Functional abdominal pain disorders and patient- and parent- reported outcomes in children with inflammatory bowel disease in remission

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

L'hypothyroïdie en pédiatrie à l'époque du dépistage néonatal

LILLE2-BU Santé-Recherche (593502101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

FIBRODYSPLASIE OSSIFIANTE PROGRESSIVE

LILLE2-BU Santé-Recherche (593502101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Le diagnostic éducatif de surcharge pondérale en hôpital de jour pédiatrique de Saint Vincent de Paul (intérêt pour l'enfant, les parents et le médecin généraliste)

LILLE2-BU Santé-Recherche (593502101) / SudocSudocFranceF

New therapeutic strategies are associated with a significant decrease in colectomy rate in pediatric ulcerative colitis

International audienceObjectives: We evaluated the impact of immunosuppressants (IS) and anti-tumor necrosis factor (TNF) introduction on ulcerative colitis (UC) long-term outcomes in a large population-based pediatric-onset cohort.Methods: All patients included in the EPIMAD registry with a diagnosis of UC made before the age of 17 between 1988 and 2011 were followed up retrospectively until 2013. Medication exposure and disease outcomes were compared between three diagnostic periods: 1988 to 1993 (period [P]1; pre-IS era), 1994 to 2000 (P2; pre-anti-TNF era), and 2001 to 2011 (P3; anti-TNF era).Results: A total of 337 patients (girls 57%) diagnosed with UC were followed up during a median duration of 7.2 years (IQR: 3.8-13.0). The IS and anti-TNF exposure rates at 5 years increased over time from 7.8% (P1) to 63.8% (P3) and from 0% (P1) to 37.2% (P3), respectively. In parallel, the risk of colectomy at 5 years decreased significantly over time (P1, 17%; P2, 19%; P3, 9%; p=0.045, p-trend=0.027), and between pre-anti-TNF era (P1+P2, 18%) and the anti-TNF era (P3, 9%) (p=0.013). The risk of disease extension at 5 years remained stable over time (P1, 36%, P2, 32%, P3, 34%, p=0.31, p-trend=0.52), and between the pre-anti-TNF era (P1+P2, 34%) and the anti-TNF era (P3, 34%) (p=0.92). The risk of flare-related hospitalization at 5 years significantly increased over time (P1, 16%; P2, 27%; P3, 42%; p=0.0012, p-trend=0.0006) and between pre-anti-TNF era (P1+P2, 23%) and anti-TNF era (P3, 42%) (p=0.0004).Conclusions: In parallel with the increased use of IS and anti-TNF, an important decline in the risk of colectomy in pediatric-onset UC was observed at the population level

Clinical and manometric characteristics of allgrove syndrome

OBJECTIVE: The association of adrenocorticotropic hormone (ACTH) insensitivity with achalasia and alacrimia (Allgrove syndrome, 3A) constitutes a rare multisystem disorder. Its evolution is not well known. The aim of this study was to describe clinical and esophageal manometric characteristics and outcomes in Allgrove syndrome. PATIENTS AND METHODS: This multicenter retrospective study compared clinical and manometric characteristics at diagnosis and on follow-up of 9 children presenting with 3A (mean age at diagnosis 7.1 years) with those of 9 children with idiopathic achalasia (IA) (mean age at diagnosis 8.3 years). RESULTS: At the time of diagnosis, 3 children with 3A presented with no digestive or respiratory signs because they were identified during a family screening; 1 remained asymptomatic 8 years later. ACTH levels were high in patients with 3A. All of the patients with IA were symptomatic at diagnosis. No significant difference was observed when comparing any of the manometric parameters of the first esophageal manometry of 3A with those of IA. Seven children with 3A were operated on using the Heller procedure, completed by pneumatic esophageal dilation in 2 of these 7. One patient with 3A was treated only by nifedipine. Failure of treatment was observed in 3 children with 3A and 1 child with IA, partial success in 4 with 3A and 1 with IA, and total success in 2 with 3A and 7 with IA (P<0.03). Control manometry showed that in the 3A group, partial success after surgery was always associated with abnormally low or normal lower esophageal sphincter (LES) pressure, whereas failure after surgery was associated with high LES pressure. CONCLUSIONS: Our data showed that 3A presented a more severe course than IA despite presymptomatic diagnosis in cases of family screening. The high LES pressure noted in some patients with 3A is suggestive of a peculiar pattern in 3A affecting the LES and the lower part of the esophagus. Copyright © 2011 by European Society for Pediatric Gastroenterology.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Increased Risk of Cancer and Mortality in a Large French Population-Based Paediatric-Onset Inflammatory Bowel Disease Retrospective Cohort.

International audienceBACKGROUND AND AIMS: Paediatric-onset IBD (pIBD) is associated with an increased risk of cancer and mortality in adulthood. The aims of this study were to measure the incidence of cancer and mortality in patients with pIBD and identify factors associated with mortality and cancer. METHODS: All patients diagnosed with Crohn's disease (CD) or ulcerative colitis (UC) before the age of 17 years between 1988 and 2011 in the EPIMAD registry, were retrospectively followed until 2013 for cancer and 2015 for mortality. Standardized incidence (SIR) and mortality ratios (SMR) were estimated compared to the general population. Cox regression was used to compare effect of exposures on cancer and mortality among IBD patients. RESULTS: We included 1,344 patients (52% males, 75% CD), totalising 12,957 patient-years for cancer incidence and 18,817 patient-years for mortality. There were 14 cases of cancer (median age 27.8 years) and 15 deaths (median age 28.8 years). The incidence of cancer and of mortality were increased compared to the general population: all-cancer SIR = 2.7 (95%CI: 1.5-4.8), SMR = 1.7 (95%CI: 1.0-2.8). Colorectal cancer had the highest SIR and SMR: SIR=41.2 (95%CI: 17.2-99.0), SMR=70.4 (95%CI 22.7-218.2). Cancer was associated with (HR, 95%CI): active smoking at diagnosis (5.5, 1.8-16.5), p=0.002, any exposure to anti-TNF (6.1, 1.7-22.3), p=0.0065 and exposure to combination therapy (7.4, 1.8-29.7), p=0.0047. Mortality was associated with extraintestinal manifestations (HR 4.9 (95% CI: 1.7-13.8), p=0.003). CONCLUSIONS: In this large population-based cohort, patients with pIBD had an increased risk of both cancer (2.7-fold) and mortality (1.7-fold), particularly for colorectal cancer

New Therapeutic Strategies Have Changed the Natural History of Pediatric Crohn’s Disease: A Two-Decade Population-Based Study

International audienc

Natural History of Anal Ulcerations in Pediatric-Onset Crohn's Disease: Long-Term Follow-Up of a Population-Based Study

International audienceBackground: Anal ulcerations are frequently observed in Crohn's disease (CD). However, their natural history remains poorly known, especially in pediatric-onset CD.Methods: All patients with a diagnosis of CD before the age of 17 years between 1988 and 2011 within the population-based registry EPIMAD were followed retrospectively until 2013. At diagnosis and during follow-up, the clinical and therapeutic features of perianal disease were recorded. An adjusted time-dependent Cox model was used to evaluate the risk of evolution of anal ulcerations towards suppurative lesions.Results: Among the 1,005 included patients (females, 450 (44.8%); median age at diagnosis 14.4 years (IQR, 12.0-16.1)), 257 (25.6%) had an anal ulceration at diagnosis. Cumulative incidence of anal ulceration at 5 and 10 years from diagnosis was 38.4% (CI95%, 35.2-41.4) and 44.0% (CI95%, 40.5-47.2), respectively. In multivariable analysis, the presence of extra-intestinal manifestations (Hazard Ratio (HR) 1.46, CI95% 1.19-1.80, p=0.0003) and upper digestive location (HR 1.51, CI95% 1.23-1.86, p<0.0001) at diagnosis were associated with the occurrence of anal ulceration. Conversely, ileal location (L1) was associated with a lower risk of anal ulceration (L2 vs L1 HR 1.51, CI95% 1.11-2.06, p=0.0087; L3 vs L1 HR 1.42, CI95% 1.08-1.85, p=0.0116). The risk of fistulizing perianal CD was doubled in patients with a history of anal ulceration (HR 2.00, CI95% 1.45-2.74, p<0.0001). Among the 352 patients with at least one episode of anal ulceration without history of fistulizing perianal CD, 82 (23.3%) developed fistulizing perianal CD after a median follow-up of 5.7 years (IQR, 2.8-10.6). In these patients with anal ulceration, the diagnostic period (pre vs biologic era), exposure to immunosuppressants and/or anti-TNF did not influence the risk of secondary anoperineal suppuration.Conclusion: Anal ulceration is frequent in pediatric-onset CD, with nearly half of patients presenting with at least one episode after 10 years of evolution. Fistulizing perianal CD is twice as frequent in patients with present or past anal ulceration