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Objectives/Background: Vitamin D-binding protein (VDBP) and free vitamin D are new markers that are being studied as a possible markers of vitamin D status. The main aim of our study was to analyze the VDBP genotype and quantify the levels of free vitamin D in a sample of cystic fibrosis (CF) patients. Methods: We conducted a multicenter, cross-sectional, and prospective study including patients with CF and exocrine pancreatic insufficiency who were clinically stable. We investigated vitamin D levels (total and free) and the different VDBP haplotypes. Free vitamin D levels were measured using an electro-chemiluminescence assay. Results: A sample of 48 patients was obtained (52% male; median age 13.8 years). The most common allele of VDBP was Gc1s (72%) > Gc2 (52%) > Gc1f (27%). The median calcidiol was 21.2 ng/mL (IR 15.3–26.9), and 81% had levels in the insufficiency range: 23 patients (48%) below 20 ng/mL, and 16 (33%) between 20 and 30 ng/mL. The median free vitamin D level was 4.2 pg/mL (IR 3.9–5.6). A positive correlation was observed between calcidiol and free vitamin D levels (r = 0.871; p < 0.0001). After adjustment for season, vitamin D supplementation, sex, and CF-related diabetes, patients with Gc1f polymorphism had a lower risk of vitamin D deficiency, OR 0.22 (95% CI 0.05–0.99), and p = 0.027. A negative linear trend was observed between the polymorphisms grouped into three categories (Gc1/Gc1, Gc1/Gc2, and Gc2/Gc2, in that order) and vitamin D and free vitamin D levels (p = 0.025 and p = 0.033, respectively). Conclusion: In CF, as in the general population, the most common VDBP haplotype in the Caucasian race is Gc1s. VDBP polymorphisms influence serum vitamin D and free vitamin D levels in CF patients. There is a good correlation between free vitamin D and calcidiol levels, suggesting that measuring the latter in CF does not seem to provide any additional benefit.

Introduction: In recent years, guidelines for vitamin D supplementation have been updated and prophylactic recommended doses have been increased in patients with cystic fibrosis (CF). Objective: To evaluate safety and efficacy of these new recommendations. Results: Two cohorts of pancreatic insufficient CF patients were compared before (cohort 1: 179 patients) and after (cohort 2: 71 patients) American CF Foundation and European CF Society recommendations were published. Cohort 2 patients received higher Vitamin D doses: 1509 (1306–1711 95% CI) vs 1084 (983–1184 95% CI) IU/Day (p < 0.001), had higher 25 OH vitamin D levels: 30.6 (27.9–33.26 95% CI) vs. 27.4 (25.9–28.8 95% CI) ng/mL (p = 0.028), and had a lower prevalence of insufficient vitamin D levels (<30 ng/mL): 48% vs 65% (p = 0.011). Adjusted by confounding factors, patients in cohort 1 had a higher risk of vitamin D insufficiency: OR 2.23 (1.09–4.57 95% CI) (p = 0.028). Conclusion: After the implementation of new guidelines, CF patients received higher doses of vitamin D and a risk of vitamin D insufficiency decreased. Despite this, almost a third of CF patients still do not reach sufficient serum calcidiol levels.

Background/Objectives: Citrulline, an amino acid produced by small bowel enterocytes, has been proposed as a potential marker of intestinal absorptive capacity. The aim of this study is to evaluate whether measuring citrulline levels could be useful for celiac disease (CD) patients, both at the time of diagnosis and during follow-up. Methods: A multicenter prospective study was conducted. Citrulline levels were measured and compared in 93 pediatric patients classified into three groups. Group A: 28 patients with newly diagnosed CD. In this group, an additional measurement was performed after 3–6 months on a gluten-free diet (GFD). Group B: 32 patients with a prior CD diagnosis and on a GFD for at least 6 months. Group C: 33 healthy controls. Citrulline levels were correlated with clinical and laboratory variables, including serological markers. Statistical analysis: t-tests for paired groups and independent groups, Pearson and Spearman correlation tests. Results: Newly diagnosed CD patients had lower citrulline levels compared to those on a GFD for more than 6 months (27.13 vs. 32.42 µmol/L; p > 0.05). Citrulline levels were nearly identical between healthy controls and CD patients on a GFD for more than 6 months (32.48 vs. 32.42 µmol/L; p > 0.05). Starting a GFD led to a significant increase in citrulline levels in group A (from 27.13 to 37.43 µmol/L, p < 0.001). Conclusions: Plasma citrulline could serve as a valuable marker for mucosal recovery in the follow-up of diagnosed celiac patients adhering to a GFD.

Background: Elimination of gluten-containing cereals and consumption of ultra-processed gluten-free foods might cause an unbalanced diet, deficient in fiber and rich in sugar and fat, circumstances that may predispose celiac children to chronic constipation. Aim: to evaluate if counseling with a registered dietitian (RD) was capable of improving eating and bowel habits in a celiac pediatric population. Methods: Dietetic, lipid profile and stool modifications were analyzed, comparing baseline assessments with those twelve months after receiving heathy eating and nutrition education sessions. At both time points, 3-day food records, a bowel habit record and a lipid panel were conducted. Calculated relative intake of macro- and micro-nutrients were compared with current recommendations by the European Food Safety Authority (EFSA). Student’s paired t-test, McNemar test, Mandasky test and Pearson correlation tests were used. Results: Seventy-two subjects (58.3% girls) with a mean (standard deviation (SD)) age of 10.2 (3.4) years were included. Baseline diets were imbalanced in macronutrient composition. Significant improvements were observed in their compliance with dietary reference values (DRVs), where 50% of the subjects met fat requirements after the education and 67% and 49% with those of carbohydrates and fiber, respectively (p < 0.001). Celiac children decreased red meat and ultra-processed foods consumption (p < 0.001) and increased fruits and vegetables intake (p < 0.001), leading to a reduction in saturated fat (p < 0.001) and sugar intake (p < 0.001). Furthermore, 92% of the patients achieved a normal bowel habit, including absence of hard stools in 80% of children constipated at baseline (p < 0.001). Conclusions: RD-led nutrition education is able to improve eating patterns in children with celiac disease (CD).

Cow's milk protein allergy (CMPA) is among the most common pediatric food allergies. Understanding tolerance acquisition and diagnostic approaches is critical for optimizing care, particularly in Spain, where regional differences may impact management. This study analyzed real-world practices for determining CMPA oral tolerance acquisition by Spanish pediatricians. A questionnaire was distributed to healthcare providers in primary and hospital pediatric settings across various Spanish provinces. The survey addressed demographic profiles, diagnostic approaches, tolerance acquisition, and dietary modifications. Among the 269 health professionals included (mean age 48.3 ± 11.0 years, 62.3% women), most worked in primary care (55.4%), 20.4% in public hospitals, and 17.8% in private practice. Overall, 35.5% routinely referred CMPA cases to specialists. Specific IgE testing (27.9%) and elimination diets (41.3%) were the main diagnostic tools. Diagnostic dairy-exclusion duration varied, mainly in non-IgE cases. Hospital-based diagnostic oral food challenges (OFC) were preferred for suspected IgE-mediated cases (95.7%), while home-based protocols were used for non-IgE suspected cases (80.7%). Guideline adherence for home-based OFC varied by specialty. Tolerance acquisition was evaluated annually by 67.7% of participants, with a mean success rate of 80%. Therapeutic dairy-exclusion duration varied, with 64.7% excluding CMP for 6-12 months. Minimum age for CMP reintroduction was considered dependent on phenotype and severity, with 12 months of age mentioned most frequently (17.1%). Despite general alignment with international guidelines, relevant variability exists in CMPA management in Spain. Harmonizing diagnostic and therapeutic practices across specialties and care levels may help standardize care and improve patient outcomes.

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.

Management of cow’s milk protein allergy (CMPA) can vary depending on the experience and area of expertise of the clinician responsible for the patient’s follow-up, which may or may not align with the recently published literature. To analyze the perspectives of Spanish pediatricians on this topic, a survey was conducted. The survey aimed to determine the current opinions and attitudes of 222 primary care and hospital pediatricians toward CMPA prevention and nutritional management. Participating pediatricians completed the questionnaire, providing insights into their daily clinical practices, including access to testing, attitudes with respect to various aspects of CMPA diagnosis, prevention, oral food challenges, and treatment. The findings revealed that pediatricians generally agree on the use of extensively hydrolyzed formulas (eHFs) to prevent CMPA in high-risk atopic children, despite limited evidence supporting the widespread use of this practice. However, consensus was lacking regarding the utility of formulas with prebiotics and probiotics for expediting tolerance development. In most cases, pediatricians preferred eHFs for the nutritional management of CMPA, followed by hydrolyzed rice formulas (HRFs), with amino-acid-based formulas (AAFs) being the third option. Certain issues remained controversial among pediatricians, such as prevention methods, symptom assessment, and the role of probiotics. These variations in management approaches reflect the influence of clinician experience and area of expertise, underscoring the need for standardized guidelines in this field.

Background: Hydrolyzed rice formulas (HRFs) are increasingly recognized as an alternative nutritional option for infants with cow’s milk protein allergy (CMPA), but real-world data on prescribing patterns and early clinical evolution remain limited. Objective: This study aimed to describe real-world use and prescribing patterns of HRF and eHF in infants with CMPA, identify factors associated with HRF recommendation, and assess short-term clinical evolution after formula use. Methods: This observational analysis from the ETAPA project included two mutually exclusive infant-level cohorts: a prospective recommendation cohort assessing variables associated with HRF versus eHF recommendation, and a retrospective treated cohort assessing 7-day clinical evolution after formula use. In the retrospective cohort, the main clinical outcome was absolute change in CoMiSS from day 0 to day 7. A propensity score-weighted sensitivity analysis was performed to address measured confounding by indication. Results: Overall, 1505 valid infant-level records were analyzed: 1094 in the prospective cohort and 411 in the retrospective cohort. In the prospective cohort, HRF was recommended in 214/1094 records (19.6%). In multi-variable analysis, older infant age was associated with higher odds of HRF recommendation (OR 1.034 per month, 95% CI 1.002–1.067; p = 0.036), whereas IgE-mediated CMPA was associated with lower odds (OR 0.673, 95% CI 0.459–0.988; p = 0.043). In the retrospective cohort, CoMiSS decreased markedly in both groups (eHF: median reduction 7.0 [IQR 4.0–10.0] points; HRF: 6.5 [4.0–10.0] points; p = 0.661). After adjustment, HRF was not associated with a statistically significant difference in absolute CoMiSS reduction compared with eHF (β −0.513 points, 95% CI −1.108 to 0.082; p = 0.091). Conclusions: In routine pediatric practice, HRF was used across a broad range of CMPA profiles and was associated with clinically relevant short-term symptom improvement. These findings support HRF as an additional nutritional option for CMPA management, while the observational design precludes conclusions of equivalence or non-inferiority versus eHF.

This ex-vivo study compares the adaptation, porosity, and sealing performance of ProRoot MTA, NeoPutty, and Biodentine in standardized, simulated furcation perforations created in extracted mandibular molars using clinical evaluation and high-resolution micro-computed tomography (micro-CT). Thirty-six mandibular molars with furcation perforations were randomly assigned to three groups ( n  = 12). Clinical evaluation assessed adaptation, porosity, and overfilling, while micro-computed tomography (micro-CT) provided quantitative data on voids and gaps. Statistical analysis used chi-square, Kruskal–Wallis, and Mann–Whitney U tests. NeoPutty and ProRoot MTA showed superior adaptation compared to Biodentine ( p  < 0.05). Biodentine showed higher porosity (28.44%) than ProRoot MTA (0%) and NeoPutty (8.3%) ( p  < 0.001). Biodentine also had the highest void volume (1.05 mm 3 ) and gap volume (1.37 mm 3 ), while ProRoot MTA recorded the lowest void volume (0.59 mm 3 ), and NeoPutty had the smallest gap volume (0.85 mm 3 ). No significant differences were observed in overfilling rates. Overall, ProRoot MTA exhibited the most consistent sealing ability, whereas NeoPutty emerged as a viable alternative due to its favorable handling and reliable adaptation. Biodentine, by contrast, showed the poorest performance in terms of structural integrity and sealing capacity. Within the limitations of this study, these findings support the use of ProRoot MTA or NeoPutty for furcation perforation repair, while suggesting more cautious use of Biodentine in such applications.

Hahsp17.6G1 is the promoter of a small heat stress protein (sHSP) from sunflower (Helianthus annuus) that is activated during zygotic embryogenesis, but which does not respond to heat stress. We report here the cloning of a transcription factor (TF), sunflower drought-responsive element binding factor 2 (HaDREB2), by one-hybrid interaction with functional cis-elements in Hahsp17.6G1. We have analyzed the functional interaction between HaDREB2 and a second transcription factor, sunflower heat stress factor A9 (HaHSFA9), which was previously assigned to the regulation of Hahsp17.6G1. HaDREB2 and HaHSFA9 synergistically trans-activate the Hahsp17.6G1 promoter in bombarded sunflower embryos. This synergistic interaction is heat stress factor (HSF) specific and requires the binding of both factors to the promoter. The C-terminal region of HaHSFA9 is sufficient for the HSF specificity. Our results represent an example of a functional interaction between members of the Apetala 2 (HaDREB2) and HSF (HaHSFA9) families of transcription factors. We suggest new roles in zygotic embryogenesis for specific members of the AP2 transcription factor family.