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ObjectiveUse of peptide-based formulas supplemented with medium chain triglycerides (MCTs) is considered a beneficial strategy to decrease the tube-feeding associated gastrointestinal tolerance. In children with cerebral palsy (CP), overall effects of enteral tube feeding as well as the utility of peptide-based specialized enteral formulas in those with gastrointestinal intolerance have not been extensively studied. This study aimed to evaluate the utility of enteral tube feeding via specialized peptide-based formula containing MCTs in children with CP in terms of gastrointestinal intolerance, anthropometrics, defecation characteristics and parental satisfaction with enteral formula.MethodsChildren with CP who received enteral tube feeding via specialized peptide-based formula containing MCTs were included in this prospective observational study. Anthropometrics (z scores for weight for age [WFA], weight for height [WFH], triceps skinfold thickness [TSFT] and mid-upper arm circumference [MUAC]), gastrointestinal intolerance symptoms, defecation frequency and stool patterns and formula satisfaction were recorded at baseline and during 6-month follow up.ResultsA total of 96 children with CP (mean ± SD age: 5.6 ± 3.2 years, 56.3% were boys) were included. Significant improvements were noted in MUAC, TSFT and WFH z scores at the 6th month visit. The rate of “severe symptoms” and the likelihood of Type-1/Type-2 (constipation) stool pattern were significantly decreased. Majority of parents were satisfied with the study formula.ConclusionOur findings revealed favorable efficacy and safety of using a specialized peptide-based formula containing MCT in provision of enteral tube feeding among children with CP in terms of improved anthropometrics, amelioration of gastrointestinal intolerance symptoms and normalization of bowel movements along with a high parental satisfaction.

IntroductionCircadian Locomotor Output Cycles Kaput (CLOCK) is one of the transcription factors from the positive end of the molecular clock and regulates biological rhythm in mammals. Studies have shown that genetic variations in the CLOCK genes are associated with chronotype, sleep patterns, obesity, dietary energy, and nutrient consumption. Although interest in the field of chrononutrition continues to increase, investigations into the temporal aspects of dietary habits in adolescents are notably limited. This study aims to investigate the relationship between CLOCK rs3749474, rs4580704, and rs1801260 polymorphisms and circadian rhythm, metabolic parameters along with chrononutrition in adolescents.Materials and methodsThis cross-sectional study included a total of 300 adolescents between the ages of 11 and 18. Anthropometric measurements, dietary intake, chrononutrition, circadian as well as biochemical and lifestyle data were collected. CLOCK rs3749474, rs4580704, and rs1801260 gene polymorphisms were analyzed.ResultsThe CLOCK rs3749474 minor T allele carriers showed a statistically significant increased risk of being overweight or obese (OR: 2.106, p = 0.003). The minor G allele carriers of the CLOCK rs4580704 indicated statistically increased dietary energy intake and eating jetlag (p < 0.05). The frequency of snacking after the last meal was positively correlated with body mass index (BMI) z-scores in minor allele carriers of the CLOCK rs3749474 (β = 0.134, p = 0.003) and rs4580704 (β = 0.142, p = 0.012) variants. The minor G allele carriers of CLOCK rs4580704 revealed a negative link between breakfast frequency along with BMI z-scores (β = −0.178, p = 0.009). Individuals with the rs1801260 minor G allele showed a positive link between BMI z score and meal frequency (β = 0.367, p = 0.049). In CLOCK gene variants, minor allele carriers in addition to non-carriers had similar biochemical parameters and distribution of dietary intake at meal (p> 0.05).ConclusionThese results suggest that the impact of some chrononutrition behaviors on BMI z-scores are partially modulated by the variability in the CLOCK gene variants. Chrononutrition may be important in the shift toward “personalized nutrition” based on gene-diet interactions. For this reason, new dietary approaches may be implemented, including the circadian distribution of macronutrients and chrononutrition behaviors according to genotype. However, studies with larger samples in different populations are needed.

Background & Aims: PEDFIC 2, an ongoing, open-label, 72-week study, evaluates odevixibat, an ileal bile acid transporter inhibitor, in patients with progressive familial intrahepatic cholestasis. Methods: PEDFIC 2 enrolled and dosed 69 patients across two cohorts; all received odevixibat 120 μg/kg per day. Cohort 1 comprised children from PEDFIC 1, and cohort 2 comprised new patients (any age). We report data through 15 July 2020, with Week 24 of PEDFIC 2 the main time point analysed. This represents up to 48 weeks of cumulative exposure for patients treated with odevixibat from the 24-week PEDFIC 1 study (cohort 1A) and up to 24 weeks of treatment for those who initiated odevixibat in PEDFIC 2 (patients who received placebo in PEDFIC 1 [cohort 1B] or cohort 2 patients). Primary endpoints for this prespecified interim analysis were change from baseline to Weeks 22–24 in serum bile acids (sBAs) and proportion of positive pruritus assessments (≥1-point drop from PEDFIC 2 baseline in pruritus on a 0–4 scale or score ≤1) over the 24-week period. Safety monitoring included evaluating treatment-emergent adverse events (TEAEs). Results: In cohort 1A, mean change from PEDFIC 1 baseline to Weeks 22–24 of PEDFIC 2 in sBAs was -201 μmol/L (p

ObjectiveTo evaluate growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula (eHCF) in infants with cow’s milk protein allergy (CMPA).MethodsA total of 226 infants (mean ± SD age: 106.5 ± 39.5 days, 52.7% were girls) with CMPA who received eHCF comprising at least half of the daily dietary intake were included. Data on anthropometrics [weight for age (WFA), length for age (LFA) and weight for length (WFL) z-scores] were recorded at baseline (visit 1), while data on infant feeding and stool records, anthropometrics and Infant Feeding and Stool Patterns and Formula Satisfaction Questionnaires were recorded at visit 2 (on Days 15 ± 5) and visit 3 (on Days 30 ± 5).ResultsFrom baseline to visit 2 and visit 3, WFA z-scores (from −0.60 ± 1.13 to −0.54 ± 1.09 at visit 2, and to −0.44 ± 1.05 at visit 3, p

Objective: Early diagnosis of biliary atresia (BA) and the timing of Kasai hepatic portoenterostomy are associated with improved survival rates of the native liver. Acholic stool is a major and earliest sign of BA. We evaluated the awareness and recognition of medical students and primary health care professionals (PHCPs) about neonatal cholestasis and acholic stool as a marker of BA. Materials and Methods: The knowledge of students and PHCP about prolonged jaundice and acholic stool was evaluated through a questionnaire. In the first step, 5 questions evaluating the knowledge of prolonged jaundice were asked. The sixth question was “Have you ever seen acholic stool before?” Following this question, stool color cards with 9 colors were shown, and participants were asked “Which of the following stool pictures would you define as acholic?” Results: A total 724 students and 88 PHCPs were included in the study. In both groups, about half of the participants could not answer the first 4questions related to prolonged jaundice and cholestasis correctly. Twenty-four percent of the students and 11.4% of PHCP answered correctly to all of the stool colors. The rate of correct answers to acholic stool colors were approximately 43.9%-87.6% and 23.9%-86.4% for students and PHCP, respectively. Whitish acholic stool colors were better known than mild yellowish pale stool colors. The percentages of recognition were less than about 50% for these stool colors. Conclusion: This study showed that recognition and awareness of prolonged jaundice are low, and acholic stool is not well known. This may lead to delay in diagnosis. Considering the international success of stool color cards, using stool color cards will improve the outcomes of biliary atresia in our country as well.

Background/Aims: Gastric outlet obstruction (GOO) is a rare condition in childhood, with the exception of infantile hypertrophic pyloric stenosis (IHPS). However, no classification exists from a pediatric gastroenterologist’s perspective. Materials and Methods: The patients with a diagnosis of GOO between 2009 and 2020 were reviewed retrospectively. We classified the patients according to GOO: presence of clinical findings accompanied by radiological and/or endoscopic findings; clinical status: intractable nonbilious postprandial vomiting alone or with abdominal pain, early satiety, weight loss, postprandial abdominal distension, and malnutrition; radiology: delayed gastric emptying and dilated stomach; endoscopy: nonbilious gastric contents after 6-8 hours of emptying and/or failed pyloric intubation; physical examination: visible gastric peristalsis. Results: A total of 30 GOO patients (15 patients with IHPS, 1 patient with annular pancreas, 4 patients with gastric volvulus, 2 patients with duodenal atresia, 2 patients with antral web, 1 patient with late-onset hypertrophic pyloric stenosis (LHPS) had surgical treatment, and remaining 5 patients had medical treatment) were enrolled to the study. The median age was 8 months (range: 3 months-16 years), and 14 patients were female. Mitochondrial disorders, LHPS, metabolic disorders, and eosinophilic gastrointestinal system diseases were added to Sharma’s GOO classification, and the classification has been expanded. Conclusion: This is the first and largest study of GOO in children. From the perspective of pediatric gastroenterology, new diseases will be addressed, and definitions will be highlighted with our classification for GOO in childhood.

IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia.MethodsThis multi-center, prospective study enrolled patients aged 3–216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels >2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients were analyzed.ResultsOverall, 589 patients [66.8% male, mean age of 63.4 months (standard deviation: 60.5)] were included. In total, 251 patients (188 male and 63 female) had CPK levels above the ULN. Of the patients assessed, 47% (85/182) of male patients were diagnosed with DMD/BMD and 1% (3/228) of male and female patients were diagnosed with PD. The median ALT, AST, and CPK levels were statistically significantly higher, and the questioned neurological symptoms and previously unnoticed examination findings were more common in DMD/BMD patients than those without DMD/BMD or PD (p

It is well-known that in children with type 1 diabetes (T1D), the frequency of Celiac disease (CD) is increased due to mechanisms which are not fully elucidated but include autoimmune injury as well as shared genetic predisposition. Although histopathologic examination is the gold standard for diagnosis, avoiding unnecessary endoscopy is crucial. Therefore, for both clinicians and patients' families, the diagnosis of CD remains challenging. In light of this, a joint working group, the Type 1 Diabetes and Celiac Disease Joint Working Group, was convened, with the aim of reporting institutional data and reviewing current international guidelines, in order to provide a framework for clinicians. Several controversial issues were discussed: For CD screening in children with T1D, regardless of age, it is recommended to measure tissue transglutaminase-immunoglobulin A (tTG-IgA) and/or endomysial-IgA antibody due to their high sensitivity and specificity. However, the decision-making process based on tTG-IgA titer in children with T1D is still debated, since tTG-IgA titers may fluctuate in children with T1D. Moreover, seronegativity may occur spontaneously. The authors' own data showed that most of the cases who have biopsy-proven CD had tTG-IgA levels 7-10 times above the upper limit. The decision for endoscopy based solely on tTG-IgA levels should be avoided, except in cases where tTG-IgA levels are seven times and above the upper limit. A closer collaboration should be built between divisions of pediatric endocrinology and gastroenterology in terms of screening, diagnosis and follow-up of children with T1D and suspicious CD.

This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology experts aimed to address the significance of mid-upper arm circumference (MUAC) assessment in diagnosis of pediatric malnutrition. Specifically, the potential utility of recently developed MUAC z-score tape in clinical practice for larger patient populations was addressed including the neurologically disabled children. In accordance with the evidence-based data, four statements were identified by the participating experts on the utility of MUAC z-score tape, including (1) MUAC z-scores correlate with body mass index (BMI) and weight for height/length (WFH/l) z-scores in diagnosing malnutrition; (2) MUAC z-score tape offers a higher sensitivity to diagnose the mild and moderate malnutrition and better ability to track the changes in nutritional status over time than the other single datapoint measurements; (3) Using single-step MUAC z-score tape in children with cerebral palsy (CP) seems to provide more reliable data on anthropometry; and (4) The clinical value of the tool in classifying secondary malnutrition in CP should be investigated in large-scale populations. In conclusion, enabling single-step estimation of nutritional status in a large-scale pediatric population regardless of age and within a wide range of weight, without formal training or the need for ancillary reference charts and calculators, MUAC z-tape offers a favorable tool for easier and earlier diagnosis of pediatric malnutrition. Nonetheless, further implementation of MUAC z-score screening in larger-scale and/or special populations is necessary to justify its utility in relation to other primary anthropometric indicators in diagnosis of malnutrition as well as in treatment monitoring in the community and hospital setting.