Background: Improved therapy in CF has led to an overall improvement in nutritional status. The objectives of our study are: to cross-sectionally assess nutritional status and serum levels of fat-soluble vitamins; to retrospectively evaluate the efficacy of modulators on nutritional status and fat-soluble vitamin levels. Methods: In patients younger than 2 years of age, we evaluated growth, in patients aged 2–18 years, we assessed BMI z-scores, and in adults, we assessed absolute BMI values. Levels of 25(OH)D, vitamins A, and E were measured. Results: A cross-sectional analysis was conducted on 318 patients, 109 (34.3%) with pancreatic sufficiency. Only three patients were under 2 years old. In 135 patients aged 2–18 years, the median BMI z-score was 0.11, and 5 (3.7%) patients had malnutrition (z-score ≤ 2SD). In 180 adults, the median BMI was 21.8 kg/m2. Overall, 15 (13.7%) males (M) and 18 (25.3%) females (F) were underweight (18 < BMI > 20); 3 (2.7%) M and 5 (7.0%) F had a BMI < 18. Suboptimal 25(OH)D levels were found in patients with pancreatic insufficiency. The prevalence of deficiency of vitamins A and E is low. After one year of treatment with modulators, the increase in BMI was more consistent (M: 1.58 ± 1.25 kg/m2 F: 1.77 ± 1.21 kg/m2) in elexacaftor/tezacaftor/ivacaftor (ETI)-treated patients compared with other modulators, with a significant increase in levels of all fat-soluble vitamins. Conclusions: Malnutrition is present in a limited number of subjects. The prevalence of subjects with suboptimal 25(OH)D levels is high. ETI showed a beneficial effect on nutritional status and circulating levels of fat-soluble vitamins.

Exclusive enteral nutrition (EEN) represents an established, evidence-based dietary therapy used in Crohn's disease (CD); although successful, EEN is extremely restrictive with limited acceptability and prolonged use. The Crohn's disease exclusion diet (CDED) is a new, sustainable and patient-friendly dietary therapy used for the management of pediatric CD. CDED is designed to reduce exposure to dietary components hypothesized to negatively affect the microbiome, intestinal barrier and immunity. By focusing on five clinical cases, this article illustrates the benefits of using CDED as mono-or cotherapy with partial enteral nutrition in children with mild to moderate CD. CDED combined with partial enteral nutrition is a safe and effective therapeutic option for both induction and maintenance therapy in children with mild to moderate CD. It ensures sustained remission and can induce mucosal healing in children with mild to moderate Crohn's disease.

Background We aimed at appraising real-life efficacy of Crohn’s Disease Exclusion Diet (CDED) coupled with partial enteral nutrition (PEN) in inducing clinical and biochemical remission at disease onset and in patients with loss of response to biologics in a tertiary-level center. We also aimed at identifying early responders to this dietary regimen. Methods We gathered clinical, anthropometric and laboratory data of patients aged less then 18 years of age with a diagnosis of CD, who were consecutively treated with CDED coupled with PEN as main treatment for the induction of remission or in the setting of loss of response to other therapies. We collected data of patients who received CDED plus PEN from April 2019 to June 2022 at diagnosis, at the beginning of the dietary treatment, and at the phase 1. Patients who interrupted diet during follow-up were considered as treatment failures on an intent to treat basis. We compared groups using chi-square, Fisher’s exact test, Mann-Whitney U or related-samples Wilcoxon signed rank tests or McNemar’s test as appropriate. Results 47 patients were retrospectively identified. Table 1. summarizes clinical and biochemical characteristics at diagnosis and at CDED with PEN initiation. 24 (51.1%) patients started CDED as induction therapy at disease onset, whereas 23 (48.9%) of them received CDED with PEN as add-on therapy. 32/47 (68%) patients achieved clinical remission (wPCDAI &lt; 12.5) at the end of phase 1, 16/24 patients (66.7%) who started CDED as induction therapy and 16/23 (69.5%) of those who started CDED as add-on, with no statistically significant difference among the two groups (p=1.00). Laboratory parameters significantly improved in both groups (Figure 1.). There were no statistically significant differences in clinical remission rates between patients with mild-to-moderate and severe disease at the end of phase 1 (25/35 vs 7/12, p=0.481). At disease onset, 14 patients added a concomitant treatment (11 patients added anti-TNF alpha, 3 patients added IMM and one patient added anti-TNF alpha + IMM) after a median time of 4 weeks (IQR: 3-5 weeks). 28 patients had clinical data gathered at week 3. Patients who achieved clinical response at week 3 (a change in wPCDAI &gt; 12.5) were more likely to be in clinical remission at the end of phase 1 (17/20 vs 1/8, p&lt;0.01), both in patients who started CDED at disease onset and in the add-on setting. Conclusion CDED with PEN confirmed its efficacy in a real-life setting, both as induction regimen and as add-on therapy, also in patients with clinically severe disease. Early clinical response predicts clinical remission at the end of phase 1, possibly allowing identification of dietary responsive disease. Table 1. Figure 1.

Cystic fibrosis (CF) is an autosomal recessive genetic disease characterized by mutations in the CF transmembrane conductance regulator (CFTR) gene. The CF phenotype is characterized by lung disease, exocrine pancreatic insufficiency, impaired growth, hepatobiliary manifestations, and male infertility. A disadvantage in survival and morbidity, called the "CF gender gap," is known in women with CF. They more commonly acquire infections at an earlier age than men with CF, have shorter life expectancies, and are more frequently malnourished. 1 Malnutrition is a common management problem in patients with CF and is defined as having a body mass index (BMI) <18.5 kg/mš for adults and a BMI percentile <10th in children and adolescents up to 20 years of age. 2 Compared with well-nourished individuals, malnourished patients show a steeper lung function decline, whereas improvements in weight gain and BMI are associated with a slower decline in lung function. 3,4 Enteral nutrition (EN) administered via a percutaneous endoscopic gastrostomy (PEG) tube is a well-tolerated method to improve nutrition status, with a low incidence of complications. 5,6 There are no data on sex-related differences in the efficacy of EN via PEG in this population. We conducted a retrospective observational study in a cohort of 18 CF patients (11 male patients, 5/18 children) followed at a CF center in Florence, Italy, and requiring EN via PEG after the failure of conservative dietary measures. All patients (or their legal guardian) provided informed consent to use anonymized clinical data for research purposes.No significant differences in pancreatic and microbiological status were identified by sex. All patients had exocrine pancreatic insufficiency, and chronic Pseudomonas aeruginosa infection was present in 6 (54.5%) of 11 male patients and four (57.1%) of seven female patients. We evaluated the effect of EN on BMI and forced expiratory volume in 1 s (FEV 1 %) values after 6 months of treatment. Comparisons between independent samples were assessed by two-tailed Student t-test for the equality of the means.