BACKGROUND:The European Cystic Fibrosis Society (ECFS) develops education resources to support members; however, these are almost exclusively in English. Many barriers to translation exist, including cost and time. Artificial intelligence (AI) provides an opportunity to support translation and address such barriers. This study aimed to pilot the use of AI-generated translation of ECFS e-learning modules and evaluate the quality. METHODS:An AI translation program was used to create subtitles of ECFS peer-reviewed education modules. Two independent native language speakers with extensive cystic fibrosis (CF) healthcare experience were identified and tasked with reviewing, editing, and validating. This was followed by the development and circulation of an online evaluation survey assessing users' views on quality. RESULTS:Education packages, each consisting of six subtitled modules, were created in three languages: Ukrainian, Romanian, and Turkish. For each language, corrections to the AI-generated translation by the independent native speakers were essential. Evaluation was conducted in two countries. Eighteen completed surveys were received. Results indicated high levels of accuracy for the final modules, and feedback was very positive regarding the utility and range of topics. CONCLUSION:The use of novel AI-generated translation shows promise and proved quick and affordable. However, quality of translation was variable, highlighting the critical role of collaborating with native-speaking CF experts to ensure linguistic accuracy. This project highlights the importance of interdisciplinary collaborative efforts between ECFS Education, the Twinning Project, CF Europe, and patient organizations. Further, it demonstrates both the feasibility and practicality of generating effective multilingual educational modules using AI.

Introduction The introduction of CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ETI) has significantly altered the nutritional trajectory of children with cystic fibrosis (cwCF). While most eligible children in high-resource countries have commenced ETI, the rollout has outpaced the development of nutritional guidance. This study aimed to: (1) systematically review the literature on ETI and nutrition (2) develop expert consensus-based statements and (3) highlight existing research gaps and priorities. Methods A systematic review and guideline development process was registered on PROSPERO (ID: CRD42024587618). An expert panel of 22 professionals, including dietitians, gastroenterologists, respirologists, endocrinologists, and a parent representative was convened with attention to global representation. Research questions were developed using the Population, Intervention, Comparison, Outcome (PICO) framework. A comprehensive literature search was conducted across Medline, Embase, CINAHL, and Cochrane (January 2010 to April 2025). Studies were independently screened, assessed for quality using Scottish Intercollegiate Guidelines Network (SIGN) criteria, and extracted into evidence tables. Draft statements were developed and refined through iterative feedback and online discussion. Final consensus was achieved using real-time Delphi, with ≥80% agreement required for endorsement. Reporting followed Accurate Consensus Reporting Document (ACCORD) guidelines. Results 67 consensus-based statements were developed. The top research priorities were identified based on the greatest proportion of “strongly agree” responses. Conclusion ETI has altered nutritional requirements in cwCF, yet evidence remains limited. This study presents the first consensus-based guidance for the nutritional care of cwCF receiving ETI, providing interim, expert informed recommendations and highlighting research priorities, particularly the need to re-evaluate traditional BMI targets.

Significant disparities in cystic fibrosis (CF) care persist across Europe. To address these inequalities, the European CF Society (ECFS) has launched the Twinning Project in collaboration with CF Europe. The project promotes structured partnerships between well-established and developing CF centres. Mentor centres from the ECFS Clinical Trials Network (ECFS-CTN) are paired with mentee centres, primarily from Eastern European countries, with the aim of strengthening CF care. Launched in 2020 with eight partnerships, the programme has grown in 2024 to encompass an additional 19 twinning pairs and national patient organisations. Twinning activities include onsite visits, virtual meetings, educational initiatives and translation of learning resources into local languages. Programme evaluation is based on annual progress reports assessing collaboration, training, multidisciplinary team development at the mentee site, and the frequency and mode of communication. Of the 25 twinning pairs that submitted their reports in March 2025, 24 had established regular online communication and 19 had conducted at least one onsite visit. Based on reports received from 23 pairs in March 2026, we identified that the proportion of pairs completing reciprocal onsite visits rose from 24% (6/25) in 2025 to 65% (15/23) in 2026, and the proportion reporting regular clinical case consultations increased from 24% (6/25) to 83% (19/23). One mentee centre was approved as a new ECFS-CTN site. As of 2026, the network comprised 26 mentor centres and 28 mentee sites. The Twinning Project has fostered sustainable collaborations between CF centres and patient communities, contributing to the harmonisation of CF standards across Europe.

BACKGROUND:Advancements in clinical care and scientific research in cystic fibrosis (CF) have transformed, and continue to shape, nutritional management and related practices. Front line clinicians delivering nutritional support require 'living guidelines' with regular updates to address emerging issues in the rapidly changing world of CF. In this paper we wish to provide context and framework recommendations on key issues that are becoming increasingly important in clinical practice where current international nutrition guidelines provide limited or no recommendations. METHODS:The study was developed by an international multidisciplinary working group made up of previous authors of the ESPEN-ESPGHAN-ECFS 2024 guidelines. Key topics were identified and new internationally recognised expert authors were allocated to provide additional expertise as required. Statements were produced and a modified Delphi was used to gain consensus. RESULTS:The working group developed recommendations to complement current guidelines on the quality of diet, the changes in pancreatic enzyme supplementation in the age of modulator therapy, evaluation and treatment of overweight and obesity, withdrawing tube feeding, nutritional support in cancer and nutrition care in older adults. These were endorsed by the ECFS Nutrition Group. The topics identified and examined in this paper represent issues that will need to be addressed in the next revision of the ESPEN-ESPGHAN-ECFS guideline. This paper therefore serves as a preliminary step in preparation for this update.

Introduction Outcomes for people with the inherited disease, cystic fibrosis, have improved greatly over the last few decades, but one result of this is a widening gap between regions with high income and well-resourced healthcare systems and low/middle income countries. The gap stretches from newborn screening programs, provision of standard diagnostics and genetic testing through to access to standard of care therapies.Methods and Results This paper describes the various initiatives of the European Cystic Fibrosis Society: our Patient Registry, a Twinning Program linking centers from different regions and our Educational Program.Conclusions The European Cystic Fibrosis Society recognizes this as a major issue and seeks through these programs to support colleagues, patients and families in low/middle income countries.

Background:Children with extreme short bowel syndrome, including those who have undergone resection of the entire jejunum and ileum-termed "No Gut Syndrome" (NGS) in the adult literature-are now surviving due to advancements in parenteral nutrition (PN) and medical care. This study aims to describe the population characteristics and long-term outcomes of children with NGS. Material and methods:A questionnaire was distributed to the paediatric intestinal failure (IF) centres in the UK to identify patients with NGS discharged home on PN. Inclusion criteria: infants/children < 18 years with duodenum plus up to 5 cm jejunum and colon (total/partial). Follow-up data was collected two years after the initial recruitment. A multicentre Health Research Authority approval was obtained. Results:19/20 (95 %) IF centres managing 379 HPN patients responded - 27/379 (7 %) had ultrashort-bowel syndrome (U-SBS) and an additional 14/379 (4 %) NGS. Aetiology of NGS was - midgut volvulus (64 %), bowel ischaemia (22 %), necrotising enterocolitis (7 %), jejuno-ileal atresia (7 %).Nine patients had a colonic remnant in continuity. Thirteen/14 patients (93 %) remained stable on PN with one weaned off following small bowel transplant. Nine/13 (69 %) patients received > 80 % of EAR as PN and 4/13 (31 %), 30-80 %. Eight/13 (61 %) tolerated some form of enteral nutrition. There were no deaths during the follow-up period. Educational outcomes were positive showing that 11/14 (79 %) are attending school, 2/14 (14 %) nurseries with 1 awaiting enrollment (7 %). Conclusion:Advances in PN and health care have improved the long-term survival of patients with NGS. In addition, these children are attending school and participating in daily activities without significant limitation.

Prader-Willi syndrome (PWS) is a rare orphan disease and complex genetic neurodevelopmental disorder, with a birth incidence of approximately 1 in 10,000-30,000. Management of people with PWS requires a multi-disciplinary approach, ideally through a multi-disciplinary team (MDT) clinic with community support. Hypotonia, poor feeding and faltering growth are characteristic features in the neonatal period, followed by hyperphagia and risk of rapid weight gain later in childhood. Children and adolescents (CA) with PWS usually display developmental delay and mild learning disability and can develop endocrinopathies, scoliosis, respiratory difficulties (both central and obstructive sleep apnoea), challenging behaviours, skin picking, and mental health issues, especially into adulthood. This consensus statement is intended to be a reference document for clinicians managing children and adolescents (up to 18 years of age) with PWS. It considers the bio-psycho-social domains of diagnosis, clinical assessment, and management in the paediatric setting as well as during and after transition to adult services. The guidance has been developed from information gathered from peer-reviewed scientific reports and from the expertise of a range of experienced clinicians in the United Kingdom and Ireland involved in the care of patients with PWS.

Nutrition has played a central role in the management and outcomes of people with cystic fibrosis (pwCF) since the 1970s. Advances in therapies and practices in recent decades have led to a significant change in the patient landscape with dramatic improvements in life expectancy, as well as quality of life, bringing with it new issues. Historically, cystic fibrosis was a condition associated with childhood and malnutrition; however, changes in patient demographics, nutritional assessment and fundamental nutritional management have evolved, and it has become an increasingly prevalent adult disease with new nutritional challenges, including obesity. This paper aims to describe these changes and the impact and challenges they bring for those working in this field. Nutritional professionals will need to evolve, adapt and remain agile to the wider range of situations and support required for a new generation of pwCF. Specialised nutrition support will continue to be required, and it will be additionally important to improve and optimise quality of life and long-term health. Historically, cystic fibrosis was a condition associated with childhood and malnutrition; however, changes in patient demographics, nutritional assessment and fundamental nutritional management have evolved, and new nutritional challenges are emerging. This paper aims to describe these changes and the impact and challenges they bring for those working in this field. image Traditional cystic fibrosis dietary advice focused on a default high-fat/high-calorie intake with limited consideration for other important nutrients. The emerging new era approach should be individualised, flexible and with an emphasis on diversity and longevity. Historically, this population was predominantly undernourished or at risk of undernutrition, but with the success and impact of nutritional guidelines, multidisciplinary teams, screening and new therapies, overnutrition is becoming an increasing concern that requires attention. Significant improvements in life expectancy mean more people with cystic fibrosis are living into old age, bringing with it age-related complications and new opportunities that require nutritional consideration from childhood.

BackgroundInterest and use of blended diets (BD) for young people who are tube fed has significantly increased in the last decade, driven primarily by the desires of motivated caregivers. This review identified, appraised and synthesised the available evidence on the benefits and complications of BD versus commercial feeds. MethodsA systematic review following PRISMA guidance and registered with PROSPERO was conducted across PubMed, Embase, CINAHL, Scopus and Cochrane up to August 2022. Inclusion criteria: English language studies including (1) children, (2) original research (interventional and observational) and (3) examination of BD outcomes. Exclusion criteria were (1) unoriginal research or case reports, (2) focus on feeding management, preparations or attitudes and (3) comparing commercial blends only. Data were synthesised using an established narrative synthesis approach using the Mixed Methods Appraisal Tool. ResultsEight hundred and six database results were identified and 61 were sought for retrieval. A full-text article review revealed seven eligible studies, involving 267 participants (age range 9 months to 26 years). Studies reported differences in gastrointestinal symptoms (n = 222), medication use (n = 119), growth (n = 189) and complications or adverse events (n = 91). The results indicate positive outcomes, particularly in gastrointestinal symptom control, with few reports of mild adverse events in the included studies. ConclusionsThere is a paucity of data in this area and much heterogeneity in the included studies, but the available literature points towards positive outcomes. This is an important and highly relevant topic, and more primary research, ideally using standardised reporting, is required to answer the key questions.

This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy.

This systematic review summarises evidence regarding oral nutritional supplement (ONS) use in children with, or at risk of, faltering growth (FG). Ten randomised controlled trials (RCTs), compared changes in outcomes amongst children receiving ONS versus control were included. Overall, 1116 children (weighted mean (WM) age 5 years; n658 (59%) male) were recruited, of which 585 (52%) received ONS (WM intake contribution 412 kcal, 16.3 g protein, 395 ml) for 116 days (WM). ONS use was associated with significantly greater gains in weight (mean difference (MD) 0.4 kg, 95% CI [0.36, 0.44]) and height (MD 0.3 cm, 95% CI [0.03, 0.57]), likely related to improvements in nutritional intake. Mean compliance to prescribed dose was 98%. Data suggested an association between ONS use and reduced infections. Further research is warranted to establish ONS dosage and effects upon other outcomes. This review provides evidence to support use of ONS in the management of children with, or at risk of, FG.

BACKGROUND:Nutritional status is paramount in Cystic Fibrosis (CF) and is directly correlated with morbidity and mortality. The first ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with CF were published in 2016. An update to these guidelines is presented. METHODS:The study was developed by an international multidisciplinary working group in accordance with officially accepted standards. Literature since 2016 was reviewed, PICO questions were discussed and the GRADE system was utilized. Statements were discussed and submitted for on-line voting by the Working Group and by all ESPEN members. RESULTS:The Working Group updated the nutritional guidelines including assessment and management at all ages. Supplementation of vitamins and pancreatic enzymes remains largely the same. There are expanded chapters on pregnancy, CF-related liver disease, and CF-related diabetes, bone disease, nutritional and mineral supplements, and probiotics. There are new chapters on nutrition with highly effective modulator therapies and nutrition after organ transplantation.

Objectives Cystic fibrosis (CF) is an inherited, life-shortening multi-systemic disease.1Patients present predominantly through the UK screening program, but a smaller population initially present as meconium ileus (MI).1 Growth outcomes in infancy, specifically growth achieved at 1 year is an important marker for later lung function and other health markers.2 The primary outcomes were to evaluate the prevalence of re-achieving birth centile by age 1 and the incidence of growth failure (defined by NICE guidelines) of infants managed in a single UK CF Centre. The secondary outcomes were to investigate the growth patterns and outcomes in the two presentation groups (screening vs MI) and in those exclusively breastfed. Methods Data was extracted from a dedicated CF database and medical notes for growth charts and additional clinical information. CF patients born between 2011 and 2021 were selected for inclusion. Anthropometric measurements (centiles, Z-scores) and methods of feeding were recorded three-monthly during the first year of life, and when available at 24 months. Results were then tabulated and statistics were run using SPSS (version 28.0.1.1 15). Results 23 patients were identified. Overall, 73% of patients re-achieved birth centiles at 1 year. There was a prevalence of 39% growth failure. 62.5% of all patients with growth failure re-achieved their birth centiles at 1 year. 15 patients presented through screening, 8 presented with MI. 67% of those with growth failure were MI. Patients presenting with MI were born heavier (Z-score-weight=-0.02±0.66) and longer (Z-score-lenght=2.51±1.31) than patients presenting through screening (Z-score-weight=-0.5±1.05; Z-score-length=1.13±1.43). All patients Z-score-weight declined by 3 months but the decline for MI patients was sharper as seen in figure 1. By 12 months, there was no statistically significant difference in the mean Z-score for weight between the MI (0.40±0.59) and the screening group (0.37±0.80). At birth, 65% of patients were exclusively breast-fed (exBF). At 3 months, that prevalence decreased to 30%, further decreasing to 17% at 12 months. There was a significant difference of -0.31 (95% CI [-0.66; -0.51]) in Z-score-weight between those exBF for 12 months (- 0.51 ± 0.56) compared to all other feeding methods (mixed feeding, exclusive formula etc) (-0.20 ± 0.94); t(27)=-1.76,p=0.045 (Graph 2). Conclusions Despite regular monitoring and intervention, growth failure was still observed in this cohort. CF patients remain high risk for sub-optimal growth, specifically MI patients. Whilst breastfeeding is internationally recommended, this data highlights the extra care required to support growth. References UK Cystic Fibrosis Registry Annual Data Report 2019, Cystic Fibrosis Trust, 2020 August Trajectories of early growth and subsequent lung function in cystic fibrosis: an observational study using UK and Canadian registry data, Macdougall et al., 2022 September