(1) Background: Pediatric dysphagia presents significant nutritional challenges, often impacting growth and development due to reduced oral intake, increased nutritional needs, and gastrointestinal complications; (2) Methods: This prospective quasi-experimental study assessed 117 children under 14 years old (20 patients were under 1 year old, 80 were aged 1–7 years, and 17 were older than 7 years), diagnosed with swallowing disorders, to analyze their caloric, macro-, and micronutrient intake and identify potential deficiencies. The severity of dysphagia was established using functional oral intake scales, and dietary records were reviewed over a 3-day period; (3) Results: The study revealed that 39.8% of participants did not meet their total energy expenditure (TEE), highlighting a high prevalence of malnutrition among these children. Furthermore, patients using feeding devices exhibited a significantly lower caloric intake, and over half required significantly modified food textures. After individualized speech therapy and nutritional rehabilitation, participants showed significant improvements in caloric intake, with their energy coverage increasing from 958% to 1198% of the daily requirement. Rehabilitation also improved tolerance to a broader range of food textures; (4) Conclusions: This research underscores the importance of multidisciplinary, individualized nutritional strategies to address the specific challenges of pediatric dysphagia, emphasizing the role of enteral nutrition and therapeutic interventions in improving the quality of life and nutritional outcomes of these children. Further studies are recommended to assess the long-term impact of such strategies.
This is a prospective, multicenter study of a cohort of 224 cystic fibrosis (CF) patients treated with CF transmembrane conductance regulator (CFTR) modulators (CFTRm). Our aim was to prospectively analyze the effect of CFTRm treatment on fat‐soluble vitamin serum levels. Demographic and clinical data were recorded, and fat‐soluble vitamin levels were analyzed at baseline, and at 6 and 12 months after starting treatment. Two groups were analyzed separately: patients receiving dual therapy lumacaftor/ivacaftor or tezacaftor/ivacaftor (Lum/Tez+Iva), and those on triple therapy with elexacaftor/tezacaftor/ivacaftor (ETI). We found that treatment with ETI produced a significant increase in vitamin D and A levels within the first 6 months, which was maintained at 12 months. However, with dual therapy, we observed an increase only in vitamin A levels within the first 6 months, which was not maintained at 12 months. No differences were found in vitamin E serum levels between the groups.
Background: Triple therapy with cystic fibrosis transmembrane conductance regulator (CFTR) modulators in patients with cystic fibrosis (CF) has led to a shift in the nutritional management of the disease. Correct assessment of nutritional status is crucial due to its significant impact in pulmonary function and overall patient survival. This study aims to provide mid-term data on anthropometric and body composition changes in pediatric patients treated with elexacaftor–tezacaftor–ivacaftor (ETI). Methods: A prospective, longitudinal, multicenter study was conducted involving pediatric CF patients receiving ETI therapy. Anthropometric measurements and bioelectrical impedance analysis (BIA) data were collected at baseline and after 24 months of treatment. Results: A group of 66 pediatric patients, median age of 11.11 years (IQR: 8.2–12.5), was studied. The BMI z-score increased from −0.63 at baseline to −0.38 (p < 0.001) after 2 years of ETI treatment. Somatometric parameters were compared with a retrospective cohort showing no significant results. Significant changes comparing body composition were also observed over the 24 months period: fat mass (FM) increased from 6.2 kg to 7.4 kg (p < 0.001) free fat mass (FFM), increased from 28.2 kg to 34.2 kg (p < 0.001) and body cellular mass (BCM), increased from 7.8 kg to 8.7 kg (p = 0.02). Conclusions: Pediatric patients undergoing ETI treatment showed mid-term improvements in anthropometric parameters, alongside notable changes in body composition. Long-term studies are needed to confirm these findings and to better understand the implications for pediatric CF care.