Exploring histological remission in pediatric patients with celiac disease.

In response to the article presented by Laura Rodríguez-Martínez et al., which discusses mucosal healing rates in celiac disease and the risk factors for persistent damage despite a gluten-free diet in adults, we present a review of biopsies from pediatric patients with celiac disease performed at our center between 2001 and 2020. Notably, there is a higher rate of mucosal healing compared to adults (83%), and factors such as age at diagnosis, histological involvement, and anti-tissue transglutaminase antibody levels at diagnosis do not appear to be significant risk factors, unlike in adults.

Faecal lipid profile as a new marker of fat maldigestion, malabsorption and microbiota.

Background: Fat malabsorption in children with cystic fibrosis (CF) leads to poor nutritional status and altered colonic microbiota. This study aimed at establishing the faecal lipid profile in children with CF, and exploring associations between the faecal lipidome and microbiota.

Methods: Cross-sectional observational study with children with CF and an age-matched control group.

Long-term evaluation of faecal calprotectin levels in a European cohort of children with cystic fibrosis.

Objective: Intestinal inflammation with contradictory data on faecal calprotectin (fCP) levels is documented in patients with cystic fibrosis (CF). The aim of this study was to longitudinally evaluate fCP in a cohort of children with CF and their relationship with clinical variables.

Design: Prospective observational study to assess evolution of fCP levels, primary aimed at improving fat absorption.

Effect of beta-glucan supplementation on cystic fibrosis colonic microbiota: an in vitro study.

Background: Children with cystic fibrosis (CF) present with gut dysbiosis, and current evidence impedes robust recommendations on the use of prebiotics. This study aimed at establishing the prebiotic potential of a commercial beta-glucan on the in vitro colonic microbiota of a child with CF compared to a healthy counterpart (H).

Methods: A dynamic simulator of colonic fermentation (twin-SHIME® model) was set up including the simulation of the proximal (PC) and distal colon (DC) of the CF and the H subjects by colonizing the bioreactors with faecal microbiota.

Study of the immune response in celiac patients with selective IgA deficiency who start a gluten-free diet.

Studies are scarce regarding IgG anti-tissue transglutaminase 2 (tTG) normalization in selective IgA deficient (SIgAD) celiac disease (CD) patients after beginning a gluten free diet (GFD). The aim of this study is to analyse the decreasing dynamics of IgG anti-tTG in patients diagnosed with CD who start a GFD. To achieve this objective, IgG and IgA anti-tTG levels at diagnosis and during follow-up in 11 SIgAD CD patients and in 20 IgA competent CD patients were retrospectively evaluated.

How macronutrients and pancreatic enzyme supplements dose variability affect fat, protein and starch absorption in children with cystic fibrosis.

Background: low evidence on the dose of enzymatic supplements used in pancreatic enzyme replacement therapy (PERT) is available.

Aim: assessing if fat, protein and starch absorption could be related to the dose of the enzymatic supplement, the intra-patient variability in the dose and macronutrient intake.

Methods: Four-day food records and 3-day faecal samples were prospectively collected in 69 children with cystic fibrosis.

Encephalopathy in short bowel syndrome: a diagnostic challenge.

A 15-year-old boy was admitted to the hospital due to ataxia, drowsiness and bradypsychia. He was known to have a short bowel syndrome Initial venous blood gases revealed a metabolic acidosis with a high anion gap of 24 mmol/L and normal L-lactate. He improved with fasting and fluids and was discharged with oral metronidazole.

Intestinal anti-tissue transglutaminase IgA deposits as a complementary method for the diagnostic evaluation of celiac disease in patients with low-grade histological lesions.

Evaluating the usefulness of intestinal anti-transglutaminase IgA (anti-TG2 IgA) deposits detection as a complementary or decision-supporting tool in the diagnosis of celiac disease (CD) in patients with low degree of enteropathy. Small intestinal biopsies (SIB) were performed from 2008 to 2017 in patients on suspicion of CD (positive CD serology and/or symptoms) referred to our Pediatric Gastroenterology Unit. We determined anti-TG2 IgA deposits by using double immunofluorescence in all the patients in whom Marsh 0 or Marsh 1 was detected in the conventional histological study and in a random selection of patients with clearly positive serology and histological Marsh 2-3 lesion.

Screening-detected positive serology for celiac disease: a real clinical challenge.

According to European Society for Paediatric Gastroenterology, Hepatology, and Nutrition 2020 criteria for celiac disease diagnosis, the small bowel biopsy (SBB) can be omitted in selected circumstances, even in asymptomatic patients. Hence, we have conducted a retrospective study to identify the histological findings of the asymptomatic patients with antitransglutaminase IgA antibodies 10 times above the upper limit of normal and positive antiendomisium antibodies; 5/24 patients fulfilling these criteria had, however, a nonconclusive SBB and were diagnosed with potential celiac disease. The nonbiopsy approach in these cases needs to be carefully evaluated and the risk of overdiagnosis pondered as the management and evolution of potential celiac disease cases is still a matter of study.

Bone mineral density in spanish children at the diagnosis of inflammatory bowel disease.

Unlabelled: The association between low bone mineral density (BMD) and inflammatory bowel disease (IBD) is already known. Our study, performed in Spanish pediatric IBD patients at diagnosis onset, shows that low BMD already existed at the beginning of the disease. Low weight and height are also associated with low BMD and have to be considered as risk factors.

Change in Nutrient and Dietary Intake in European Children with Cystic Fibrosis after a 6-Month Intervention with a Self-Management mHealth Tool.

Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and a healthy diet with a specific nutrient distribution. Nutritional status is an indicator of disease prognosis and survival. This study aimed at assessing the effectiveness of a self-management mobile app in supporting patients with CF to achieve the dietary goals set by the CF nutrition guidelines.

Analysis of gluten immunogenic peptides in feces to assess adherence to the gluten-free diet in pediatric celiac patients.

Purpose: In celiac disease (CD) there is a need for precise and non-invasive tools to assess dietary compliance to the gluten-free diet (GFD). Our aim is to evaluate the efficacy of the detection of gluten immunogenic peptides (GIP) in feces, to monitor in real life, the adherence to GFD in pediatric patients with CD.

Methods: A cross-sectional, prospective study was conducted.

Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.

Background: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency (PI), leading to fat malabsorption, malnutrition, abdominal discomfort and impaired growth. Pancreatic enzyme replacement therapy (PERT) is effective, but evidence based guidelines for dose adjustment are lacking. A mobile app for self-management of PERT was developed in the context of the HORIZON 2020 project MyCyFAPP.

Association between faecal pH and fat absorption in children with cystic fibrosis on a controlled diet and enzyme supplements dose.

Background: Despite treatment with pancreatic enzyme replacement therapy (PERT), patients with cystic fibrosis (CF) can still suffer from fat malabsorption. A cause could be low intestinal pH disabling PERT. The aim of this study was to assess the association between faecal pH (as intestinal pH surrogate) and coefficient of fat absorption (CFA).

Recurrent Metabolic Alkalosis in a Cystic Fibrosis Patient: Coexistence with Congenital Chloride Diarrhea.

Metabolic alkalosis is uncommon in infancy. Cystic fibrosis (CF) patients can develop dehydration because of sweat salt or gastrointestinal losses; with the correct salt supplementation, the electrolyte alterations can be reversed. Here, we present a CF patient with recurrent metabolic alkalosis, initially oriented as pseudo-Bartter's syndrome.

Assessing gastro-intestinal related quality of life in cystic fibrosis: Validation of PedsQL GI in children and their parents.

Background: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency, leading to fat malabsorption, malnutrition and abdominal discomfort. Until recently, no specific tool was available for assessing gastro-intestinal related quality of life (GI QOL) in patients with CF. As the Horizon2020 project MyCyFAPP aims to improve GI QOL by using a newly designed mobile application, a sensitive and reliable outcome measure was needed.

Efficacy Study of Anti-Endomysium Antibodies for Celiac Disease Diagnosis: A Retrospective Study in a Spanish Pediatric Population.

The aim of this study was to assess the efficacy of anti-endomysium antibodies (EMA) as a serological marker for celiac disease (CD) diagnosis in a pediatric population. A retrospective study of pediatric patients who underwent a CD serological markers study: EMA and anti-tissue transglutaminase antibodies (anti-TG2). Clinical symptomatology, degree of histological lesion, human leukocyte antigen (HLA) haplotype compatible with CD (HLA DQ2 and/or DQ8), and final diagnosis were taken into account.

Common Problems Found in the Methodological Approach to Small Bowel Biopsies in the Diagnosis of Celiac Disease.

Small bowel biopsy (SBB) is not always helpful to establish celiac disease diagnosis. Hence we have conducted a retrospective study to know the amount of SBB in our center that was not optimal for this purpose. Histological findings were not appropriate for diagnosis in 3.

The Relative Contribution of Food Groups to Macronutrient Intake in Children with Cystic Fibrosis: A European Multicenter Assessment.

Background: Optimal nutrition for children with cystic fibrosis (CF) improves prognosis and survival, but an increased caloric intake recommendation for this population raises concerns about the nutrient profile of their diets.

Objective: Our aim was to assess the relative contribution of food groups to the total macronutrient intake of European pediatric patients with CF.

Design: We conducted a cross-sectional study in which the participants recorded dietary intake from 2016 to 2017.

Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis.

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.

Detection and quantification of gluten immunogenic peptides in feces of infants and their relationship with diet.

Background: there are no effective methods to easily control the correct adherence to a gluten-free diet (GFD) in celiac disease (CD) patients.

Aim: to assess the sensitivity and specificity of a rapid immunochromatographic (IC) test that detects gluten immunogenic peptides (GIP) in feces, compared to an enzyme-linked immunosorbent assay (ELISA) method.

Methods: fecal samples from healthy infants were analyzed by a rapid IC test and ELISA, both methods are based on the anti-gliadin 33-mer monoclonal antibody.

Pancreatic enzyme replacement therapy in cystic fibrosis: dose, variability and coefficient of fat absorption.

Objectives: Pancreatic enzyme replacement therapy (PERT) remains a backbone in the nutritional treatment of cystic fibrosis. Currently, there is a lack of an evidence-based tool that allows dose adjustment. To date, no studies have found an association between PERT dose and fat absorption.

Nutritional status, nutrient intake and use of enzyme supplements in paediatric patients with Cystic Fibrosis; a European multicentre study with reference to current guidelines.

Background: The New European guidelines have established the most updated recommendations on nutrition and pancreatic enzyme replacement therapy (PERT) in CF. In the context of MyCyFAPP project - a European study in children with CF aimed at developing specific tools for improvement of self-management - the objective of the current study was to assess nutritional status, daily energy and macronutrient intake, and PERT dosing with reference to these new guidelines.

Methods: Cross sectional study in paediatric patients with CF from 6 European centres.