BMI 24.2 kg/m2 (21.9-26.4 kg/m2). Mean FFMDXA was 51.6 ± 12 kg. FFMBIS was highly correlated with FFMDXA (rp = 0.97; P < 0.001); however, BIS significantly overestimated FFM compared with DXA by a mean 3.4 ± 2.6 kg (P < 0.001) and showed wide 95% LOA (-1.7 to 8.4 kg). FFMBIS estimations improved when FFM was adjusted for by BMI using Moissl's predictive algorithm, reducing mean bias to 0.1 ± 2 kg (P = 0.858; 95% LOA -3.9 to 4 kg). The bias between FFMMFBIA and FFMDXA was 1.3 ± 2 kg (P = 0.002) with 95% LOA -2.6 to 5 kg (n = 28). CONCLUSION: Unadjusted BIS overestimated FFM in IBD outpatients compared with DXA with poor agreement at an individual level. Adjusting for BMI ameliorated the overestimation. It is suggested for the estimation of FFM in outpatients with IBD that MFBIA or the Moissl algorithm with BIS be used if DXA is unavailable.

Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand.

Optimal nutrition care is important in the management of cystic fibrosis (CF). This paper summarises the '2017 Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand (NZ)'. CF dietitians formulated 68 practice questions which were used to guide a systematic literature search and review of the evidence for nutrition in CF. Identified papers underwent quality and evidence assessment using the American Dietetic Association quality criteria checklist and the National Health and Medical Research Council of Australia (NHMRC) rankings. Evidence statements, graded recommendations and practice points were developed covering core nutrition topics (assessment and nutrition interventions including oral, enteral and micronutrient supplementation); nutrition-related co-morbidities (including pancreatic insufficiency, CF-related diabetes, bone health and distal intestinal obstruction syndrome); and key new topic areas (genetic modulator therapies, overweight/obesity and complementary therapies). This paper showcases highlights from the guidelines, focussing on new topic areas and geographic and climate considerations for vitamin D, salt and hydration.