Esophageal varices (EV) are a complication of cystic fibrosis-associated liver disease. Esophagogastroduodenoscopy (EGD) is currently used to diagnose varices but is invasive for pediatric patients. ...The goal of this study was to explore the relationship between transient elastography (FibroScan®) and the presence of EV in patients with liver disease defined by clinical, laboratory, sonographic, and/or endoscopic criteria.
18 patients with cystic fibrosis underwent EGD and transient elastography. 12 patients had EV.
Patients with EV had higher FibroScan values than those without varices with median values of 22.4 kPa (14.4-30.4 kPa) vs. 7.9 kPa (4.4-13.7 kPa) (p=0.01). Using a threshold of 12 kPa, four of six patients without EV would not have needed EGD.
Elastography should be recommended for all cystic fibrosis patients with liver disease to follow its progression. A prospective study is needed to define an elastography threshold value that predicts the presence of EV.
Eosinophilic esophagitis (EE) is a recent pathology defined by abnormal immune response of the esophageal mucosa to exogenous allergens, leading to chronic mucosa infiltration by 15 eosinophils per ...High-Power-Field (Eos/HPF). The present retrospective study was designed to assess the hospital care for children suffering from EE in several hospitals in western France in order to highlight discrepancies and improve future care. Twenty-eight children ranging from 1.5 months to 17 years old were included in the study. Episodes of food blockage were the most frequently reported symptoms (46 %). A ratio of 29 % of EE patients reported macroscopically normal endoscopy; diagnosis was then established upon histological anomalies found in biopsies. The mean eosinophil count was 72.4 Eos/HPF. Centralized immunohistochemical staining revealed the presence of IgG4-responding plasma cells in 76.5 % of patients, as well as IgG4 intraepithelial degranulation in 14 % of them. The evaluation of the treatment plan showed important inter-center discrepancies with only 43 % of patients receiving endoscopic reevaluation. This study objectively highlights heterogeneities in diagnosis and care provided to children suffering from EE. Therefore, improving the consistency of practices seems to be crucial to optimize the patients' outcome. The role of IgG4 as a new diagnosis marker remains to be clarified.
To assess knowledge acquired by adolescents about their inflammatory bowel disease (IBD).
An anonymous questionnaire was given during consultation to adolescents followed for IBD by pediatricians ...from 13 hospitals between 1 September 2012 and 1 July 2013. After parental consent, these physicians completed a form at the inclusion of each patient, in which the characteristics of IBD were detailed. The patients mailed back their questionnaire.
A total of 124 patients from 12 to 19 years of age were included with a response rate of 82% (all anonymous); 23% of the patients thought that diet was a possible cause of IBD and 22% that one of the targets of their treatment was to cure their disease for good. Of the patients reported having Crohn disease, 46% knew the anoperineal location and 14% knew that Crohn disease can affect the entire digestive tract. Twenty-five percent of the patients were able to name one side effect of azathioprine (88% had already received this treatment), 24% were able to name one side effect of infliximab (54% had already received this treatment), 70% of the adolescents knew that smoking worsens Crohn disease, 68% declared they had learned about their IBD from their pediatrician, and 81% said they would like to receive more information.
Adolescents with IBD have gaps in their general knowledge and the different treatments of their disease. Their main source of information is their pediatrician, warranting the implementation of customized patient education sessions.
Abstract Background Esophageal varices (EV) are a complication of cystic fibrosis-associated liver disease. Esophagogastroduodenoscopy (EGD) is currently used to diagnose varices but is invasive for ...pediatric patients. The goal of this study was to explore the relationship between transient elastography (FibroScan®) and the presence of EV in patients with liver disease defined by clinical, laboratory, sonographic, and/or endoscopic criteria. Methods 18 patients with cystic fibrosis underwent EGD and transient elastography. 12 patients had EV. Results Patients with EV had higher FibroScan values than those without varices with median values of 22.4 kPa (14.4-30.4 kPa) vs. 7.9 kPa (4.4-13.7 kPa) (p=0.01). Using a threshold of 12 kPa, four of six patients without EV would not have needed EGD. Conclusions Elastography should be recommended for all cystic fibrosis patients with liver disease to follow its progression. A prospective study is needed to define an elastography threshold value that predicts the presence of EV.
L’œsophagite à éosinophiles (OE) est une affection récemment décrite, définie par une réponse immunitaire anormale de la muqueuse œsophagienne vis-à-vis d’allergènes exogènes se traduisant par une ...infiltration chronique de cette muqueuse par des polynucléaires éosinophiles>15 polynucléaires éosinophiles par champ au fort grossissement (PNEo/CFG). Cette étude rétrospective avait pour but d’évaluer la prise en charge d’enfants atteints d’OE dans 5 centres hospitaliers de l’Ouest de la France, afin d’en montrer la disparité et d’améliorer les pratiques futures. Vingt-huit enfants âgés de 1,5 mois à 17 ans ont été inclus. Le blocage alimentaire avait été le symptôme le plus fréquemment rapporté (46 %). L’endoscopie s’était avérée macroscopiquement normale chez 29 % des patients : le diagnostic avait alors été posé sur les anomalies histologiques des biopsies. Le taux moyen d’éosinophiles dans la muqueuse œsophagienne était de 72,4 PNEo/CFG. Un immunomarquage centralisé avait permis d’identifier la présence de plasmocytes positifs à immunoglobulines G de type 4 (IgG4) chez 76 % des patients et une dégranulation intra-épithéliale d’IgG4 chez 14 % d’entre eux. L’évaluation de la prise en charge thérapeutique a identifié des divergences importantes entre les centres, une réévaluation endoscopique n’étant réalisée que chez 43 % des patients. Cette étude a permis d’objectiver l’hétérogénéité dans le diagnostic et la prise en charge des enfants atteints d’OE. Il paraît indispensable d’uniformiser les pratiques afin de mieux comprendre cette pathologie et d’en améliorer la prise en charge. La place des IgG4 comme nouveau marqueur diagnostique reste à préciser.
Eosinophilic esophagitis (EE) is a recent pathology defined by abnormal immune response of the esophageal mucosa to exogenous allergens, leading to chronic mucosa infiltration by 15 eosinophils per High-Power-Field (Eos/HPF). The present retrospective study was designed to assess the hospital care for children suffering from EE in several hospitals in western France in order to highlight discrepancies and improve future care. Twenty-eight children ranging from 1.5 months to 17 years old were included in the study. Episodes of food blockage were the most frequently reported symptoms (46 %). A ratio of 29 % of EE patients reported macroscopically normal endoscopy; diagnosis was then established upon histological anomalies found in biopsies. The mean eosinophil count was 72.4 Eos/HPF. Centralized immunohistochemical staining revealed the presence of IgG4-responding plasma cells in 76.5 % of patients, as well as IgG4 intraepithelial degranulation in 14 % of them. The evaluation of the treatment plan showed important inter-center discrepancies with only 43 % of patients receiving endoscopic reevaluation. This study objectively highlights heterogeneities in diagnosis and care provided to children suffering from EE. Therefore, improving the consistency of practices seems to be crucial to optimize the patients’ outcome. The role of IgG4 as a new diagnosis marker remains to be clarified.
Les auteurs rapportent 3 observations d'enfants ayant bénéficié d'un traitement endoscopique après cholangiopancréatographie rétrograde endoscopique (CPRE) pour pancréatite. Le premier enfant ...présentait une pancréatite familiale ; il a été traité par sphinctérotomie et extraction de lithiase du wirsung par sonde de Fogarty avec des suites favorables. La seconde enfant présentait une pancréatite récurrente sur pancréas divisum ; elle a bénéficié d'une sphinctérotomie de la petite caroncule avec pose d'endoprothèse. La troisième enfant présentait une pancréatite compliquée de pseudokyste puis de sténose et lithiase du wirsung ; elle a été traitée par dilatation du wirsung et extraction de lithiase. Après récidive de la sténose canalaire elle a été traitée par dérivation wirsungojéjunale chirurgicale. La CPRE permet chez l'enfant, outre l'apport diagnostique, une intervention thérapeutique alternative au traitement chirurgical.
Authors report on 3 cases of children treated by therapeutic endoscopic retrograde cholangiopancreatography for pancreatitis (ERCP). The first child presented with familial pancreatitis: he was treated by sphincterotomy and lithiasis extraction. The second child presented with pancreatitis secondary to pancreas divisum: she was treated by sphincterotomy and stunt of small caroncula. The third child presented with severe pancreatitis with pseudocyst: after drainage she was treated by sphincterotomy, Wirsung dilatation and lithiasis extraction. ERCP is a beneficial treatment and may be alternative to surgery in complicated pancreatitis.
Les enfants atteints de maladie inflammatoire chronique de l’intestin (MICI) sont à risque d’immunodépression et d’infections opportunistes du fait de leurs traitements. Les vaccinations ont fait ...l’objet de recommandations spécifiques.
Améliorer la couverture vaccinale d’une population d’enfants dont le statut vaccinal était préalablement connu, par l’information de leurs parents et de leur médecin via un courrier de sensibilisation sur le calendrier vaccinal et le risque infectieux.
Cent cinq patients de la région Grand-Ouest ont reçu le courrier de sensibilisation. Quatre-vingt-douze enfants ont été inclus de mars 2013 à janvier 2014 au cours de consultations suivant l’envoi de ce courrier de sensibilisation. Un questionnaire rempli par le gastropédiatre recueillait l’âge au moment du diagnostic et au moment du recueil, le sexe, le type de MICI, les traitements reçus, le statut vaccinal concernant diphtérie-tétanos-poliomyélite (DTP), Coqueluche, Haemophilus influenzae (Hi), hépatite B (VHB), hépatite A (VHA), Pneumocoque, Méningocoque C (méningoC), tuberculose (BCG), Rougeole-Oreillons-Rubéole (ROR).
L’augmentation du taux de vaccination après sensibilisation était significative pour les vaccins contre DTP (92 % vs 100 %), Hi (88 % vs 98 %), VHB (52 % vs 71 %), pneumocoque (36 % vs 57 %) et méningoC (17 % vs 41 %) (Mac Nemar ; p<0,05). Les patients atteints de maladie de Crohn avaient un meilleur rattrapage que les autres pour le méningoC (OR=4,21,1–25,3 ; p=0,03) et le pneumocoque (OR=4,71,1–29,3 ; p=0,02). Selon un schéma vaccinal minimal (DTP, coqueluche, Hi, ROR et BCG), les enfants plus âgés au moment du diagnostic avaient 1,26 fois plus de chance d’être à jour (p=0,002).
La sensibilisation par courrier est efficace pour augmenter significativement la couverture vaccinale et vient compléter l’information donnée en consultation de suivi. Elle permet d’impliquer le médecin traitant. Cette information devrait être donnée à tous les enfants suivis pour des MICI dès le diagnostic.
Évaluer les connaissances acquises par les adolescents au sujet de leur maladie inflammatoire chronique intestinale (MICI).
Un questionnaire anonyme a été proposé en consultation aux adolescents ...suivis pour une MICI par les gastropédiatres de 13 centres hospitaliers entre le 1er décembre 2012 et le 1er juillet 2013. Après le consentement des parents, les médecins ont rempli une fiche à l’inclusion de chaque adolescent précisant les caractéristiques de la MICI. Les adolescents ont renvoyé leur questionnaire par courrier.
Sur les 124 adolescents de 12 à 19ans inclus, 101 (81,5 %) ont renvoyé une réponse (toutes anonymes). Vingt-trois pour cent pensaient que l’alimentation était une cause possible de MICI et 22 % qu’un objectif de leur traitement était de guérir définitivement leur maladie. Quarante-six pour cent de ceux déclarant avoir une maladie de Crohn connaissaient la localisation ano-périnéale et 14 % savaient que cette maladie pouvait atteindre tout le tube digestif. Vingt-cinq pour cent des adolescents ont su citer un effet secondaire de l’azathioprine (88 % ayant déjà reçu ce traitement) et 24 % un effet secondaire de l’infliximab (54 % ayant déjà reçu ce traitement). Soixante-dix pour cent savaient que le tabac aggravait la maladie de Crohn, et 68 % ont déclaré se renseigner sur leur MICI avec leur gastropédiatre.
Les adolescents atteints de MICI ont des connaissances incomplètes sur leurs maladies et leurs traitements, ce qui justifie la mise en place de séances personnalisées d’éducation thérapeutique.
To assess knowledge acquired by adolescents about their inflammatory bowel disease (IBD).
An anonymous questionnaire was given during consultation to adolescents followed for IBD by pediatricians from 13 hospitals between 1 September 2012 and 1 July 2013. After parental consent, these physicians completed a form at the inclusion of each patient, in which the characteristics of IBD were detailed. The patients mailed back their questionnaire.
A total of 124 patients from 12 to 19 years of age were included with a response rate of 82% (all anonymous); 23% of the patients thought that diet was a possible cause of IBD and 22% that one of the targets of their treatment was to cure their disease for good. Of the patients reported having Crohn disease, 46% knew the anoperineal location and 14% knew that Crohn disease can affect the entire digestive tract. Twenty-five percent of the patients were able to name one side effect of azathioprine (88% had already received this treatment), 24% were able to name one side effect of infliximab (54% had already received this treatment), 70% of the adolescents knew that smoking worsens Crohn disease, 68% declared they had learned about their IBD from their pediatrician, and 81% said they would like to receive more information.
Adolescents with IBD have gaps in their general knowledge and the different treatments of their disease. Their main source of information is their pediatrician, warranting the implementation of customized patient education sessions.
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