Delphine Ley

Incidence and Phenotype at Diagnosis of Very-early-onset Compared with Later-onset Paediatric Inflammatory Bowel Disease: A Population-based Study [1988-2011].

Background and Aims Very-early-onset inflammatory bowel disease [VEO-IBD] is a form of IBD that is distinct from that of children with an older onset. We compared changes over time in the incidence and phenotype at diagnosis between two groups according to age at IBD diagnosis: VEO-IBD diagnosed before the age of 6 years, and early-onset IBD [EO-IBD] diagnosed between 6 and 16 years of age. Methods Data were obtained from a cohort enrolled in a prospective French population-based registry from 1988 to 2011. Results Among the 1412 paediatric cases [< 17 years], 42 [3%] were VEO-IBD. In the VEO-IBD group, the incidence remained stable over the study period. In contrast, the incidence of EO-IBD increased from 4.4/105 in 1988-1990 to 9.5/105 in 2009-2011 [+116%; p < 10-4]. Crohns disease [CD] was the most common IBD, regardless of age, but ulcerative colitis [UC] and unclassified IBD were more common in VEO-IBD cases [40% vs 26%; p = 0.04]. VEO-IBD diagnosis was most often performed in hospital [69% vs 43%; p < 10-3]. Rectal bleeding and mucous stools were more common in patients with VEO-IBD, whereas weight loss and abdominal pain were more frequent in those with EO-IBD. Regarding CD, isolated colonic disease was more common in the VEO-IBD group [39% vs 14%; p = 0.003]. Conclusions In this large population-based cohort, the incidence of VEO-IBD was low and stable from 1988 to 2011, with a specific clinical presentation. These results suggest a probable genetic origin for VEO-IBD, whereas the increase in EO-IBD might be linked to environmental factors.

Eosinophilic esophagitis after desensitization to dust mites with sublingual immunotherapy

Eosinophilic esophagitis (EoE) is a chronic inflammatory disorder characterized by esophageal dysfunction and eosinophilic infiltration of the esophageal mucosa. The disease affects both children and adults, with a prevalence of 5 per 10,000 inhabitants in Europe and the United States.1 Childhood cases are generally diagnosed between 5 and 10 years of age and adult cases between 30 and 40 years of age. The male-female sex ratio is 3:1 in a published series.2 The symptoms vary according to the age of the individual and consist of difficulties in feeding and in gaining weight in small children, abdominal pain and vomiting in older children, and solid food dysphagia and symptoms that suggest gastroesophageal reflux in adults.3 Histopathologic examination revealing more than 15 polynuclear eosinophils (PNEs) per 40 field is indispensable to establish the diagnosis of EoE. An atopic or allergic history and genetic susceptibility are risk factors for the development of EoE.4 In predisposed individuals, exposure to certain food allergens and aeroallergens can trigger the disorder.Wedescribe thefirst reportedcase, toourknowledge, ofEoE ina child after sublingual immunotherapy (SLIT) for dustmite allergy. A 10-year-old girl, one of twins, with a history of asthma, allergic rhinitis, and dust mite allergy started daily SLIT (where drops are placed under the tongue for 2 minutes and then swallowed) for dust mite desensitization at the end of January 2013. SLIT wasmade of a Staloral (Stallergenes S.A., Antony, France) standardized Dermatophagoides pteronyssinus and Dermatophagoides farinae 50/50 extract at 300 IR concentration. In March 2013, six weeks after the start of SLIT, the patient attended the emergency department for reflux and vomiting of 1-month duration accompanied by pain, retrosternal chest discomfort, and weight loss. There was no trigger factor and no time pattern. She had no fever or abdominal injury. Local antacids and domperidone were not effective. During a previous emergency consultation, she had been prescribed proton pump inhibitors (PPIs), omeprazole (20 mg/d), and sodium alginate, which temporarily relieved the symptoms for 48 hours. The complete blood cell count, erythrocyte sedimentation rate, and liver function test results were normal. The C-reactive protein level was 14 mg/L. Upper endoscopy, performed 12 days after starting PPI treatment, revealed a slightly congested appearance of the lower third of the esophagus, without ulceration. The gastric mucosa revealed focal congestion, with no ulcer, ulceration, or nodular appearance. Eight esophageal biopsy specimens (3 from the distal third, 3 from the middle third, and 2 from the upper third), 5 stomach specimens (3 from the antrum and 2 from the fundus), and 2 duodenal specimens (1 from the bulb) were obtained. Histopathologic examination of these specimens

Growth Pattern in Paediatric Crohn Disease Is Related to Inflammatory Status.

Objectives: The respective role of disease activity and steroid therapy in growth impairment in paediatric-onset Crohn disease (CD) is still debated. Our aim was to investigate whether the growth pattern of children with CD was correlated with the inflammatory status during the disease course, regardless the cumulative duration of steroid therapy. Methods: One hundred and seven patients with a diagnosis of CD <17 years, followed during ≥2 years and for whom ≥2 height measures were available during follow-up, were identified between 1998 and 2010. Height, C-reactive protein (CRP), orosomucoid, and steroid therapy duration were collected at each visit. The relationship between the evolution of growth velocity and inflammatory status during follow-up was investigated using a linear mixed model with random coefficients. Results: Median age at diagnosis was 11.7 years (Q1–Q3: 9.8–13.5). Mean height for age (H/A) z score was 0.14 ± 1.29 at diagnosis and 0.05 ± 1.23 among the 75 patients who had reached their final height at maximal follow-up (median: 4.9 years; Q1–Q3: 3.8–6.4). Growth failure (H/A z score <−2) was present in 7 (8%) patients at diagnosis and 5 (5%) at maximal follow-up. Growth velocity was negatively correlated with the evolution of CRP (P < 0.0001) and orosomucoid (P < 0.0001) during follow-up. After adjustment for the cumulative duration of steroid therapy, these 2 correlations remained significant (CRP: P = 0.0008; orosomucoid: P < 0.0001). Conclusions: Children with CD with uncontrolled inflammatory status have a lower growth velocity. The inflammatory status should be kept as close to normal as possible in paediatric-onset patients with CD to optimize their growth pattern.

Importance of an International Registry for and Collaborative Research on Esophageal Atresia

Esophageal atresia (EA) is a rare congenital defect. Data on EA prevalence, management, and long-term outcome are lacking because the available data come from small retrospective series from tertiary referral centers. An international multicenter registry would provide strong epidemiological data from large population-based cohorts on EA prevalence and incidence, treatment, long-term morbidity, and prognosis and would thus provide accurate data for evaluation of the current guidelines for EA management. The future challenge of the new international network on EA, which was created in 2013, is to promote the creation of a collaborative database and further studies.

Su1924 Incidence and Phenotype at Diagnosis in Very Early Compared to Later-Onset Pediatric Inflammatory Bowel Disease: A Population-Based Study (1988-2011)

1 Gastroentérologie pédiatrique, Université et CHRU de Lille, 2 Epidémiologie, Université et CHRU de Lille, 3 Gastroentérologie, Université et CHU d’Amiens, 4 Gastroentérologie, Université et CHU de Rouen, 5 Gastroentérologie, Université et CHRU de Lille, 6 Unité de Pédiatrie, Hôpital St Vincent, Faculté Catholique de Lille, 7 Gastroentérologie, Hôpital Les Bonnettes d’Arras, 8 Gastroentérologie, Université et CHRU de Nancy, 9 LIRIC UMR 995 Inserm , Université Lille 2 / CHRU de Lille, France.

P-077: Growth pattern in pediatric Crohn’s disease is related to inflammatory status and not to cumulative duration of steroid therapy

P-076 Persistence of growth retardation in pediatric Crohn’s disease J. Amil Dias1 *, K.L. Kolho2, M. Sladek3, D. Turner4, T. Pfeffer Gik5, G. Veres6, R. Shaoul7, A. Staiano8, J. Escher9, A. Paerregaard10, J. Martin de Carpi11, G. Veereman Wauters12, S. Koletzko13, O. Shevah5, L. Finnby14, C.C. Dias15, A. Levine5. 1Hospital S. Joao, Porto, Portugal, 2Children’s Hospital, Helsinki University Central Hospital, Helsinki, Finland, 3Jagiellonian University Medical College, Krakow, Poland, 4Shaare Zedek Medical Center, The Hebrew University of Jerusalem, Jerusalem, Israel, 5Wolfson Medical Center, Tel Aviv, Israel, 6Semmelweis University, Budapest, Hungary, 7Meyer Children’s Hospital, Haifa, Israel, 8University of Naples “Federico II”, Naples, Italy, 9Erasmus MC Sophia Children’s Hospital, Rotterdam, The Netherlands, 10Hvidovre Hospital, Hvidovre, Denmark, 11Hospital Sant Joan de Deu, Barcelona, Spain, 12Hospital UZ Brussels, Brussels, Belgium, 13Dr. v Haunersches Kinderspital, Munich, Germany, 14Lovisenberg Diakonale Hospital, Oslo, Norway, 15Medical School, Porto, Portugal

Sa1155 Growth Pattern and Growth Failure in Paediatric Crohn's Disease Are Related to Inflammatory Status but Not to Duration of Steroid Therapy

age and symptoms of PSC were related to impairment of HRQoL scores, but gen-der, ERC score or comorbidity with IBD did not have a significant impact on them. The total 15D scores between PSC patients (mean 0.934) and the general population (mean 0.939) did not differ significantly but the dimensions of elimination (p < 0.001), depression (p = 0.003), distress (p = 0.003) and vitality (p = 0.005) were significantly lower in PSC. In the newly diagnosed, no significant changes were observed in the 15D scores in a mean 1.58 years’ follow-up. The 15D scores of age-, genderand IBD activity matched IBD patients were lower than those of the PSC patients (0.876 versus 0.914, p = 0.04). Conclusions: HRQoL of PSC patients was mostly comparable to that of the general population but special attention should be paid to the patients’ psychological well-being. No significant HRQoL changes were observed in the mean 1.58 years’ follow-up after diagnosis of PSC. Newly diagnosed PSC patients have better HRQoL than do newly diagnosed IBD patients, probably because of more intensive follow-up and a different spectrum of symptoms.