The Care of Children and Adolescents With Chronic Inflammatory Bowel Disease: A Cluster-Randomized Trial on Improving the Guideline Conformity of Treatment by the Use of the CEDATA-GPGE Patient Registry. Dtsch Arztebl Int. 2024 Sep 20;(Forthcoming):arztebl.m2024.0168. doi: 10.3238/arztebl.m2024.0168. Epub ahead of print. PMID: 39163213.

DOI: 10.3238/arztebl.m2024.0168

Abstract

Background: For children and adolescents with chronic inflammatory bowel disease (IBD), treatment that is not in adequate conformity with the guidelines can adversely affect both the course of disease and the patients‘ development. The targeted use of digital patient registries may improve real-life adherence to the recommendations of evidence-based guidelines. 

Methods: In a cluster-randomized, controlled trial (DRKS00015505), treatment providers for the intervention group (IG) documented the treatment of children and adolescents with IBD in the CEDATA-GPGE patient registry; they received automated feedback on the data they entered and on potential deviations of the documented treatment from recommendations contained in the guidelines (care deficits). Treatments providers for the control group (CG) documented treatments as previously, i.e., only in the patients‘ charts. At the end of a twelve-month observation period, the data from both groups at baseline and on follow-up were analyzed in an intergroup comparison. The primary endpoint was the number of care deficits at twelve months. 

Results: 319 patients were recruited from 47 pediatric gastroenterological centers in Germany (IG: 21 centers and 160 subjects; CG: 26 centers and 159 subjects). Among the 146 subjects in the IG who were followed up at 12 months, there were an average (mean) of 0.17 care deficits per patient (95% confidence interval [0.10; 0.24]). Among the 134 subjects in the CG who were followed up at 12 months, there were an average (mean) of 0.55 [0.43; 0.66] identified care deficits per patient (p < 0.0001). 

Conclusion: Registry-based feedback can help bring treatment and its documentation into better conformity with the relevant guidelines and thereby reduce or prevent care deficits in children and adolescents with IBD. 

Der Beitrag The Care of Children and Adolescents With Chronic Inflammatory Bowel Disease: A Cluster-Randomized Trial on Improving the Guideline Conformity of Treatment by the Use of the CEDATA-GPGE Patient Registry  erschien zuerst auf CEDATA GPGE – Patientenregister.

Schiller B, Wirthgen E, Weber F, Schiller S, Radke M, Claßen M, Däbritz J; CEDATA-GPGE Study Group. Fecal calprotectin and platelet count predict histologic disease activity in pediatric ulcerative colitis: results from a projection-predictive feature selection. Eur J Pediatr. 2024 Aug;183(8):3277-3288. doi: 10.1007/s00431-024-05554-y. Epub 2024 May 6. PMID: 38709314; PMCID: PMC11263432.

PMID: 38709314
PMCID: PMC11263432
DOI: 10.1007/s00431-024-05554-y

Abstract 

Especially for pediatric patients, proxies of mucosal inflammation are needed. The Pediatric Ulcerative Colitis Activity Index (PUCAI) has been established to predict clinical and endoscopic disease activity. However, histologic inflammation might persist. We applied a special variable selection technique to predict histologic healing in pediatric ulcerative colitis (UC) as parsimoniously (but still as precisely) as possible. The retrospective analysis included data from two study cohorts, comprising 91 visits from 59 pediatric patients with UC. A Bayesian ordinal regression model was used in combination with a projection-predictive feature selection (PPFS) to identify a minimal subset of clinical and laboratory parameters sufficient for the prediction of histologic disease activity. Following the PPFS, CEDATA-GPGE patient registry data were analyzed to investigate the relevance of the selected predictors in relation to PUCAI and Physician Global Assessment (PGA) in up to 6697 patient visits. Fecal calprotectin (FC) and platelet count were identified as the minimal subset of predictors sufficient for prediction of histologic disease activity in pediatric UC. FC and platelet count also appeared to be associated with increasing disease activity as measured by PUCAI and PGA in the CEDATA-GPGE registry. Based on the selected model, predictions can be performed with a Shiny web app. Conclusion: Our statistical approach constitutes a reproducible and objective tool to select a minimal subset of the most informative parameters to predict histologic inflammation in pediatric UC. A Shiny app shows how physicians may predict the histologic activity in a user-friendly way using FC and platelet count. To generalize the findings, further prospective studies will be needed. What is Known: • Histologic healing is a major endpoint in the therapy of ulcerative colitis (UC). • The PUCAI score has been established to predict disease activity in pediatric UC but is not suitable for the prediction of histologic healing. What is New: • Our Bayesian ordinal regression model in combination with a projection-predictive feature selection is a reproducible and objective tool to select the minimal subset of clinical and laboratory parameters to predict histologic inflammation in pediatric UC. • Histologic inflammation in pediatric UC can be non-invasively predicted based on the combination of fecal calprotectin levels and platelet count. 

Keywords: Bayesian; Calprotectin; Histopathology; Inflammatory bowel disease; Platelets; Ulcerative colitis. 

Der Beitrag Fecal calprotectin and platelet count predict histologic disease activity in pediatric ulcerative colitis: results from a projection-predictive feature selection  erschien zuerst auf CEDATA GPGE – Patientenregister.

Leiz M, Knorr M, Moon K, Tischler L, Sohrabi K, Cantez S, Däbritz J, de Laffolie J, van den Berg N; CEDATA GPGE Study Group. How can patient registries facilitate guideline-based healthcare? A retrospective analysis of the CEDATA-GPGE registry for pediatric inflammatory bowel disease. BMC Health Serv Res. 2023 Jun 17;23(1):648. doi: 10.1186/s12913-023-09639-6. PMID: 37330476; PMCID: PMC10276369.

PMID: 37330476
PMCID: PMC10276369
DOI: 10.1186/s12913-023-09639-6

Abstract

Background: Early diagnosis is mandatory for the medical care of children and adolescents with pediatric-onset inflammatory bowel disease (PIBD). International guidelines (‚Porto criteria‘) of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition recommend medical diagnostic procedures in PIBD. Since 2004, German and Austrian pediatric gastroenterologists document diagnostic and treatment data in the patient registry CEDATA-GPGE on a voluntary basis. The aim of this retrospective study was to analyze whether the registry CEDATA-GPGE reflects the Porto criteria and to what extent diagnostic measures of PIBD according to the Porto criteria are documented. 

Methods: Data of CEDATA-GPGE were analyzed for the period January 2014 to December 2018. Variables representing the Porto criteria for initial diagnostic were identified and categorized. The average of the number of measures documented in each category was calculated for the diagnoses CD, UC, and IBD-U. Differences between the diagnoses were tested by Chi-square test. Data on possible differences between data documented in the registry and diagnostic procedures that were actually performed were obtained via a sample survey. 

Results: There were 547 patients included in the analysis. The median age of patients with incident CD (n = 289) was 13.6 years (IQR: 11.2-15.2), of patients with UC (n = 212) 13.1 years (IQR: 10.4-14.8) and of patients with IBD-U (n = 46) 12.2 years (IQR: 8.6-14.7). The variables identified in the registry fully reflect the recommendations by the Porto criteria. Only the disease activity indices PUCAI and PCDAI were not directly provided by participants but calculated from obtained data. The category ‚Case history‘ were documented for the largest part (78.0%), the category ‚Imaging of the small bowel‘ were documented least frequently (39.1%). In patients with CD, the categories ‚Imaging of the small bowel‘ (χ² = 20.7, Cramer-V = 0.2, p < 0.001) and ‚Puberty stage‘ (χ² = 9.8, Cramer-V = 0.1, p < 0.05) were documented more often than in patients with UC and IBD-U. 

Conclusion: The registry fully reproduces the guideline’s recommendations for the initial diagnosis of PIBD. The proportion of documented diagnostic examinations varied within the diagnostic categories and between the diagnoses. Despite technological innovations, time and personnel capacities at participating centers and study center are necessary to ensure reliable data entry and to enable researchers to derive important insights into guideline-based care. 

Keywords: Pediatric inflammatory bowel disease (PIBD); Registry; Treatment guidelines. 

Der Beitrag How can patient registries facilitate guideline-based healthcare?  A retrospective analysis of the CEDATA-GPGE registry for pediatric inflammatory bowel diseaseHow can patient registries facilitate guideline-based healthcare? erschien zuerst auf CEDATA GPGE – Patientenregister.

PMID: 36873644
PMCID: PMC9975712
DOI: 10.3389/fped.2023.1043067

Abstract

Background: Complications of Crohn’s disease (CD) often impair patients‘ quality of life. It is necessary to predict and prevent these complications (surgery, stricturing [B2]/penetrating [B3] disease behavior, perianal disease, growth retardation and hospitalization). Our study investigated previously suggested and additional predictors by analyzing data of the CEDATA-GPGE registry. 

Methods: Pediatric patients (< 18 years) diagnosed with CD with follow up data in the registry were included in the study. Potential risk factors for the selected complications were evaluated by performing Kaplan-Meier survival curves and cox regression models. 

Results: For the complication surgery, the potential risk factors older age, B3 disease, severe perianal disease and initial therapy with corticosteroids at the time of diagnosis were identified. Older age, initial therapy with corticosteroids, low weight-for-age, anemia and emesis predict B2 disease. Low weight-for-age and severe perianal disease were risk factors for B3 disease. Low weight-for-age, growth retardation, older age, nutritional therapy, and extraintestinal manifestations (EIM) of the skin were identified as risk factors for growth retardation during the disease course. High disease activity and treatment with biologicals were predictors for hospitalization. As risk factors for perianal disease, the factors male sex, corticosteroids, B3 disease, a positive family history and EIM of liver and skin were identified. 

Conclusion: We confirmed previously suggested predictors of CD course and identified new ones in one of the largest registries of pediatric CD patients. This may help to better stratify patients‘ according to their individual risk profile and choose appropriate treatment strategies. 

Keywords: complication; disease behavior; growth; hospitalization; outcome; perianal disease; prediction; surgery. 

Der Beitrag Predicting complications in pediatric Crohn’s disease patients followed in CEDATA-GPGE registry  erschien zuerst auf CEDATA GPGE – Patientenregister.

Scientific Reports volume 12, Article number: 21162 (2022) Published: 07 December 2022

DOI: https://doi.org/10.1038/s41598-022-25487-6

Abstract

The incidence and prevalence of pediatric-onset inflammatory bowel disease (PIBD) are on the rise worldwide. Initial symptoms are often recognized with a delay, which reduces the quality of life and may lead to an increased rate of complications. The aim of this study was to determine the diagnostic delay in PIBD and to identify potential influencing factors. Therefore, data from the German-Austrian patient registry CEDATA-GPGE for children and adolescents with PIBD were analyzed for the period January 2014 to December 2018. There were 456 children identified in the data, thereof 258 children (57%) with Crohn’s disease (CD) and 198 children (43%) with Ulcerative colitis (UC). The median age was 13.3 years (interquartile range (IQR) = 10.9−15.0), and 44% were females. The median diagnostic delay was 4.1 months (IQR = 2.1–7.0) in CD and 2.4 months (IQR = 1.2–5.1) in UC (p = 0.01). UC was associated with earlier diagnosis than CD (p < 0.001). Only a few factors influencing the diagnostic delay have been verified, e.g., abdominal pain at night and if video capsule endoscopy was performed. Diagnostic delay improved over the years in participating centers, but the level of awareness needs to be high even in common symptoms like abdominal pain.

Der Beitrag Diagnostic delay in children with inflammatory bowel disease in the German-Austrian patient registry CEDATA-GPGE 2014–2018 erschien zuerst auf CEDATA GPGE – Patientenregister.

DOI: https://doi.org/10.21203/rs.3.rs-1901469/v1

Abstract

Background: Early diagnosis is mandatory for the medical care of children and adolescents with pediatric-onset inflammatory bowel disease (PIBD). International guidelines (‘Porto criteria’) of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition recommended adequate medical diagnostic procedures in PIBD. Since 2004, German and Austrian pediatric gastroenterologists document diagnostic and treatment data in the patient registry CEDATA-GPGE. The aim of this retrospective study was to analyze whether the registry CEDATA-GPGE reflects the Porto criteria and to what extent diagnostic measures of PIBD according to the Porto criteria are documented.

Methods: Data of CEDATA-GPGE were analyzed for the period December 2013 to December 2018. Variables representing the Porto criteria for initial diagnostic were identified and categorized. The average of the number of measures documented in each category was calculated for the diagnoses CD, UC, and IBD-U. Differences between the diagnoses were tested by Chi-square test. Data on possible differencesbetween data documented in the registry and diagnostic procedures that were actually performed were obtained via a sample survey.

Results: There were 547 patients included in the analysis. The median age of patients with incident CD (n=289) was 13.6 years (IQR: 11.2-15.2), of patients with UC (n=212) 13.1 years (IQR: 10.4-14.8) and of patients with IBD-U (n=46) 12.2 years (IQR: 8.6-14.7).

The variables identified in the registry fully reflect the recommendations by the Porto criteria. Only the disease activity indices PUCAI and PCDAI were not included. The category ‘Case history’ were documented for the largest part (78.0%), the category ‘Imaging of the small bowel’ were documented least frequently (39.1%). In patients with CD, the categories ‘Imaging of the small bowel’ (χ2=20.7, Cramer-V=0.2, p<0.001) and ‘Puberty stage’ (χ2=9.8, Cramer-V=0.1, p<0.05) were documented more often than in patients with UC and IBD-U.

Conclusion: The registry fully reproduces the guideline’s recommendations for the initial diagnosis of PIBD. The proportion of documented diagnostic examinations varied within the diagnostic categories and between the diagnoses. Despite technological innovations, time and personnel capacities at participating centers and study center are necessary to ensure reliable data entry and to enable researchers to derive important insights into guideline-based care.

© Research Square 2023 | ISSN 2693-5015 (online)

Der Beitrag Can patient registries contribute to guideline-based healthcare? A retrospective analysis of the CEDATA-GPGE registry for pediatric inflammatory bowel disease erschien zuerst auf CEDATA GPGE – Patientenregister.

Front. Pediatr., 19 July 2022, Sec. Pediatric Gastroenterology, Hepatology and Nutrition
Volume 10 – 2022 | https://doi.org/10.3389/fped.2022.903677

Abstract

Background and aims: In recent years, biological agents, such as anti-TNF-α blockers, have been introduced and have shown efficacy in pediatric patients with inflammatory bowel disease (IBD). Here, the prescription mode differentiated into a first/second line application, and efficacy and side effects are evaluated beginning from 2004 until today.

Methods: Statistical analyses of the prospective and ongoing CEDATA multicenter registry data from the Society of Pediatric Gastroenterology and Nutrition (GPGE) were performed for patients receiving a biological agent at least once during the period from June 2004 until November 2020 (n = 487). The analyzed parameters were patient demographics, disease extent and behavior, prior or concurrent therapies, duration and outcome of biological therapy, disease-associated complications, drug-related complications, laboratory parameters and treatment response as determined by the Physician’s Global Assessment.

Results: Crohn’s disease (CD) was present in 71.5% of patients, and 52% were boys. Patients showed high disease activity when receiving a first-line TNF-α blocker. After 2016, patients who failed to respond to anti-TNF-α induction therapy were treated with off-label biologics (vedolizumab 4.3% and ustekinumab 2.1%). Propensity score matching indicated that patients with CD and higher disease activity benefitted significantly more from early anti-TNF-α therapy. This assessment was based on a clinical evaluation and lab parameters related to inflammation compared to delayed second-line treatment. Additionally, first-line treatment resulted in less treatment failure and fewer extraintestinal manifestations during TNF-α blockade.

Conclusion: First-line treatment with anti-TNF-α drugs is effective and safe. An earlier start significantly reduces the risk of treatment failure and is associated with fewer extraintestinal manifestations during longitudinal follow-up.

Copyright © 2022 Claßen, de Laffolie, Claßen, Schnell, Sohrabi and Hoerning. 

Der Beitrag Significant advantages for first line treatment with TNF-alpha inhibitors in pediatric patients with inflammatory bowel disease – Data from the multicenter CEDATA-GPGE registry study erschien zuerst auf CEDATA GPGE – Patientenregister.

Front. Pediatr., 03 June 2022, Sec. Pediatric Gastroenterology, Hepatology and Nutrition
Volume 10 – 2022 | https://doi.org/10.3389/fped.2022.883183

Abstract

Objective: Among patients with inflammatory bowel disease (IBD), the risk of thromboembolism (TE) is increased, representing a relevant cause of morbidity and mortality. In contrast to other extraintestinal IBD manifestations, TE receives much less attention because of its low incidence, estimated at merely 0.4–0.9% in hospitalised children with IBD.

Methods: Cases with TE, as documented in the German-Austrian Paediatric IBD registry gesellschaft für pädiatrische gastroenterologie und ernährung – large paediatric patient registry (CEDATA-GPGE), were analyzed retrospectively. For all patients with signs of TE, a questionnaire was filled in by the treating paediatric gastroenterologist.

Results: Over 10 years, 4,153 paediatric patients with IBD (0–18 years) were registered in the registry, and 12 of them identified with TE. Eight patients were diagnosed with ulcerative colitis (UC), three with Crohn’s disease (CD), and one with IBD-unclassified. The median age at IBD diagnosis was 10 years and at the manifestation of TE 13 years, respectively, with a median latency to TE of 2 years. Prevalence of TE was 0.3%, with a significantly higher risk for patients with UC than CD (OR 5.9, CI 1.56–22.33, p = 0.008). More girls than boys were affected (f:m = 7:5) without reaching significance. Approximately 90% of patients experienced TE during active disease, with relevant cerebral and limb involvement in 6/12 patients. Various risk factors, e.g., hospitalisation, coagulopathy, or anaemia were identified. TE management included intensive care and surgery. Among the 12 patients, 11 recovered fully, in which one patient has focal epilepsy as a sequela.

Conclusion: Paediatric patients with IBD have a substantially increased risk for TE. Risk factors, such as those identified should be considered when managing paediatric IBD and preventive measures for those hospitalised taken routinely. Initiating pharmacological thromboprophylaxis is challenging for the lack of published trials on efficacy and safety in paediatric IBD but should be considered carefully in each case.

Copyright © 2022 De Laffolie, Ballauff, Wirth, Blueml, Rommel, Claßen, Laaß, Lang, Hauer and the CEDATA-GPGE Study Group. 

Der Beitrag Occurrence of Thromboembolism in Paediatric Patients With Inflammatory Bowel Disease: Data From the CEDATA-GPGE Registry erschien zuerst auf CEDATA GPGE – Patientenregister.

Front. Pediatr., 31 May 2022, Sec. Pediatric Gastroenterology, Hepatology and Nutrition
Volume 10 – 2022 | https://doi.org/10.3389/fped.2022.8759

Abstract

Introduction: Pediatric patients with inflammatory bowel disease (IBD) are classified into Crohn’s disease (CD), ulcerative colitis (UC), and unclassifiable (IBD-U). However, data provide evidence that ileal CD (L1) is distinct from colonic CD (L2). The aim of this study was to investigate the clinical features of isolated Crohn’s colitis in a pediatric population.

Material and Methods: Children who were prospectively included in the CEDATA–GPGE registry on diagnosis were compared according to the diagnosis of CD with L2 vs. L1 and ileocolonic (L3) involvement pattern as well as IBD-U and UC. The clinical significance of L2 was investigated with regard to extraintestinal manifestations, treatment, surgery, and disease activity.

Results: Fifty-two patients with L2 CD at a median age of 13.4 years (±3.8 SD) were compared with 182 L1 (13.8 ± 2.9 SD), 782 with L3 (12.8 ± 3.3 SD), 653 with UC (12.7 ± 3.8 SD), and 111 patients with IBD-U (11.9 ± 4.7 SD). Bloody stools at diagnosis were more common in L2 (44%) than in L1 (19.7%) and L3 (28.8%), but not as common as in UC (66.5%) and IBD-U (61.3%). Fewer CD patients with L2 (10.2%) received exclusive enteral nutrition therapy (EEN) as induction than patients with L1 (34.3%) and L3 (33.3%). After induction therapy, 42.3% of patients with L2 received immunosuppressants and 21% biologicals during follow-up (L1 56.5/10.5%; L3 59/21%; CU 43.5/11.9%; IBD-U 26.1/12.6%). Extraintestinal manifestations were more frequent in L2 (23.1%) vs. L1 (18.7%), L3 (20.2%), CU (15.8%), and IBD-U (11.7%). The number of patients requiring surgery did not differ within the CD subgroups and was significantly lower in UC and IBD-U. Perianal fistula surgery was significantly more common in L2 (44%) than in L1 (4.8%) or L3 (21.7%). In addition, the frequency of surgery for perianal abscesses was also more frequent in L2 (55.6%) than in L1 (12.7%) or L3 (38.4%).

Conclusions: The consideration of pediatric Crohn’s colitis as a distinct disease seems necessary as it is characterized by extraintestinal manifestations (EIMs) with mainly joint involvement and perianal fistulas or abscesses requiring surgery and biologic therapy. Thus, colonic Crohn’s disease may have an influence on the therapeutic stratification and should be addressed in further studies.

Copyright © 2022 Elonen, Wölfle, de Laffolie, Posovszky and the CEDATA–GPGE-Study-Group. 

Der Beitrag Isolated Crohn’s Colitis: Is Localization Crucial? Characteristics of Pediatric Patients From the CEDATA–GPGE Registry erschien zuerst auf CEDATA GPGE – Patientenregister.

Molecular and Cellular Pediatrics volume 9, Article number: 6 (2022) Published: 05 April 2022

• PMID: 21051046
• DOI:https://doi.org/10.1186/s40348-022-00139-x

Abstract

Background: Exclusive enteral nutrition (EEN) is the first-line therapy for pediatric-onset Crohn’s disease (CD) patients. CEDATA-GPGE® is the largest patient registry for children and adolescents with inflammatory bowel disease (IBD) in Europe, collecting data from over 5000 patients since 2004 in Germany and Austria. Since the application of EEN over 8 weeks is difficult and a high dropout rate is often described, the mode of application including a supporting structure is crucial for success. The aim of this study was to ascertain the variation in the application of EEN across the participating centers and to associate these with the outcome.

Results: Thirty-one centers responded to the survey (81.6%). 88.5% of CD patients were recommended EEN for induction therapy, 71.8% actually started with EEN, and 22.1% terminated the EEN prematurely. The duration of EEN typically lasted 6 to 8 weeks, and the polymeric formula was mainly used. 80.6% of the clinics added flavorings to the formulas. After EEN, the most preferred diet for maintenance therapy was a healthy, well-balanced diet considering individual intolerances.

Conclusions: EEN is widely recommended as an induction therapy by the German and Austrian pediatric gastroenterologists for children and adolescents with CD. However, this questionnaire-based study has shown a wide variation in EEN protocols used by the different pediatric clinics of CEDATA-GPGE®.

© 2023 BioMed Central Ltd unless otherwise stated. Part of Springer Nature.

Der Beitrag Implementation of exclusive enteral nutrition in pediatric patients with Crohn’s disease—results of a survey of CEDATA-GPGE reporting centers erschien zuerst auf CEDATA GPGE – Patientenregister.