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  • 1
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    DOP27 Disease activity patterns in the first 5 years after diagnosis in children with Crohn’s disease: a population-based study.
    Oxford University Press (OUP) ; 2023
    In:  Journal of Crohn's and Colitis Vol. 17, No. Supplement_1 ( 2023-01-30), p. i91-i93
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 17, No. Supplement_1 ( 2023-01-30), p. i91-i93
    Abstract: This study aimed to define clusters of disease activity and prognostic factors of the disease course in a well-characterized cohort of children with Crohn’s disease (CD). Methods All patients with CD identified from the SIGENP IBD registry with a follow-up of at least 5 years and a 6-monthly evaluation were included. Active disease was defined for each yearly semester as follows: clinical activity (wPCDAI≥12,5 or MINI index ≥8), need for treatment escalation, hospitalization, or surgery, active disease on endoscopy (SES-CD & gt;3) or imaging. Formula-based clusters were generated based on previously published patterns in adults 1. Prediction models were created based on clinical, surgical, endoscopic, and laboratory findings at diagnosis and at 6-month follow-up. Results Data from 332 patients were analyzed. Ninety-six (29%) had a quiescent course; 52 (16%) and 34 (10%) had a moderate-to-severe chronically active and chronic intermittent disease; 107 (32%) and 43 (13%) had an active disease in the first two years after diagnosis and remission thereafter and the opposite course, respectively. Overall, 129 (39%) children presented with an active disease at 5 years of follow-up, with no patient off-therapy. Surgery at the time of diagnosis was significantly associated with a quiescent course [OR 10.05 (CI 3,05-25,22), p 0.0005], while growth impairment at the diagnosis and moderate-severe activity requiring corticosteroids at 6 months were inversely related to the quiescent group [OR 0.48 (CI 0,27-0,81) p 0.007 and OR 0.35 (CI 0,16-0,71) p 0.005, respectively] . Perianal involvement at diagnosis and moderate-severe activity at 6 months positively correlated with disease progression (i.e., B1 to B2/B3) at follow-up [OR 2.55 (CI 1,05-6,07) p & lt;0.04 and OR 3.85 (CI 1,20-12,85) p 0.02]. Seventy-three patients (22%) required surgery, of which 31 (43%) in the first 6 months after diagnosis. Conclusion Approximately one-third of our cohort of children with CD had a quiescent course during the first five years after diagnosis. In contrast, more than one-third of the patients had a chronically or intermittently active disease during the entire follow-up, or an initial remission followed by a persistent activity. Surgery at the time of diagnosis, mild disease onset without growth impairment, and lower disease activity without corticosteroid use in the first six months after diagnosis predicted a quiescent course over time. 1. Wintjens D., Bergey F., Saccenti E. et al. Disease activity patterns of Crohn's disease in the first ten years after diagnosis in the population-based IBD South Limbrug Cohort. J Crohns Colitis. 2021; 15 (3):391-400.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjac190.0067
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
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  • 2
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    P458 Safety and Potential Efficacy of Escalating Dose of Ustekinumab in Pediatric Crohn’s disease (the SPEED-UP study) - A multi-center study from the paediatric IBD Porto group of ESPGHAN
    Oxford University Press (OUP) ; 2022
    In:  Journal of Crohn's and Colitis Vol. 16, No. Supplement_1 ( 2022-01-21), p. i435-i435
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 16, No. Supplement_1 ( 2022-01-21), p. i435-i435
    Abstract: Ustekinumab (UST) is an effective therapy for induction and maintenance of remission in Crohn’s disease (CD). Intensification of UST maintenance dosage has shown effectiveness in some adult patients, but no similar data are available in children. The aim of the study was to evaluate the effectiveness and safety of dose escalation of UST in paediatric CD. Methods This was a retrospective multicenter study from 25 centers affiliated to the IBD Interest and Porto groups of ESPGHAN. We included children with CD who initiated UST at a standard dosing and subsequently underwent dose escalation to intervals shorter than 8 weeks, or re-induction due to active disease. Demographic, clinical, laboratory, endoscopic and imaging data were collected at escalation and during a 12 months follow-up. Clinical remission was defined as weighted Paediatric Crohn’s Disease Activity Index (wPCDAI) & lt;12.5 and clinical response as a decline in & gt;17.5 points. Adverse events were explicitly recorded. Results Sixty-nine children, with a median age of 15.8 (IQR 13.8–16.9) years and disease duration of 4.3 (2.9–6.3) years were included. Sixty-eight (98.6%) and 59 (86.8%) children were biologic and immunomodulators experienced, respectively. UST dose was escalated after a median of 6 (3.6–12) months of therapy. Clinical response and remission were observed in 46 (67%) and 29 (42%) children at 3 months, respectively. The strongest predictor of clinical remission was lower wPCDAI at escalation (p=0.001). The median serum levels of C-reactive protein decreased from 14 (3–28.03) to 5 (1.1–20.5) mg/L at 3 months (p=0.012), and fecal calprotectin from 1110 (499–2300) to 248 (118–1159) mcg/g at 12 months (p=0.05). Endoscopic and transmural healing were achieved in 3/19 (16%) and 2/15 (13%) patients, respectively, of those with available tests. Overall, 13 patients (19%) discontinued therapy due to active disease, at a median of 3 (3–4.5) months. No serious adverse events were reported. Conclusion Two-thirds of children with active CD achieved response following dose escalation of UST.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjab232.585
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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  • 3
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    P150 Identification of features associated with poor outcomes in pediatric patients with ulcerative proctitis: A Multicentre Study From the Paediatric IBD Porto Group of ESPGHAN
    Oxford University Press (OUP) ; 2023
    In:  Journal of Crohn's and Colitis Vol. 17, No. Supplement_1 ( 2023-01-30), p. i309-i311
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 17, No. Supplement_1 ( 2023-01-30), p. i309-i311
    Abstract: Ulcerative proctitis (UP) is an uncommon presentation in paediatric patients with ulcerative colitis, accounting for & lt;10% of cases. Here we aimed to characterize the clinical features and natural history of paediatric patients with UP, and to identify predictors of poor outcomes. Methods A retrospective cohort study involving 37 sites affiliated with the IBD Interest group of ESPGHAN. Data were collected at different timepoints from patients diagnosed with UP aged & lt;18 years between 01/01/2016-31/12/2020. Outcomes included time to initiation of systemic steroids, thiopurines or biologics, time to acute severe colitis (ASC), IBD-related admission and colectomy. Univariate cox regression was used to study the association between potential predictors and study outcomes. Results Two hundred and fifty patients with UP were included, with a median age at diagnosis of 14.5 (IQR 12.3-15.9) years, and a median follow-up of 2.7 (IQR 1.7-3.9) years. The most common presenting symptoms were bloody stools (94%), abdominal pain (59%) and diarrhea (53%). At diagnosis, the median pediatric ulcerative colitis activity index (PUCAI) score was 25 (IQR 20-35), and only 3 patients (1.3%) presented with ASC. Most children had normal inflammatory markers and albumin levels. the median fecal calprotectin level was 720 mcg/g (IQR 310-1800), while 16 patients (9.9%) had a calprotectin level & lt;100mcg/g at diagnosis. Administration of oral or topical 5-ASA resulted in clinical remission rates of 52% and 48%, respectively, by the end of induction, while the combination of both led to remission in 73% of patients. The rates of treatment escalation to thiopurines and biologics at 1, 3 and 5 years were 11%, 27% and 45%, and 11%, 23% and 45%, respectively (Figure 1). Within 5 years from diagnosis, 20% of patients had presented with ASC. The PUCAI score at diagnosis of UP was highly associated with initiation of systemic steroids, thiopurines or biologics, as well as later ASC event and IBD admission, while a Mayo endoscopic score of 3 was associated with the initiation of biologics and subsequent ASC event and IBD admission (Table 1). Seven patients (4%) underwent colectomy by the end of follow-up. Among the 151/250 patients who had a repeat endoscopy, only 16% achieved mucosal healing and in 48% inflammation extended proximally. Cecal patch (P=0.009) and higher PUCAI score (P=0.009) at diagnosis, as well as higher PUCAI score (P=0.009) and lack of steroid-free clinical remission (P=0.005) by the end of induction were associated with proximal disease extension. Conclusion Disease burden is significant in paediatric patients that present with UP, with high rates of proximal disease extension and requirement for treatment escalation.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjac190.0280
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
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  • 4
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    DOP66 Disease course of Ulcerative proctitis in children: A population based study on behalf of the SIGENP IBD Group
    Oxford University Press (OUP) ; 2022
    In:  Journal of Crohn's and Colitis Vol. 16, No. Supplement_1 ( 2022-01-21), p. i110-i111
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 16, No. Supplement_1 ( 2022-01-21), p. i110-i111
    Abstract: Ulcerative proctitis (UP), defined as a colonic location limited to the rectum, is a poorly investigated condition in children, usually considered as a minor form of Ulcerative Colitis (UC). The aim of the present study was to compare the disease course of paediatric patients affected by UP at diagnosis with the other UC locations. Methods This multicentre retrospective observational study has been carried out starting from the data prospectively registered in the IBD Registry of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP). Seventeen IBD referral centres adhering to the registry were included in the study. Patients age 0 to 18 years, who were diagnosed with UC according to the Porto criteria starting from January 1, 2009, to May 1st, 2021 were identified. Only children with a minimum follow-up of 12 months were included in the study. Once enrolled, children were subsequently divided in two groups based on Paris classification: group 1 (E1) and group 2 (E2, E3 and E4). Results Eight-hundred-eighty-five children were finally included in the study (median age at diagnosis: 11.2 years, range: 0–18 years; M/F: 434/451), of whom 176 (19.8%) belonging to group 1 and 709 (80.1%) to group 2. The median age at diagnosis was significantly higher in group 1 when compared to group 2 [11.9 (0–18) versus 11 (0–18) years, respectively; (p & lt;0.001)]. At diagnosis, the induction therapy was significantly different with 68 (39.5%) patients of group 1 undergoing steroid therapy versus 505 (71.2%) of group 2 (p & lt;0.001) and 79 (41.9%) of group 1 practising only mesalamine respect to 186 (26.2%) of group 2 (p & lt;0.001). A higher number of children from group 2 started immunosuppressive or biologic therapy as maintenance therapy at diagnosis [Group 1: 11 (6.2%) versus 173 (24.4%), respectively; (p & lt;0.001)]. The median follow-up of our cohort was 4.5 years (range 1–13 years). At the last follow-up, 67/176 (38%) children with UP showed an extension of their disease location without significant difference when compared to group 2 [265 (37.5%); p=0.9] , while 81 (45%) children from Group 1 were under immunosuppressive or biologic therapy versus 566 (79.8%) from group 2 (p & lt;0.001). Five children (3%) of Group 1 underwent colectomy during the follow up versus 45 (6.9%) of Group 2 (p=0.06). Conclusion UP is a frequent location of paediatric onset UC and the risk of endoscopic extension of proctitis is similar to the more extensive forms. A considerable number of patients with UP required immunosuppressive or biologic therapy during the follow-up and no significant difference was observed in terms of surgery. Overall, UP cannot be considered as a minor form of UC.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjab232.105
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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  • 5
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    CO10 THALIDOMIDE AFTER INFLIXIMAB FAILURE IN CHILDREN AND YOUNG ADULTS WITH CROHN'S DISEASE AND ULCERATIVE COLITIS
    Elsevier BV ; 2012
    In:  Digestive and Liver Disease Vol. 44 ( 2012-10), p. S244-S245
    Details
    In: Digestive and Liver Disease, Elsevier BV, Vol. 44 ( 2012-10), p. S244-S245
    Type of Medium: Online Resource
    ISSN: 1590-8658
    URL: Article
    DOI: 10.1016/S1590-8658(12)60638-0
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2012
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  • 6
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    P028 THALIDOMIDE FOR TREATING REFRACTORY CROHN'S DISEASE AND ULCERATIVE COLITIS IN CHILDREN: RANDOMIZED CONTROLLED TRIAL (PRELIMINARY RESULTS)
    Oxford University Press (OUP) ; 2009
    In:  Journal of Crohn's and Colitis Supplements Vol. 3, No. 1 ( 2009-9), p. 8-
    Details
    In: Journal of Crohn's and Colitis Supplements, Oxford University Press (OUP), Vol. 3, No. 1 ( 2009-9), p. 8-
    Type of Medium: Online Resource
    ISSN: 1873-9954
    URL: Article
    DOI: 10.1016/S1873-9954(09)70042-4
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2009
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  • 7
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    P141 Scoring endoscopy in pediatric inflammatory bowel disease: a way to improve quality
    Oxford University Press (OUP) ; 2020
    In:  Journal of Crohn's and Colitis Vol. 14, No. Supplement_1 ( 2020-01-15), p. S208-S208
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 14, No. Supplement_1 ( 2020-01-15), p. S208-S208
    Abstract: The aim of mucosal healing (MH) as a therapeutic target in paediatric inflammatory bowel diseases (IBD) has emphasised the role of the endoscopy. There is a great variability in evaluating mucosal lesions among different operators, especially in paediatric patients. This multicentre prospective study aims to evaluate the interobserver agreement among paediatric endoscopists in using validated endoscopic scores of IBD in children. Methods Fifteen videos of follow-up ileocolonoscopies in children with IBD (8 ulcerative colitis –UC-, 7 Crohn’s disease –CD-) were selected from 3 different referral sites in Italy. Eleven paediatric endoscopists from different centres were asked to evaluate all videos as independent and blinded readers. The scoring systems used were ulcerative colitis Endoscopic Index of Severity (UCEIS) for UC and simple endoscopic score for Crohn’s disease (SES-CD) for CD. Kappa statistics and intraclass correlation coefficients were used to measure agreement. Furthermore, an experienced adult gastroenterologist evaluated the same videos and scores them. His results were compared with paediatric endoscopists’ findings. Results The median age of the participants was 40 (interquartile range: 6) with a median experience of 12 (14) years in centres with a median number of 140 (230) of paediatric IBDs. Intercluster correlation agreement was 0.298 (95% CI: 0.13–0.55) for UC and 0.266 (0.11–0.52) for CD. When a disease activity categorisation was adopted (remission, moderate, mild and severe) Fleiss’ kappa coefficient was 0.408 (0.29–0.53) for UC and 0.552 (0.43–0.73) for CD (Figure 1). When stratified for item vascular pattern of UC was the most reliable item IC: 0.624 (0.321–0.854). The comparison between paediatric and expert gastroenterologist’s scores is shown in Figure 2. In the multivariate analysis none of the reviewer characteristic affected the readers’ errors. Conclusion This pilot multicentre study shows that there is a low level of agreement among paediatric endoscopists in evaluating children with IBDs. Agreement improved after using a disease activity categorisation, with better results for CD. Regardless to experience, all readers showed a low-grade accordance with adult gastroenterologist. According to these findings, the use of scoring systems should be implemented for all paediatric endoscopists. Future specific training programs should be considered to pursue this goal.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjz203.270
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
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  • 8
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    P364 New insights into the molecular mechanisms of thalidomide in paediatric inflammatory bowel disease patients
    Oxford University Press (OUP) ; 2020
    In:  Journal of Crohn's and Colitis Vol. 14, No. Supplement_1 ( 2020-01-15), p. S345-S346
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 14, No. Supplement_1 ( 2020-01-15), p. S345-S346
    Abstract: Thalidomide is an effective drug in children with inflammatory bowel disease (IBD) refractory to standard treatments, however, its use is often limited by its safety profile, in particular for the risk of teratogenicity and peripheral neuropathy. Multiple hypotheses exist to explain the molecular mechanism of thalidomide action but no data have been published on IBD patients. To identify determinants of thalidomide action in paediatric IBD, high-throughput microRNA (miRNA) and messenger RNAs (mRNA) profiles during treatment were analysed. Methods IBD patients responsive to thalidomide were enrolled. miRNA and mRNA profiles from peripheral blood obtained before and after 12 weeks of treatment were determined using next-generation sequencing. Differentially expressed genes were identified by fold change from the general linear model. In order to detect the potentially altered pathways, the hypergeometric test based on gene ontology annotations was used. The identification of putative mRNA targets of thalidomide sensitive miRNAs was performed by Targetscan database. Results Ten IBD paediatric patients (mean age 13.1 years, 6 males) were enrolled. Sequencing analysis identified 10 miRNAs (3 downregulated) and 252 mRNAs (76 downregulated) deregulated after treatment. Five upregulated miRNAs could putatively recognise the 3′UTR of several Hox genes, a group of transcription factors that play important roles in the development of structures such as limbs, lungs and nervous system. The hypergeometric test highlighted the altered pathways involved in inflammatory response, regulation of immune system, prostaglandin receptor activity and G protein-coupled receptor signalling. Among the 10 miRNAs deregulated by thalidomide, six could putatively regulate the differentially expressed mRNAs resulting from the analyses. Conclusion Thalidomide induces specific gene expression alterations, which could help to elucidate its molecular mechanism in paediatric IBD patients. miRNA results may suggest new molecular targets involved in the teratogenic effects of this drug. Moreover, mRNA profiles indicate a strong involvement of adenylate cyclase-modulating G protein-coupled receptor signalling in the thalidomide mechanism of action.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjz203.493
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
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  • 9
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    OC.04.5 DISEASE ACTIVITY PATTERNS IN THE FIRST 5 YEARS AFTER DIAGNOSIS IN CHILDREN WITH CROHN'S DISEASE: A POPULATION-BASED STUDY
    Elsevier BV ; 2023
    In:  Digestive and Liver Disease Vol. 55 ( 2023-03), p. S95-
    Details
    In: Digestive and Liver Disease, Elsevier BV, Vol. 55 ( 2023-03), p. S95-
    Type of Medium: Online Resource
    ISSN: 1590-8658
    URL: Article
    DOI: 10.1016/S1590-8658(23)00264-5
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2023
    detail.hit.zdb_id: 2061359-3
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  • 10
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    P385 Tailoring induction treatment for children with Crohn’s disease
    Oxford University Press (OUP) ; 2022
    In:  Journal of Crohn's and Colitis Vol. 16, No. Supplement_1 ( 2022-01-21), p. i385-i385
    Details
    In: Journal of Crohn's and Colitis, Oxford University Press (OUP), Vol. 16, No. Supplement_1 ( 2022-01-21), p. i385-i385
    Abstract: Multiple clinical studies in children with Crohn’s disease (CD) have demonstrated the high efficacy of nutritional therapy with exclusive enteral nutrition (EEN) to induce remission and even to promote mucosal healing. However, EEN as inductive treatment may be not tolerated or inefficient and we are well aware of the paramount importance of a well conducted induction treatment for paediatric CD with the goal of an individualized induction treatment to improve the prognosis and the quality of life of inflammatory bowel disease (IBD) children. Methods A retrospective multicentre study including newly diagnosed children with CD treated with EEN as induction therapy was designed. The primary aim of the study was to study predictive factors of non- adherence to treatment. The secondary endpoint were predictive factors of clinical non-remission at the end of induction treatment. Those data together were analysed with the ultimate goal of trying to define an individualized induction treatment for children with CD. Results 376 CD children from, 14 IBD paediatric referral centres were enrolled in the study. The rate of EEN adherence was, 89 %. Colonic involvement and FC & gt;, 600 μg/g at diagnosis were found associated with a reduced EEN adherence in univariate and multivariate analysis. The remission rate of those who completed induction treatment was, 67%. A multivariate analysis showed that factor determining lower remission rate were age & gt;, 15 years and PCDAI & gt;, 50. With those results we were able to create a decisional algorithm which is provided in figure 1. Conclusion EEN administered for, 8 weeks is effective for inducing clinical remission. The rate of adherence is high but there is a group of patients (colonic involvement + hight FC) which are at risk of non-adherence and thus may be candidate for alternative dietary induction regimens. Moreover, older patients with moderate to severe active disease (PCDAI & gt;40), are at higher risk of failing clinical remission achievement after EEN and may benefit from an early anti-TNF alpha treatment. Personalized treatment strategies should be proposed weighing the benefits and risks based on each patient’s disease location, phenotype and disease activity with the overall aim of obtaining rapid control of inflammation to reduce long-term bowel damage.
    Type of Medium: Online Resource
    ISSN: 1873-9946 , 1876-4479
    URL: Article
    DOI: 10.1093/ecco-jcc/jjab232.512
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
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