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BACKGROUND/OBJECTIVES: The objective of this disease was to determine the prevalence of malnutrition in children with congenital heart disease (CHD). SUBJECTS/METHODS: A total of 125 children with CHD, under 6 months of age, were divided into four groups: no pulmonary hypertension (PH) or cyanosis (group 1, n = 47), isolated cyanosis (group 2, n = 52), isolated PH (group 3, n = 16), and PH and cyanosis (group 4, n = 10). Six children died at 6 months (n = 4), 12 months (n = 1) and 19 months (n = 1). The remaining children were followed-up for 24 months. Prevalence of moderate to severe malnutrition (weight/weight for height < 80%), caloric intake and medications were compared between the four groups. RESULTS: Moderate or severe malnutrition was more frequent in group 4 (100%) compared with others groups (group 1, 20%; group 2,16.7% and group 3, 50%; P < 0.05). Low oral caloric intake was more frequent in group 3 (71.4%) and group 4 (75%) than in group 1 (28%) and 2 (28.6%) (P < 0.05). Food enrichment was practised in half of the children of group 4 and rarely in other groups (group 1, 15.8%; group 2, 8.6% and group 3, 11.1%; P < 0.05). Enteral feeding was used more often in groups 3 (33.3%) and 4 (50%) than in groups 1 (15.8%) or 2 (14.3%; P < 0.05). CONCLUSIONS: Moderate or severe malnutrition is present in 15% of children with CHD, and it is more frequent in case of PH. Half of these children demonstrate low caloric intake, whereas few have proper nutritional support. European Journal of Clinical Nutrition (2016) 70, 528-531; doi: 10.1038/ejcn.2015.209; published online 23 December 2015, INTRODUCTION The incidence of moderate and severe forms of congenital heart disease (CHD) is about 6/1000 live births. (1) Most of the children have normal birth weight, but they develop [...]

Background: Malnutrition is both a feature and major cause of morbidity in cystic fibrosis (CF). Therefore, nutritional management is an essential element of patient care. In 2016, an international guideline for nutritional management in patients with CF was published. In light of these recommendations, the aim of this study was to investigate the dietary intake of children with CF at the University Hospital of Bordeaux., Methods: We conducted a retrospective study at the Paediatric CF Centre of the University Hospital of Bordeaux. Patients aged 2-18 years with CF who completed a 3-day food diary at home between January 2015 and December 2020 were included., Results: A total of 130 patients, with a median age of 11.8 [interquartile range (IQR): 8.3; 13.4] years, were included. The median Z-score for BMI was -0.35 (IQR: -0.9; 0.2) and 20% of the patients had a Z -score for BMI < -1. Recommended total energy intakes were achieved in 53% of the patients, particularly those with nutritional support. Recommended protein intake was met in 28% of the cases, while fat and carbohydrate intakes were met in 54%. Vitamin and micronutrient levels were normal in 80% of the patients, with the exception of vitamin K, which was within the therapeutic range in only 42% of the cases., Conclusion: Recommended nutritional targets are difficult to achieve in patients with CF, and providing nutritional support during follow-up remains a challenge., Competing Interests: MG, PG, HC, JM, AC, LN, CT and TL report no conflict of interest. FB has received a research grant from AstraZeneca and reports personal fees and non-financial support from Novartis, Chiesi and AstraZeneca. LD and RE are the co-coordinators of the French national cohort “Lum Iva Biota”, which received grants from Vertex. RE also reports a grant from Biocodex, personal fees from Biocodex and Menarini, and non-financial supports from Pfizer, Vertex, MSD, Nutricia, Nestle, Abbvie, Mayoly Spindler, Gilead sciences, Hospira and Aptalis Pharma. MF has received a research and educational grant from Novartis, and reports personal fees as a speaker from GlaxoSmithKine. In addition, MF has 1 co-patent (N°; FR 19/14751: i.e., New in situ pulmonary treatment device in premature babies). SB has received fees for expertise and boards for Zambon and Vertex., (© 2023 Gaschignard, Beaufils, Gallet, Clouzeau, Menard, Costanzo, Nouard, Delhaes, Tetard, Lamireau, Fayon, Bui and Enaud.)

Background: Nutritional status is a major prognostic factor for breathing and the survival of patients with cystic fibrosis (CF). Since 2012, the development of CFTR modulators has considerably transformed the outcome of this disease. Indeed, both lung function and body mass index are improved by CFTR modulators, such as Lumacaftor/Ivacaftor. However, few data exist regarding the outcome of nutritional intakes under Lumacaftor/Ivacaftor., Methods: We conducted a prospective single-center study in children with CF treated with Lumacaftor/Ivacaftor to evaluate their nutritional intake before and after treatment., Results: Thirty-four children were included in this study, with a median age of 12.4 years [11.9; 14.7]. There was no significant improvement in weight, height or BMI. Patients' total energy intake was not significantly changed with Lumacaftor/Ivacaftor, while carbohydrate intakes decreased significantly. We found that blood levels of vitamin E and Selenium were significantly increased under Lumacaftor/Ivacaftor, without a significant increase in supplementation. In patients with a BMI Z-score < 0 at treatment initiation, there was a significant improvement in weight and BMI Z-score, while TEI and carbohydrate intakes were significantly lower., Conclusion: We showed that treatment with Lumacaftor/Ivacaftor improved the nutritional status of patients without necessarily being associated with an increase in nutritional intake. Although these data need to be confirmed in larger cohorts, they support the hypothesis that weight gain under modulators is multifactorial, and may be related to a decrease in energy expenditure or an improvement in absorption., Competing Interests: MG, FLS, PG, HC, JM, AC, LN, CT and TL report no conflict of interest. FB has received a research grant from AstraZeneca and reports personal fees and non-financial support from Novartis, Chiesi and AstraZeneca. LD and RE are the co-coordinators of the French national cohort “Lum Iva Biota”, which received grants from Vertex. RE also reports a grant from Biocodex, personal fees from Biocodex and Menarini, and non-financial supports from Pfizer, Vertex, MSD, Nutricia, Nestle, Abbvie, Mayoly Spindler, Gilead sciences, Hospira and Aptalis Pharma. MF has received a research and educational grant from Novartis, and reports personal fees as a speaker from GlaxoSmithKine. In addition, MF has 1 co-patent (N° 176; FR 19/14751: i.e., New in situ pulmonary treatment device in premature babies). SB has received fees for expertise and boards for Zambon and Vertex., (© 2023 Gaschignard, Beaufils, Lussac-Sorton, Gallet, Clouzeau, Menard, Costanzo, Nouard, Delhaes, Tetard, Lamireau, Fayon, Bui and Enaud.)

Enteral nutrition (EN) allows adequate nutritional intake in children for whom oral intake is impossible, insufficient or unsafe. With maturation and health improvements, most children ameliorate oral skills and become able to eat orally, therefore weaning from EN becomes a therapeutic goal. No recommendations currently exist on tube weaning, and practices vary widely between centres. With this report, the French Network of Rare Digestive Diseases (FIMATHO) and the French-Speaking Group of Paediatric Hepatology, Gastroenterology and Nutrition (GFHGNP) aim to develop uniform clinical practice recommendations for weaning children from EN. A multidisciplinary working group (WG) encompassing paediatricians, paediatric gastroenterologists, speech-language therapists, psychologists, dietitians and occupational therapists, was formed in June 2018. A systematic literature search was performed on those published from January 1, 1998, to April 30, 2020, using MEDLINE. After several rounds of e-discussions, relevant items for paediatric tube weaning were identified, and recommendations were developed, discussed and finalized. The WG members voted on each recommendation using a nominal voting technique. Expert opinion was applied to support the recommendations where no high-quality studies were available., (© 2021. The Author(s), under exclusive licence to Springer Nature Limited.)

Background: Chronic abdominal pain occurs frequently in pediatric patients with inflammatory bowel disease (IBD) in remission., Aims: To assess the prevalence and factors associated with Functional Abdominal Pain Disorders among IBD children in remission (IBD-FAPD)., Methods: Patients with IBD for > 1 year, in clinical remission for ≥ 3 months were recruited from a National IBD network. IBD-FAPDs were assessed using the Rome III questionnaire criteria. Patient- or parent- reported outcomes were assessed., Results: Among 102 included patients, 57 (56%) were boys, mean age (DS) was 15.0 (± 2.0) years and 75 (74%) had Crohn's disease. Twenty-two patients (22%) had at least one Functional Gastrointestinal Disorder among which 17 had at least one IBD-FAPD. Past severity of disease or treatments received and level of remission were not significantly associated with IBD-FAPD. Patients with IBD-FAPD reported more fatigue (peds-FACIT-F: 35.9 ± 9.8 vs. 43.0 ± 6.9, p = 0.01) and a lower HR-QoL (IMPACT III: 76.5 ± 9.6 vs. 81.6 ± 9.2, p = 0.04) than patients without FAPD, and their parents had higher levels of State and Trait anxiety than the other parents., Conclusions: Prevalence of IBD-FAPD was 17%. IBD-FAPD was not associated with past severity of disease, but with fatigue and lower HR-QoL., Competing Interests: Declaration of Competing Interest The authors have no conflict of interest to declare., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objectives: Crohn disease (CD) can affect patient's quality of life (QOL) with physical, social, and psychological impacts. This study aimed to investigate the QOL of children with CD and its relationship with patient and disease characteristics., Methods: Children ages from 10 to 17 years with diagnosed CD for more than 6 months were eligible to this cross-sectional study conducted in 35 French pediatric centers. QOL was assessed by the IMPACT-III questionnaire. Patient and disease characteristics were collected., Results: A total of 218 children (42% of girls) were included at a median age of 14 years (interquartile range [IQR]: 13--16). Median duration of CD was 3.2 years (IQR: 1.7-5.1) and 63% of children were in clinical remission assessed by wPCDAI. Total IMPACT-III score was 62.8 (±11.0). The lowest score was in "emotional functioning" subdomain (mean: 42.8 ± 11.2). Clinical remission was the main independent factor associated with QOL of children with CD (5.74 points higher compared with those "with active disease", 95% confidence interval [CI] 2.77--8.70, P < 0.001). Age of patient at the evaluation was found negatively correlated with QOL (-0.76 per year, 95% CI: -1.47 to -0.06, P = 0.009). Presence of psychological disorders was associated with a lower QOL (-9.6 points lower to those without, 95% CI: -13.34 to -5.86, P < 0.0001). Total IMPACT-III and its subdomains scores were not related to sex, disease duration, or treatments., Conclusions: These results not only confirm that clinical remission is a major issue for the QOL of patients, but also highlights the importance of psychological care., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

In cystic fibrosis (CF), cystic fibrosis transmembrane regulator (CFTR) dysfunction leads to digestive disorders that promote intestinal inflammation and dysbiosis enhancing gastrointestinal symptoms. In pancreatic insufficiency CF patients, both intestinal inflammation and dysbiosis, are associated with an increase in the fecal calprotectin (FC) level. However, associations between the FC level, gastrointestinal symptoms, and quality of life (QoL) remain poorly studied. We aimed to assess such associations in pancreatic insufficiency CF children. The FC level was measured in pancreatic insufficiency CF children's stool samples. Children and their parents completed two questionnaires: The Gastrointestinal Symptoms Scales 3.0-PedsQL TM and the Quality of Life Pediatric Inventory 4.0-PedsQL TM . Lower scores indicated worse symptomatology or QoL. Thirty-seven CF children were included. A FC level above 250 µg/g was associated with worse gastrointestinal symptoms and QoL scores. The FC level was inversely correlated with several gastrointestinal scores assessed by children (i.e., Total, "Heart Burn Reflux", "Nausea and Vomiting", and "Gas and Bloating"). Several QoL scores were correlated with gastrointestinal scores. The FC level was weakly associated with clinical parameters. Some gastrointestinal and QoL scores were related to disease severity associated parameters. In CF, the FC level, biomarker previously related to intestinal inflammation and dysbiosis, was associated with worse digestive symptoms and QoL scores.

Cystic fibrosis (CF) is a systemic genetic disease that leads to pulmonary and digestive disorders. In the majority of CF patients, the intestine is the site of chronic inflammation and microbiota disturbances. The link between gut inflammation and microbiota dysbiosis is still poorly understood. The main objective of this study was to assess gut microbiota composition in CF children depending on their intestinal inflammation. We collected fecal samples from 20 children with CF. Fecal calprotectin levels were measured and fecal microbiota was analyzed by 16S rRNA sequencing. We observed intestinal inflammation was associated with microbiota disturbances characterized mainly by increased abundances of Staphylococcus , Streptococcus, and Veillonella dispar , along with decreased abundances of Bacteroides , Bifidobacterium adolescentis , and Faecalibacterium prausnitzii . Those changes exhibited similarities with that of Crohn's disease (CD), as evidenced by the elevated CD Microbial-Dysbiosis index that we applied for the first time in CF. Furthermore, the significant over-representation of Streptococcus in children with intestinal inflammation appears to be specific to CF and raises the issue of gut-lung axis involvement. Taken together, our results provide new arguments to link gut microbiota and intestinal inflammation in CF and suggest the key role of the gut-lung axis in the CF evolution.

Objectives: Cystic fibrosis-related liver disease (CFLD) can develop silently in early life and approximately 10% of children with cystic fibrosis (CF) become cirrhotic before adulthood. Clinical, biological, and ultrasound criteria used to define CFLD often reveal liver involvement at an advanced stage. The aim of this retrospective study was to assess the progression of liver stiffness measurement (LSM) in pediatric patients with CF., Methods: The change of LSM, expressed as kPa/year and %/year, was measured using transient elastography (Fibroscan) in 82 children with CF (median age: 6.8 years, interquartile range [IQR]: 5.8). Mean time interval between the 2 LSM was 3.5 years., Results: Median initial liver stiffness was 3.7 kPa (IQR: 1.3), and then progressed by 0.23 kPa/year, that is, 6%/year. The 7 patients who developed CFLD had a higher initial level of alanine aminotransferase (50 [IQR: 15] vs 30 [IQR: 18], P = 0.0001) and presented a more rapid progression of LSM (0.94 vs 0.23 kPa/year, P = 0.02)., Conclusions: The present study shows that the slope of worsening of liver stiffness is greater in patients who will develop CFLD, suggesting that annual transient elastography may be useful to detect risk of severe liver disease at an earlier stage.

In cystic fibrosis (CF), approximately 5-8% of the patients develop multilobular cirrhosis during the first decade of life. Annual screening (clinical examination, liver biochemistry, ultrasonography) is recommended in order to identify early signs of liver involvement, initiate ursodeoxycholic acid therapy and detect complications (portal hypertension and liver failure). Management should focus on nutrition and prevention of variceal bleeding. The gut may also be involved in children with CF. Gastroesophageal reflux is frequent, although often neglected and should be investigated by pH monitoring and impedancemetry, if available. Acute pancreatitis occurs in patients with persistent exocrine pancreatic activity. Intussusception, appendicular mucocele, distal intestinal occlusion syndrome, small bowel bacterial overgrowth and Clostridium difficile colitis should be considered in case of abdominal pain. Preventive nutritional support should be started as soon as possible after diagnosis of CF. Attainment of normal growth is one of the main goals and can be achieved with hypercaloric and salt supplemented food. Pancreatic enzyme replacement therapy should be started as soon as exocrine pancreatic insufficiency is confirmed and ingested immediately prior to meals with intake of fat-soluble vitamins. Curative nutritional interventions are more likely to be effective in the early stages of pulmonary disease. Feeding disorders, related to the physiopathology and the psychologic aspects of the disease are frequent. Repeated corporeal aggressions, associated with inappropriate medical and parental pressure, may increase the child's refusal of food. The multidisciplinary team should guide parents in order to avoid all intrusive feeding practices and promote pleasant mealtimes., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Celiac disease is an autoimmune disorder induced by gluten in genetically susceptible individuals. Patients may present with typical symptoms of enteropathy with diarrhoea and failure to thrive, but atypical symptoms, or even silent forms are more often recognized since serologic markers are available. This progress led to the increase of the incidence of the celiac disease during the last 30 years, which reflect probably the improvement in diagnosing atypical forms of the disease. The prevalence is comparable from a country to another, around 0,7 to 2%. There is a progression with time from latent to silent form, and then to the active disease. The exclusion of the gluten from the diet leads to the regression from the active to the latent form of the disease., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Inflammation in Cystic Fibrosis is higher than bacterial clearance needs and contributes significantly to the deterioration of lung tissue and vital prognosis. Its physiology remains controversial and is more complex than the cycle infection-obstruction-inflammation previously described with many interactions and potentiating of the responsible different mechanisms (Mechanical factors, cells, protease/anti-protease, oxidative stress, leukotriens...). This perpetual inflammatory spiral is an important therapeutic target due to its crucial prognosis., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)