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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 evidence-based method in PERT dosing based on food characteristics. Results have to be confirmed in free dietary settings. [ABSTRACT FROM AUTHOR]

Background: Expressing anthropometric parameters (height, weight, BMI) as z-score is a key principle in the clinical assessment of children and adolescents. The Centre for Disease Control and Prevention (CDC) growth charts and the CDC-LMS method for z-score calculation are widely used to assess growth and nutritional status, though they can be imprecise in some percentiles. Objective: To improve the accuracy of z-score calculation by revising the statistical method using the original data used to develop current z-score calculators. Design: A Gaussian Process Regressions (GPR) was designed and internally validated. Z-scores for weight-for-age (WFA), height-for-age (HFA) and BMI-for-age (BMIFA) were compared with WHO and CDC-LMS methods in 1) standard z-score cut-off points, 2) simulated population of 3000 children and 3) real observations 212 children aged 2 to 18 yo. Results: GPR yielded more accurate calculation of z-scores for standard cut-off points (p<<0.001) with respect to CDC-LMS and WHO approaches. WFA, HFA and BMIFA z-score calculations based on the 3 different methods using simulated and real patients, showed a large variation irrespective of gender and age. Z-scores around 0 +/- 1 showed larger variation than the values above and below +/- 2. Conclusion: The revised z-score calculation method was more accurate than CDC-LMS and WHO methods for standard cut-off points. On simulated and real data, GPR based calculation provides more accurate z-score determinations, and thus, a better classification of patients below and above cut-off points. Statisticians and clinicians should consider the potential benefits of updating their calculation method for an accurate z-score determination. [ABSTRACT FROM AUTHOR]

Objective: The Cow’s Milk-related Symptom Score (CoMiSSTM) was developed as an awareness tool to recognize possible manifestations of cow’s milk protein allergy (CMPA). Arbitrarily, a cut-off value of ≥12 was defined as a "positive score." The aim of this study was to determine an age-related CoMiSS in healthy infants to minimize the risk of false reassurance or over-diagnosis of CMPA in case of a negative or positive score, respectively. Methods: General pediatricians determined the CoMiSS in presumed healthy infants aged ≤6 months during a routine visit. Exclusion criteria included any known acute or chronic disease, preterm delivery (< 37 weeks), therapeutic formula, any food supplement (except vitamins) or medication. Results: Data from 891 consecutive infants were collected. Complete information was obtained from only 413 (46.4%) infants: Belgium: 31.2%, Italy 18.2%, Poland 19.1% and Spain 31.5%. Since gender (girls vs boys) (p = 0.579) had no influence on the CoMiSS, the data were re-calculated to include those infants with missing gender. The overall median and mean (SD) CoMiSS scores were, respectively, 3.0 and 3.7 (2.9). The 95th percentile was 9. Median crying (p<0.001), regurgitation (p = 0.009) and eczema (p = 0.039) scores differed significantly across the age categories. The other components of the CoMiSS were not age dependent. Conclusion: In healthy infants ≤ 6 months, the median CoMiSS is 3.0. More prospective studies in different sites and comparing healthy and allergic infants are warranted to obtain further evidence on the utility of the CoMiSS. [ABSTRACT FROM AUTHOR]