Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis

Background A method to adjust Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis is not currently available. Objectives To assess the in vivo efficacy of a method to adjust the dose of enzymatic supplement in CF extrapolated from previous in vitro digestion studies (theoretical optimal dose, TOD). Secondly, to assess how individual patient characteristics influence the expected coefficient of fat absorption (CFA) and thus to identify an individual correction factor to improve TOD. Methods A prospective interventional study in 43 paediatric patients with CF from 5 European centres. They followed a 24h fixed diet with the theoretical optimal dose for each meal. Faecal collection was carried out between colorimetric markers in order to include all the faeces corresponding to the fixed diet. Beta regression models were applied to assess the associations of individual patient characteristics with the CFA. Results Median CFA was 90% (84, 94% 1st, 3rd Q.) with no significant differences among centres. Intestinal transit time was positively associated with CFA (p = 0.007), but no statistical associations were found with and age, gender, phenotype or BMI. Regression model showed no improvement of the in vitro predicted theoretical optimal dose when taking individual patient characteristics into account. Conclusion Strict adherence to the theoretical optimal dose of enzymatic supplement for a prescribed meal, led to median CFA levels at the clinical target of 90% with a low variability between patients. The proposed method can be considered as a first approach for an evidencebased method in PERT dosing based on food characteristics. Results have to be confirmed in free dietary settings

Establishing a large-scale Greenhouse Gas Removal sector in the United Kingdom by 2030: First mover dilemmas

In June 2019 the UK legislated a 2050 net-zero emissions target. This will require the realisation of a technical Greenhouse Gas Removal (GGR) sector potentially generating over 60 MtCO2 pa of negative emissions by 2050. In October 2021, the UK pledged that at least 5 MtCO2 of engineered negative emissions be deployed by 2030. At present less than 0.5 ktCO2 pa of engineered negative emissions are deployed. The 10,000-fold scale-up to 2030 will require the co-ordinated engagement of first movers to establish and realise at least one GGR value chain. The 120,000-fold scale up to 2050 will require the integration of multiple GGR value chains with existing infrastructure systems and substantive societal engagement to enhance positive social outcomes. This scaling is fundamental to the UK, and arguably international efforts, to address the worst impacts of climate change. A series of exploratory exercises have been undertaken to identify the financial and non-financial barriers to the establishment of a UK multi-MtCO2 pa scale GGR sector from a first mover perspective. This is the first synthesis of first mover drivers in the UK GGR sector. The key findings include: (1) The GGR sector represents a multi-billion pound opportunity in 2050; and (2) inspite of this opportunity - as the incentive, policy, regulatory and governance ecosystem presently stands - first movers face too much risk, uncertainty and a multiplicity of dilemmas to commit substantive investments in establishing the UK GGR multi-MtCO2 pa sector

Evaluation of visual and taste preferences of some gluten-free commercial products in a group of celiac children

The purpose of this study was to investigate the visual and taste liking of three commercial gluten-free (GF) foods in a group of celiac children. Thirty celiac children, with diagnosis of celiac disease from more than 2 years, were recruited and 28 (21 females and 7 males, mean age 8.7 years) completed the study. Subjects performed individually six sensory tests, two for each product in different days separated by a week. In the test day, children had to evaluate the liking of the same type of product but of different four brands, before and after tasting them with a 5-point facial rating scale. Overall, the results showed that the majority of children appreciated more the appearance than the taste of all the GF products analysed. The present study provides the first and useful indications on the hedonic perceptions of celiac children about some commercial GF products

Evaluation of visual and taste preferences of some gluten free commercial products in a group of celiac children

The purpose of this study was to investigate the visual and taste liking of three commercial gluten-free (GF) foods in a group of celiac children. Thirty celiac children, with diagnosis of celiac disease from more than 2 years, were recruited and 28 (21 females and 7 males, mean age 8.7 years) completed the study. Subjects performed individually six sensory tests, two for each product in different days separated by a week. In the test day, children had to evaluate the liking of the same type of product but of different four brands, before and after tasting them with a 5-point facial rating scale. Overall, the results showed that the majority of children appreciated more the appearance than the taste of all the GF products analysed. The present study provides the first and useful indications on the hedonic perceptions of celiac children about some commercial GF products

Efficacy and tolerability of Creon for Children in infants and toddlers with pancreatic exocrine insufficiency caused by cystic fibrosis : an open-label, single-arm, multicenter study

Objectives: To evaluate the efficacy and safety of a pancreatic enzyme preparation specifically developed for infants and small children with cystic fibrosis (CF). Methods: Twelve patients with CF younger than 24 months with pancreatic exocrine insufficiency and a coefficient of fat absorption (CFA) less than 70% were treated with Creon for Children (Solvay Pharmaceuticals GmbH, Hannover, Germany) minimicrospheres for 8 weeks. The primary end point was the mean change from baseline in the CFA after 2 weeks of treatment, based on 72-hour fat balance assessments. Results: Two weeks' treatment with Creon for Children resulted in a significant increase in the mean CFA from 58.0% at baseline to 84.7% (P = 0.0013) in the full analysis sample. There was a significant reduction of mean stool fat (from 13.3 to 5.3 g/d; P = 0.001) and mean fecal energy loss (from 238.5 to 137.9 kJ/d; P = 0.018) at 2 weeks. Dietary fat intake did not change, whereas an improvement was observed in stool frequency and characteristics. Patient weight and height increased over 8 weeks of treatment. No serious adverse event was reported. Conclusions: Creon for Children was well tolerated and significantly decreased fat malabsorption in infants with pancreatic exocrine insufficiency due to CF