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In 2019, the French National Authority for Health (Haute Autorité de Santé, HAS) published guidelines on the diagnosis of undernutrition. The present article focuses on the impact of switching from the 2012 guidelines of the Nutrition Committee of the French Paediatric Society (CNSFP) to the HAS guidelines on the frequency of hospital undernutrition in children. We selected for the period 2010-2019 from the ePINUT database: (1) all children aged more than 2 years with (2) clinically confirmed nutritional status in (3) French sites. The frequency of undernutrition was 15.4% vs. 28.8% using the CNSFP and HAS criteria, respectively (p 0.01; n = 6304). When compared to non-malnourished children regardless of the criteria used, malnourished children: (1) stayed longer in hospital (CNSFP: 9.0 ± 11.8 vs. 6.5 ± 8.7 days, p 0.01; HAS: 7.8 ± 10.1 vs. 6.4 ± 8.4 days, p 0.01), (2) gained more weight during hospitalization (% of weight at admission) (CNSFP: +1.4 ± 4.1 vs. -0.3 ± 3.5%, p 0.01; HAS: +2.3 ± 4.7 vs. -0.1 ± 3.4%, p 0.01), and (3) received nutritional support more frequently during hospitalization (CNSFP: 20% vs. 5%, p 0.01; HAS: 13% vs. 4%, p 0.01). Switching to the HAS guidelines resulted in an almost twofold higher frequency of undernutrition in hospitalized children. Initiation of nutritional care remained low considering the nutritional status. The present study warrants interventional studies to determine which children may benefit more from nutritional therapy to improve their outcome.

OBJECTIVES: To systematically review the current evidence on Helicobacter pylori-negative chronic gastritis including natural history, available therapies and outcomes. METHODS: Articles providing data on the prevalence, treatment or outcomes of Helicobacter pylori-negative gastritis were identified through a systematic search in the MEDLINE and EMBASE databases. All original research articles from human studies until October 31, 2021, were included. RESULTS: A total of 54 studies were included consisted of eosinophilic gastritis (n = 9), autoimmune gastritis (n = 11), collagenous gastritis (n = 16), focally enhanced gastritis (n = 6), lymphocytic gastritis (n = 5) and other causes including idiopathic gastritis and chronic renal failure related (n = 7). Most of the included studies were either cross-sectional or longitudinal cohorts except for collagenous gastritis, which mainly included case reports and case series. The prevalence of paediatric eosinophilic gastritis ranges between 5 and 7/100,000 and patients have generally favourable outcome with 50% to 70% clinical and histological response to either corticosteroids or elimination diets. Autoimmune gastritis and collagenous gastritis are extremely rare entities, commonly present with refractory iron deficiency anaemia, while lymphocytic gastritis is relatively common (10%-45%) in children with coeliac disease. Data on treatments and outcomes of autoimmune, collagenous, and focally enhanced gastritis are lacking with limited data implying poor response to therapy in the former 2 diagnoses. CONCLUSIONS: Helicobacter pylori-negative gastritis is uncommonly reported, mainly in small cohorts, mixed adult-paediatric cohorts or as sporadic case reports. As common symptoms are not specific, thus not always result in an endoscopic evaluation, the true prevalence of these distinct disorders may be underestimated, and thus under reported.

OBJECTIVES/BACKGROUND: Disease-related malnutrition is common in patients with chronic diseases and has detrimental effects, therefore, skills in nutrition care are essential core competencies for paediatric digestive medicine. The aim of this survey, conducted as part of a global survey of paediatric gastroenterology, hepatology and nutrition (PGHN) training in Europe, was to assess nutrition care-related infrastructure, staff, and patient volumes in European PGHN training centres. METHODS: Standardized questionnaires related to clinical nutrition (CN) care were completed by representatives of European PGHN training centres between June 2016 and December 2019. RESULTS: One hundred training centres from 17 European countries, Turkey, and Israel participated in the survey. Dedicated CN clinics exist in 66% of the centres, with fulltime and part-time CN specialists in 66% and 42%, respectively. Home tube feeding (HTF) and home parenteral nutrition (HPN) programmes are in place in 95% and 77% of centres, respectively. Twenty-four percent of centres do not have a dedicated dietitian and 55% do not have a dedicated pharmacist attached to the training centre. Even the largest centres with >5000 outpatients reported that 25% and 50%, respectively do not have a dedicated dietitian or pharmacist. Low patient numbers on HTF and HPN of

International audience; Thiopurines, alone or in combination with other agents, have a pivotal role in the treatment of specific gastrointestinal and hepatological disorders. In inflammatory bowel disease and autoimmune hepatitis thiopurines have proven their value as steroid sparing agents for the maintenance of remission and may be considered for preventing postoperative Crohn disease recurrence where there is moderate risk of this occurring. Their use with infliximab therapy reduces antibody formation and increases biologic drug levels. The routine clinical use of thiopurines has, however, been questioned due to a number of potential adverse effects. The aim of this article is to provide information regarding the use, and in particular, safety of these agents in clinical practice in the light of such potentially severe, albeit rare, effects.

Background: The combination of the CFTR corrector Lumacaftor (LUM) and potentiator Ivacaftor (IVA) has been labelled in France since 2015 for F508del homozygote cystic fibrosis (CF) patients over 12 years. Aims: Main: to compare the changes in ppFEV1 2 years before and after initiation of LUM/IVA treatment. Secondary: to assess (i) the evolution of clinical (weight, height, BMI, pulmonary exacerbations, antibiotic use), biological (sweat chloride concentration) and radiological parameters; (ii) to identify parameters associated with response to treatment; (iii) to assess the tolerance to treatment. Methods: In this tri-centre non-interventional observational cohort study, children (12-18 years old) were assessed prospectively during the two years of therapy, and retrospectively during the two years preceding treatment. Data collected and analysed for the study were exclusively extracted from the patients' medical electronic system. Findings: Forty adolescents aged 12.0 to 17.4 years at LUM/IVA initiation were included. Regarding ppFEV1, a decrease was present during the two years prior to treatment, followed by a significant increase after LUM/IVA initiation, becoming significant after 2 years of treatment. LUM/IVA significantly improved the BMI Z-score and sweat chloride concentration. By contrast, there was no significant change in exacerbation rates, antibiotic use or CT-scan scores. Age at LUM/IVA initiation was lower in good responders and was associated with greater ppFEV1 change during the 2 years of treatment. LUM/IVA was well tolerated. Interpretation: In F508del homozygote adolescents, real-life long-term LUM/IVA improves the ppFEV1 trajectory (in particular if introduced early), nutritional status and sweat chloride concentration but not exacerbation rates or radiological scores. LUM/IVA was generally well tolerated and safe. Funding Information: This study did not receive any specific funding. Declaration of Interests: SB, FG, CC, RE, FB, MF conduct clinical trials with Vertex pharmacological agents, on behalf of the European Cystic Fibrosis Society – Clinical Trials Network (ECFS-CTN) and within the scope of ECFS-CTN activities. The other authors have no conflicts of interest to declare. Ethics Approval Statement: The use of the data collected and analysed for the study were exclusively extracted from the patients' medical records (MUCODOMEOS, https://www.vaincrelamuco.org/2019/05/09/mucodomeos-un-logiciel-adapte-aux-besoins-des-crcm-2684) after having obtained their informed consent. In this context and according to the French law in force, the approval of an ethics committee was not required.

International audience; Octreotide, a somatostatin analogue, has been used for more than 20 years in children with gastrointestinal bleeding, chylothorax or chylous ascites, intestinal lymphangiectasia, pancreatitis, intestinal dysmotility, and severe diarrhoea; however, until now, there is a lack of randomised clinical trials evaluating the efficacy of this compound in childhood. Hence, we aimed to review the literature in order to determine the evidence of its use and safety in children, using PubMed from 2000 to 2021 with the search terms "octreotide" and "children" and "bleeding or chylous ascites or chylothorax or acute pancreatitis or lymphangiectasia or diarrhoea or intestinal dysmotility".

International audience; Objectives: Given a lack of a systematic approach to the use of breath testing in paediatric patients, the aim of this position paper is to provide expert guidance regarding the indications for its use and practical considerations to optimise its utility and safety. Methods: Nine clinical questions regarding methodology, interpretation, and specific indications of breath testing and treatment of carbohydrate malabsorption were addressed by members of the Gastroenterology Committee (GIC) of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN). A systematic literature search was performed from 1983 to 2020 using PubMed, the MEDLINE and Cochrane Database of Systematic Reviews. Grading of Recommendations, Assessment, Development, and Evaluation was applied to evaluate the outcomes. During a consensus meeting, all recommendations were discussed and finalised. In the absence of evidence from randomised controlled trials, recommendations reflect the expert opinion of the authors. Results: A total of 22 recommendations were voted on using the nominal voting technique. At first, recommendations on prerequisites and preparation for as well as on interpretation of breath tests are given. Then, recommendations on the usefulness of H2-lactose breath testing, H2-fructose breath testing as well as of breath tests for other types of carbohydrate malabsorption are provided. Furthermore, breath testing is recommended to diagnose small intestinal bacterial overgrowth (SIBO), to control for success of Helicobacter pylori eradication therapy and to diagnose and monitor therapy of exocrine pancreatic insufficiency, but not to estimate oro-caecal transit time (OCTT) or to diagnose and follow-up on celiac disease. Conclusions: Breath tests are frequently used in paediatric gastroenterology mainly assessing carbohydrate malabsorption, but also in the diagnosis of small intestinal overgrowth, fat malabsorption, H. pylori infection as well as for measuring gastrointestinal transit times. Interpretation of the results can be challenging and in addition, pertinent symptoms should be considered to evaluate clinical tolerance.

International audience; Button batteries (BB) remain a health hazard to children as ingestion might lead to life-threatening complications, especially if the battery is impacted in the esophagus. Worldwide initiatives have been set up in order to prevent and also timely diagnose and manage BB ingestions. A European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) task force for BB ingestions has been founded, which aimed to contribute to reducing the health risks related to this event. It is important to focus on the European setting, next to other worldwide initiatives, to develop and implement effective management strategies. As one of the first initiatives of the ESPGHAN task force, this ESPGHAN position paper has been written. The literature is summarized, and prevention strategies are discussed focusing on some controversial topics. An algorithm for the diagnosis and management of BB ingestions is presented and compared to previous guidelines (NASPGHAN, National Poison Center). In agreement with earlier guidelines, immediate localization of the BB is important and in case of esophageal impaction, the BB should be removed instantly (preferably 12 hours after ingestion or time point of removal >12 hours after ingestion) and esophageal impaction the guideline suggests to perform a CT scan in order to evaluate for vascular injury before removing the battery. In delayed diagnosis, even if the battery has passed the esophagus, endoscopy to screen for esophageal damage and a CT scan to rule out vascular injury should be considered even in asymptomatic children. In asymptomatic patients with early diagnosis (

The current craze for vegan diets has an effect on the pediatric population. This type of diet, which does not provide all the micronutrient requirements, exposes children to nutritional deficiencies. These can have serious consequences, especially when this diet is introduced at an early age, a period of significant growth and neurological development. Even if deficiencies have less impact on older children and adolescents, they are not uncommon and consequently should also be prevented. Regular dietary monitoring is essential, vitamin B12 and vitamin D supplementation is always necessary, while iron, calcium, docosahexaenoic acid, and zinc should be supplemented on a case-by-case basis.

International audience; Gastrointestinal symptoms are common findings in children with severe acute respiratory syndrome coronavirus 2 infection, including vomiting, diarrhoea, abdominal pain, and difficulty in feeding, although these symptoms tend to be mild. The hepato-biliary system and the pancreas may also be involved, usually with a mild elevation of transaminases and, rarely, pancreatitis. In contrast, a late hyper-inflammatory phenomenon, termed multisystem inflammatory syndrome (MIS-C), is characterized by more frequent gastrointestinal manifestations with greater severity, sometimes presenting as peritonitis. Gastrointestinal and hepato-biliary manifestations are probably related to a loss in enterocyte absorption capability and microscopic mucosal damage caused by a viral infection of intestinal epithelial cells, hepatocytes and other cells through the angiotensin conversion enzyme 2 receptor resulting in immune cells activation with subsequent release of inflammatory cytokines. Specific conditions such as inflammatory bowel disease (IBD) and liver transplantation may pose a risk for the more severe presentation of coronavirus disease 2019 (COVID-19) but as adult data accumulate, paediatric data is still limited. The aim of this review is to summarize the current evidence about the effect of COVID-19 on the gastrointestinal system in children, with emphasis on the emerging MIS-C and specific considerations such as patients with IBD and liver transplant recipients.

International audience; Acute diarrhoea is a leading cause of morbidity and mortality in the paediatric population. Racecadotril is an antisecretory drug recommended as an adjuvant anti-diarrhoeal treatment.In the small bowel, the enzyme neutral endopeptidase (NEP) inhibits the action of enkephalins, which prevent water and electrolyte hypersecretion. By inhibiting NEP, racecadotril allows enkephalins to exhibit their antisecretory effects. Consequently, racecadotril reduces the secretion of water and electrolytes in the small intestine, without having an effect on intestinal motility. No serious adverse events related to racecadotril have been reported.Racecadotril has proven its efficacy as an adjuvant anti-diarrhoeal drug with a good safety profile. Its addition to oral rehydration solution (ORS) appears clinically beneficial and potentially leads to health care savings.

International audience

International audience; OBJECTIVES: The use of semielemental diets concerns a small proportion of children on enteral nutrition whose characteristics have never been reported. Our aim was to describe a cohort of patients on home enteral nutrition with Peptamen Junior, including the tolerance and nutritional efficacy of this product. METHODS: We performed a retrospective multicenter survey on a cohort of patients receiving this semielemental diet at home between 2010 and 2015 in 14 tertiary pediatric French centers. We recorded at baseline, 3, 6, and 12 months, and then every year the anthropometric characteristics of the patients, indications and modalities of administration of the diet, and the tolerance and adverse events. RESULTS: We recruited 136 patients ages 9.8 +/- 4.4 years at baseline. Mean body mass index z score was -1.0 +/- 1.8; mean height z score was -1.1 +/- 1.9. The main underlying diseases were digestive (35.3%), neurological (33.1%), and hematological (19.9%). The indications for a semielemental diet were failure of another diet in 70 patients (51.9%), severe malnutrition in 19 (14.1%), cystic fibrosis in 11 (8.1%), and switch from parenteral nutrition in 11 (8.1%). Side effects were observed in 39.2% of the patients, and required medical attention in 8.2%. Body mass index improved or remained normal in 88.3% of children. CONCLUSIONS: This semielemental diet seems to be well tolerated and efficient in the setting of home enteral nutrition in children with complex diseases featuring malabsorption and/or after failure of polymeric diet.

International audience

The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Polyposis Working Group developed recommendations to assist clinicians and health care providers with appropriate management of patients with juvenile polyposis. This is the first juvenile polyposis Position Paper published by ESPGHAN with invited experts. Many of the published studies were descriptive and/or retrospective in nature, consequently after incorporating a modified version of the GRADE system many of the recommendations are based on expert opinion. This ESPGHAN Position Paper provides a guide for diagnosis, assessment, and management of juvenile polyposis syndrome in children and adolescents, and will be helpful in the appropriate management and timing of procedures in children and adolescents. The formation of international collaboration and consortia is proposed to monitor patients prospectively to advance our understanding of juvenile polyposis conditions.

International audience; Pseudomonas aeruginosa and Staphylococcus aureus toothbrush contamination in cystic fibrosis (CF) is unknown. This pilot study aimed to determine their prevalence and the potential involvement of toothbrushes in pulmonary infection. Methods. Toothbrush bacteriological analysis for children aged 8–18 years was conducted on 27 CF patients, 15 healthy siblings, and 15 healthy children from the general population. Results : S. aureus was detected on 22% of the patients’ toothbrushes, and 13% of healthy children's toothbrushes and P. aeruginosa on 15% of patients’ toothbrushes and 0–13% of healthy children's toothbrushes. There was no statistical correlation between pulmonary colonization and toothbrush contamination. P. aeruginosa genotyping showed two identical clones on the patients’ toothbrushes and in their sputum, and between one patient's sputum and his sibling's toothbrush. Conclusion : S. aureus and P. aeruginosa can colonize CF patients’ toothbrushes. The impact on pulmonary colonization remains unknown. Toothbrush decontamination methods need to consider these bacteria in CF patients.

Digestive complications related to the ingestion of magnetic foreign bodies in children are increasing, especially in Asia and North America. In France, several case reports have been reported since 2008. We conducted a retrospective multicentric study to evaluate the frequency of ingestion of magnet foreign bodies and to describe the complicated cases that have occurred in France over the last 5 years. We report 40 cases of which 60% were multiple magnet ingestions. Eighty-eight percent of the children of the group who had swallowed multiple magnets needed interventional management by endoscopy (33%) or surgery (58%). Only two children (12.5%) of the group who swallowed one magnet required removal. This problem is not uncommon in France (2% of the 1132 foreign bodies investigated in the Toulouse center over 5 years), which justifies clear information for healthcare professionals and caregivers in order to avoid potential intestinal complications. We suggest interventional management or very close monitoring in the cases of multiple magnet ingestion. Meanwhile, in the majority of confirmed cases of simple magnet ingestions, we propose home monitoring.

Resume Le test de concordance de scripts (TCS) est un moyen d’evaluer les processus hypothetico-deductifs qui constituent le raisonnement clinique. Un TCS en gastro-enterologie pediatrique peut permettre d’evaluer objectivement les etudiants en medecine a la fin de leur stage. Le but de ce travail etait d’evaluer les difficultes rencontrees dans l’elaboration d’un TCS et l’interet de cet outil pour apprecier le raisonnement clinique des etudiants. Un TCS a ete elabore comprenant 10 vignettes et 31 questions relatives aux sujets de l’examen national classant et a ete utilise pour evaluer 5 etudiants du 2 e cycle des etudes medicales a la fin de leur stage en pediatrie. Compte tenu des personnes examinees, le groupe d’experts a ete constitue par 10 internes. Les corrections ont ete etablies selon la reponse modale. Selon la distribution des reponses des experts a chaque item, les ancrages ont ete consideres corrects, anormaux ou discutables. Le TCS a ete effectue aussi par 5 gastro-enterologues pediatres. Les resultats ont ete exprimes en moyenne/20 ± ESM (ecart standard a la moyenne). L’analyse statistique a constitue en un test t de Student. Les notes ont ete de 15,35 ± 0,38 et 12,44 ± 0,58 ( p = 0,0006), respectivement pour les experts et pour les etudiants. Les analyses complementaires ont montre que l’evaluation n’etait pas modifiee par des items ou des ancrages anormaux ou discutables. La moyenne des gastro-enterologues pediatres etait plus basse : 13,37 ± 0,69 ( p = 0,01). La fiabilite de ce TCS pourrait etre discutee. Les etudiants ont juge ce test adapte a son but, meme si toutes les personnes etaient evaluees pour la premiere fois par un TCS. Les TCS sont un outil interessant mais leur elaboration est complexe. Elle impose de constituer un groupe d’enseignants pour suivre les recommandations actuelles et eviter une variabilite trop importante.

In children, caustic ingestion is due to accidents at home and inadequate storage of caustic agents. In emergency, it is useful to remove the soiled clothes, rinse the affected area, and prevent vomiting and feeding. Caustic ingestion (pH 12) induces burns of the upper gastrointestinal tract requiring esophagogastro-duodenoscopy between H12 and H24. Strong alkalis cause necrosis with liquefaction of the esophagus, penetrating deeply with a high-risk of perforation. Management of these children requires a specialized care center with an intensive care unit, endoscopic equipment, and a surgical team. Esophageal stricture is the main complication; no prophylactic treatment (steroids) is effective. Strictures occur after the 3rd week, and barium swallow should be performed by the end of the 1st month. Stricture are often multiple, long, and tortuous; endoscopic dilatation is difficult with a high-rate of perforation and a low-rate of success. In situ application of mitomycin C or injection of triamcinolone could reduce the recurrence rate of stricture. In recalcitrant or recurrent strictures, it is recommended to perform an esophageal replacement using a colonic interposition or a gastric tube. Endoscopy should also be performed 15-20years after caustic ingestion to screen for early neoplastic lesions. Prevention is very important for avoiding caustic ingestions. Information and education should be given specifically to the parents of toddlers; caustic products should be stored out of reach of children and they should not be kept with food.

La fibroscopie œsogastroduodenale (FOGD) est l’examen de reference pour rechercher des varices oesophagiennes (VO). Nous rapportons la premiere etude pediatrique permettant de valider la videocapsule oesophagienne (VCO) chez l’enfant. Patients et methodes De novembre 2011 a juillet 2013, 102 patients âges de 7 a 18 ans ayant une hypertension portale (HTP) et/ou une cirrhose etaient selectionnes. La VCO (PillCam ® ESO 2, Given Imaging) et la FOGD etaient realisees dans un delai d’une semaine. Pour les VCO la classification de Schreibman modifiee etait utilisee. L’analyse statistique a ete realisee avec le logiciel SPSS. Le critere principal etait la sensibilite (Se) d’un examen par VCO par rapport a la FOGD sous anesthesie generale (AG). Les objectifs secondaires etaient l’etude de la specificite (Sp), la valeur predictive positive (VPP) et negative (VPN) de la VCO, la presence de varices gastriques (VG) et d’une gastropathie d’HTP (GHTP), la tolerance et la faisabilite. Resultats Quatre-vingt-un enfants etaient inclus et 16 recuses (echec du test au bonbon). L’âge moyen etait de 13,96 ± 0,25 ans. Concernant la presence de VO la Se de la VCO etait de 90,5 %, Sp : 100 %, VPP : 100 % et VPN : 75 %, avec un indice Kappa de concordance entre la VCO et la FOGD de 0,842. La VCO permettait de bien classer 73/81 patients avec un indice Kappa de 0,851. Concernant la presence d’une GHTP ( n = 70), la Se etait de 98,2 %, Sp : 53,8 %, VPP : 90,3 %, VPN : 87,5 % et un indice Kappa de 0,612. Pour les VG (43 cas analysables) la Se etait de 58,3 %, Sp : 90,3 %, VPP : 70 %, VPN : 84,4 % et un indice Kappa de 0,513. Pour les enfants sans VO connues ( n = 21), la VCO obtenait une Se de 100 %, Sp : 100 % avec un indice Kappa de 1. Pour le controle d’eradication apres ligature de VO (LVO) ( n = 24), la VCO indiquait ou non une nouvelle LVO dans 16/24 cas, avec un indice Kappa de 0,80. Tous les patients preferaient la VCO a la FOGD, aucune retention de capsule n’etait enregistree. Conclusions En utilisant la classification de Schreibman modifiee, la VCO a une Se de 90,5 % dans le depistage des VO, permettant de confirmer que la VCO peut etre utilisee pour le diagnostic et la surveillance des VO chez l’enfant. Une des limites est la mauvaise visualisation des VG. Un test au bonbon reussi est un prealable obligatoire.

But Evaluer l’impact du depistage neonatal de la mucoviscidose sur l’evolution nutritionnelle precoce. Methode Etude retrospective monocentrique realisee du 1/10/02 au 31/12/12 qui a evalue au moment du diagnostic et vers un an les parametres nutritionnels cliniques et biologiques. L’analyse a ete faite en 3 sous-groupes en fonction de la valeur de l’elastase fecale initiale : suffisant (SP), intermediaire et insuffisant pancreatique (IP), respectivement elastase > 200, 15–200 et t de Student non apparie pour les variables continues, exprimees en moyenne ± DS, et en un test de Chi 2 pour les variables categorielles, exprimees en pourcentage. Resultats Cinquante-deux enfants ont ete diagnostiques durant cette periode dont 43 par depistage neonatal (7 pathologies chirurgicales neonatales et 2 faux negatifs). Ils etaient repartis en 8 SP (17 %), 10 intermediaires (21 %) et 28 IP (59 %). Lors de l’annonce, a 37 ± 14 jours, le poids etait significativement plus bas dans le groupe IP (−1,13 ± 1,18 DS, p p Au niveau biologique, les valeurs au diagnostic de l’albumine et du zinc serique etaient plus basses dans le groupe IP. Au cours du suivi, a ete notee une augmentation des taux d’acide eicosapentaenoique et une diminution du taux d’acide docosahexaenoique quel que soit le statut pancreatique. Concernant les vitamines liposolubles, la principale difference concernait la vitamine E dont le taux etait significativement plus bas au diagnostic dans le groupe IP vs. SP, respectivement de 5,8 ± 4,2 vs. 11,1 ± 3,4 mg/L ( p = 0,01). Conclusion Le depistage neonatal de la mucoviscidose a permis une correction a un an des anomalies nutritionnelles presentes au moment du diagnostic. Toutefois, une attention particuliere doit etre portee aux nourrissons ayant une fonction pancreatique « intermediaire », dont le statut nutritionnel a un an etait moins bon.

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.; Environ 5 à 8% des patients atteints de mucoviscidose développent une cirrhose multilobulaire au cours de la première décennie. Le dépistage annuel de signes d'atteinte hépatique par l'examen clinique, les tests biologiques hépatiques, l'échographie et le suivi sont recommandés afin d'instaurer un traitement par acide ursodésoxycholique et d'identifier les complications: hypertension portale et insuffisance hépatocellulaire. La correction de la malnutrition et la prévention de l'hémorragie digestive sont les principaux objectifs de la prise en charge.Différentes atteintes digestives peuvent être rencontrées chez ces enfants. Le reflux gastro-œsophagien, très fréquent mais souvent négligé, doit être recherché par pH-métrie couplée éventuellement à une impédancemétrie. Des épisodes de pancréatite aigüe peuvent survenir chez des patients ayant une activité pancréatique exocrine résiduelle suffisante. Invagination intestinale aigüe, mucocèle appendiculaire (accumulation de mucus épais dans l'appendice), syndrome d'occlusion intestinale distale (impaction de selles et de mucus au niveau iléo-caecal), syndrome de pullulation bactérienne de l'intestin grêle et colite à Clostridium difficile sont à évoquer devant des douleurs abdominales.Sur le plan nutritionnel, la prise en charge préventive et la surveillance attentive de la croissance débutent dès le diagnostic. L'alimentation, tout en restant équilibrée, doit être hyper-énergétique et supplémentée en sel. Les extraits pancréatiques lors des repas sont prescrits dès l'insuffisance pancréatique exocrine confirmée, de même que les vitamines liposolubles. En cas de dénutrition, la prise en charge nutritionnelle curative par assistance nutritionnelle est d'autant plus efficace qu'elle est précoce, chez des patients à l'état respiratoire satisfaisant.Les difficultés d'alimentation sont très fréquentes, liées à la physiopathologie de la maladie mais également à son retentissement psychologique. Le vécu corporel négatif de l'enfant, la pression médicale et parentale parfois excessive peuvent majorer les refus alimentaires. L'équipe multidisciplinaire doit veiller à proscrire toute attitude contre-productive de nourrissage intrusif et valoriser l'instauration d'un climat de plaisir au cours des repas.

Since the discovery of chloride secretion by the Cystic Fibrosis Transport regulator CFTR in 1983, and CFTR gene in 1989, knowledge about CFTR synthesis, maturation, intracellular transfer and function has dramatically expanded. These discoveries have led to the distribution of CF mutations into 6 classes with different pathophysiological mechanisms. In this article we will explore the state of art on CFTR synthesis and its chloride secretion function. We will then explore the consequences of the 6 classes of mutations on CFTR protein function and we will describe the new therapeutic developments aiming at correcting these defects.

International audience; Evaluation of serum lipase as predictor of severity of acute pancreatitis in children. [J Pediatr Gastroenterol Nutr. 2014]Serum lipase as an early predictor of severity in pediatric acute pancreatitis.

Background: Allergic bronchopulmonary aspergillosis (ABPA) is a common complication among patients with CF that produces significant respiratory morbidity. Standard therapy for ABPA includes systemic corticosteroids for a prolonged period of time with potentially severe side effects and use of antifungal agents. Omalizumab is a humanized monoclonal antibody directed against IgE that prevents its binding to receptors on effector cells. It has been shown to be effective in improving asthma control in patients with a strong allergic component. Recently, sporadic reports of omalizumab use against ABPA have been reported. Methods: We present our long term experience of omalizumab in three children with CF and ABPA who were steroid dependent. Results: Five (3.4%) out of 148 children and adolescents with CF followed in our Unit over a seven year period, developed ABPA (75% females, mean age at start of therapy 14.3 years). Omalizumab was given to 3 of them (2 males, mean age 17.1 years, mean FEV1 35%). All three were already experiencing significant side effects from chronic steroid therapy (invasive aspergillosis, diabetes mellitus, depression, relapse of ABPA and severe osteoporosis) and received regular antifugal therapy. Omalizumab treatment was given for a period of 12−18 months. Oral steroids were gradually stopped. Omalizumab resulted in significant improvement of respiratory symptoms and lung function (mean FEV1 52%, p: 0.01). Conclusion: According to our results in a small number of patients, omalizumab has a significant potential as a strategy to control ABPA in steroid dependent patients. However, more studies are needed to confirm these findings.

International audience

Severity scores are used to predict the outcome of acute pancreatitis (AP). Several scores are used in adult patients, but none has been thoroughly validated for specific use in paediatric patients. We retrospectively collected data from 48 children with AP (13 severe and 35 mild). The main causes were trauma (23%), idiopathic (23%), lithiasis (12.5%), and virus (10.5%). We evaluated 3 clinical scores (Ranson, Glasgow modified, and DeBanto) and Balthazar computed tomography severity index. The clinical scores had a good specificity (approximately 85%) but a low sensitivity (approximately 55%) in predicting the severity of paediatric AP. The radiological score is better (sensitivity 80%, specificity 86%). The area under the receiver operator characteristic curve was 0.699 (95% CI 0.508%-0.891%, P = 0.054) for the DeBanto score, 0.846 (95% CI 0.69%-1%, P = 0.001) for the Ranson score, and 0.774 (95% CI 0.584%-0.964%, P = 0.008) for the Glasgow and 0.898 (95% CI 0.73%-1%, P = 0.011) for the Balthazar computed tomography severity index score. In our paediatric cohort, the severity of AP was best predicted by Balthazar computed tomography-based scoring scale. Our results confirm previously reported low sensitivity of adult-based clinical scoring scales.

Objectifs Les cellules souches intestinales (CSI) permettent le developpement de mini-intestins in vitro . Notre but etait de cultiver et caracteriser des colonospheres humains, monocouche de cellules epitheliales organisee en 3D. Materiels et methodes A partir de resection colique, les cryptes sont re-suspendues dans du Matrigel puis ensemencees. Un milieu complet est ajoute pour reconstituer le microenvironnement des CSI, remplace par un milieu de differenciation pendant 5 jours. Les colonospheres ont ete fixes a J8 pour analyse immunohistochimique. Resultats principaux Les colonospheres ont un pole apical (actine +) oriente vers une lumiere centrale et un pole baso-lateral (β-catenine +). Il y a une zone apoptotique centrale (caspase 3 active +) et des zones de proliferation (Ki-67 +) ; des cellules immatures (CD44 et CD24 +) et des cellules differenciees : Goblet (mucine 2 +), enteroendocrines (chromogranine A +) et colonocytes (cytokeratine 20 +). Apres differenciation, nous retrouvons une diminution de cellules immatures et l’expression d’occludine (jonctions serrees), dans des zones distinctes. Conclusions Ce modele est bien etabli dans nos mains, avec des perspectives de recherche sur les maladies inflammatoires chroniques de l’intestin.

Objectifs Evaluer le degre de denutrition d’enfants ayant une affection maligne a haut risque et les circonstances d’utilisation d’une nutrition enterale (NE). Sujets et methodes C’est une etude retrospective monocentrique sur 2 ans d’enfants Resultats principaux Nous avons inclus 108 enfants, âge moyen 8,2 ans : tumeurs cerebrales de haut grade (28) et bas grade (9), Hodgkin (18), tumeurs osseuses (16), Wilms (13), sarcomes des tissus mous (7), neuroblastomes (7), lymphomes non Hodgkiniens (7), hepatoblastomes (7) et 1 tumeur germinale. Au cours des 6 mois de suivi, 21 sont decedes. Selon le rapport P/T, 26% des enfants etaient denutris au diagnostic et 30% a 6 mois, avec plus de denutrition severe a 6 mois. La NE (24% des enfants ; 40% de gastrostomie) etait debutee rapidement (46% 3 mois). Conclusions La denutrition est frequente et sa severite s’aggrave au cours des premiers mois, necessitant une NE. La pose d’une gastrostomie parait justifiee au diagnostic dans certains cas.

Introduction: Physical activity is beneficial for cystic fibrosis (CF) patients. This induces to stimulate their habitual physical activity (HAP) from childhood. Thus, for HAP evaluation, we need methods that are simple, reliable, objective and well accepted by the patients. Accelerometry is one of the possibilities for evaluating HAP. We studied its feasibility, acceptability and correlation with other HAP evaluation methods in a population of paediatric CF patients. Methods: 34 CF patients, aged between 9 and 18 years, were proposed to wear accelerometers. The reasons for not wearing the accelerometer were evaluated. A correlation was measured between the data produced by accelerometry, the answers to HAES and Veerschuur questionnaires, and evaluations by medical staff. Results: – 14 children out of 34 (41%) did not wear the accelerometer, 5 of them simply refused it; 6 children (17%) have worn the accelerometer less than 5 days. Finally, 14 recordings were interpretable, i.e. 41% of the 34 children to whom the measurement was proposed. – The answers to the questionnaires appeared not to be correlated to the accelerometry data. But there was a good correlation of 62.1% (p = 0.017) between the data obtained by accelerometry and the medical evaluation. Conclusion: The feasibility of the HAP evaluation by accelerometry in a pediatric CF population is only around 40%. Therefore it cannot be used in every-day clinical practice. However, in clinical trials, the objective evaluation by accelerometry should be preferred because of the discrepancies observed with answers to standardized questionnaires on physical activity.