bluuebunny/crossref_metadata_2025_split huggingface dataset:
P360 Aggressive Inflammatory Bowel Disease is associated to Hermansky-Pudlak Syndrome: Characterization of a Puerto Rican Cohort
Authors: Marrero Torres R , Coste-Sibilia S , Nieves-Jimenez H , Torres G , Rivera E , Torres E A 
Published: May 2021

Abstract

Background
Hermansky-Pudlak Syndrome (HPS), a rare autosomal recessive disorder, is the most common single-gene disorder in Puerto Rico. It is characterized by an aberrant biogenesis of lysosome-related organelles inducing oculocutaneous albinism, bleeding diathesis, and damage to other organs. Around 30% of patients with HPS develop granulomatous enterocolitis, a Crohn’s-like inflammatory bowel disease (IBD). The mutant HPS1 gene is associated with a more severe Early Onset IBD (EO-IBD). We wish to describe a group of patients with HPS-associated IBD.

Methods
Puerto Ricans with HPS and concomitant IBD were recruited in our IBD clinic for a prospective cohort study. After informed consent, demographic and medical data were collected from the patient and the medical record. Variables included sex, HPS genotype, age at the time of IBD diagnosis, IBD location and behavior according to Montreal and Paris Classification, surgical interventions, medications, and extra-intestinal manifestations (EIM). Descriptive analysis was performed calculating mean and standard deviations. This study is approved by the MSC IRB.

Results
13 patients with HPS and IBD, 9 with confirmed genotype 1, were identified: 8 males and 5 females. The mean age at the time of IBD diagnosis was 22 ± 11 years. 6 were diagnosed at ≤ 16 y/o (A1), 6 were diagnosed between 17-40 years (A2) and 1 patient was in the range of EO-IBD (7 y/o). 9 patients had advanced penetrating behavior (B3) of which 7 had concomitant perianal involvement. Most patients (9) had a surgical intervention; 8 have an ostomy. The majority (10) are using biologics (4 with concurrent immunomodulators). The most frequent EIM was peripheral arthropathy seen in 45%.

Conclusion
HPS patients had a severe course of IBD, as shown by age at diagnosis, phenotype, the prevalence of perianal disease, surgery and use of biologics. In comparison to a database of 836 Puerto Ricans with non HPS Crohn’s Disease, those with HPS were more likely to have surgeries (69% vs 55%, P=.303) and use biologics (77% vs 43%, P=.015). Gastrointestinal symptoms including bleeding may be overlooked by the primary physician. Proactive vigilance in patients with HPS to make an early diagnosis of IBD and aggressive medical treatment may result in better outcomes for these patients.
P385 Results of a teleconsultation campaign for Inflammatory Bowel Disease (IBD) patients to check their treatment adherence and assess their level of anxiety during the first COVID-19 lockdown
Authors: Paupard T , Richez C , Verlynde J , Zaharia O , Quartier G , Hudziak H , Delhoustal L 
Published: May 2021

Abstract

Background
Long-term treatment adherence of patients treated for IBD is quite low, around 50% according to literature. Patients are often suspicious of the infectious risks vis-à-vis immunosuppressants and in particular biotherapies.

Methods
Analysis of the treatment adherence and the level of anxiety of IBD patients treated by immunosuppressants during the first French COVID-19 lockdown.
Monocentric prospective observational study.
Teleconsultations were made by the same gastroenterologist not usually involved in patient care, using randomly an active file of 96 IBD patients monitored in a database (Focus MICI®). Treatments: Corticosteroids n = 0; Azathioprine n = 2; Adalimumab n = 9; Infliximab n= 44 (53%); Golimumab n = 1; Vedolizumab n = 11; Tofacitinib n = 0; Methotrexate n = 3; Ustekinumab n = 5; Combotherapy n = 20.

Results
Calls from 09/4 to 07/5 2020. 13/96 (13.5%) unreachable patients (answering machine, wrong number, no answer, working, etc.). No call was refused when patients were reached. Analysis on 83 patients. Average age: 41.7 years; M / F (55/41) [20-80]. Crohn / RCH: 72/24. Call duration: 5 to 22 minutes. Clinical remission estimated by the patient: Yes / No: 73/10 (88%). Clinical signs possibly linked to Covid: 9/86 (10.4%); discontinuation of treatment: 2/9 (22%). Finally, the treatment was stopped for 11/83 (13%) patients in this IBD population, most of them were in remission. Only 2/9 (22%) with potential Covid symptoms had stopped their treatment. On an anxiety scale from 1 to 10, 41% of patients were moderately to very worried. Among the main fears: increased risk of Covid-19 infection during treatment and fear of coming to the hospital during the epidemic (nosocomial risk). None of the patients spontaneously took the initiative to call first their referent gastroenterologist to discuss the situation. For the others (87%), continuation of treatment with an adherence estimated between 8 and 10 on a scale from 1 to 10.

Results of the call
the phone call was generally much appreciated by IBD patients. Most of the patients were reassured and convinced to continue their treatment and to contact their referent gastroenterologist.

Conclusion
Our results show that the first Covid-19 lockdown was responsible for a high level of anxiety among patients (41%) and a negative effect on patient compliance (not necessarily potential Covid + patients). Advantages of teleconsultation: good acceptability, excellent effectiveness in terms of reassurance and treatment adherence. The cessation of the treatment was in most cases hidden to the doctor. We think that there is a subsequent interest in maintaining tele/visio consultations in addition to face-to-face consultations.
P467 Perception Gap between Patients with Inflammatory Bowel Disease (IBD) and Physicians in China: A Cross-sectional Study
Authors: Liang J , Cao Q , Chen M , Wu K 
Published: May 2021

Abstract

Background
The aim of the study was to assess the perception of inflammatory bowel disease (IBD) among Chinese patients and physicians.

Methods
Data in this cross-sectional study was collected from 6/2020 to 8/2020, which covered 24 provinces/cities in China and based on two surveys that was designed for collecting information from patients with Crohn’s disease (CD)/ulcerative colitis (UC) and the physicians.

Results
Surveys were completed by 1058 patients (601 CD and 457 UC patients) and 351 physicians (181 treating CD and 170 treating UC). For the control of disease symptoms over the past 12 months, physicians believed that 65.19% and 57.65% of CD and UC patients, respectively, were managed without affecting their quality of life. Meanwhile, 56.07% and 49.46% of CD and UC patients, respectively, reported that disease control did not affect their quality of life. Physicians estimated that 87.85% of CD and 88.24% of UC patients were satisfied with treatment results. And 86.36% of CD and 79.43% of UC patients were satisfied with the current medications. Physicians prescribed biologics for 69.52% of IBD patients, and 48.3% of IBD patients actually used them. Repeated medication (30.95%), diarrhea (27.95%) and abdominal pain (25.29%) were most frequently ranked as most bothersome symptoms by CD patients. For physicians, abdominal pain (42.54%) was the most bothersome symptoms, followed by repeated medication (23.76%) and diarrhea (17.13%). Regarding UC symptoms, 51.18% of physicians believed that bloody stool was the most bothersome symptom for patients, and 41.58% of patients held this view. The sense of urgency was concerned as one bothersome symptom by 19.69% patients, while only 4.12% of physicians held the same views (Fig 1). Regarding the efficacy evaluation, in view of the CD physicians, 42.5% of CD patients were believed achieving completely or mostly controlled, while from the patients’ perception, only 32.6% of CD patients agreed. Similarly, the proportion of UC patients who was believed by physicians that achieving completely or mostly controlled (40.0%) was also higher than that of UC patients who believed by themselves (Fig 2).

Conclusion
Most of the IBD patients and physicians were satisfied with the treatment results and the current medications. The disease control expectation of the patients was slightly more than that of the physicians. Further studies are needed to explore factors that associated with the treatment expectation of the patients.
P479 Biological therapies and bio-microbial dynamics in inflammatory bowel diseases
Authors: Tavakoli Medical scientist P , Vollmer-Conna U , Hadzi-Pavlovic D , Grimm M C , Vázquez-Campos X 
Published: May 2021

Abstract

Background
Therapeutic aims in IBDs are to induce remission through rapidly effective treatment. Conventional therapeutic methods including immunosuppression have been available for more than half a century with biological therapies the treatment of choice in patients who fail immunosuppressive drugs. There has been little attempt to longitudinally examine the differences in biopsychological factors and their associations with treatment modalities in IBD patients.

Methods
50 IBD participants (24 UC, 26 CD) in clinical remission were followed for 12 months. Complete longitudinal datasets and monthly stool and blood samples for inflammatory biomarkers were collected for analysis of association. Microbiome analysis was performed using V4 16SrRNA for identification of microbial phylogenetic relationships. Patients were grouped on whether or not they were in remission and on biological agents.

Results
At the baseline there were similar ecological indices between the two treatment options in both disease classes; (Shannon, p = 0.380; Pielou’s, p = 0.246; Chao1, p = 0.934). At family level, the CD group showed significant differences (Wilcoxon, p ≤ 0.05) between the treatment received (biologics vs non-biologic) in the abundance of Barnesiellaceae, Bifidobacteriaceae, unclassified Clostridia, and Clostridiaceae (Figure 1A). Results showed similar ecological index values in samples from the two treatment options.
Samples in UC groups on biological and non-biologic treatment modes were found to have significantly different microbial communities based on Bray-Curtis dissimilarity (PERMANOVA, p = 0.0031). Microbial family composition in UC group and on two treatment modalities showed a total of 12 families that were significantly different (Wilcoxon, p ≤ 0.05): Acidaminococcaceae, unclassified Bacteroidales, Barnesiellaceae, Christensenellaceae, Clostridiales vadinBB60 group, Coriobacteriaceae, Defluvitaleaceae, Eggerthelaceaem Fusobacteriaceae, Muribaculaceae, Prevotellaceae, and Streptococcaceae (Figure1B). Results identified significant differences in microbial diversity (Shannon index, p = 0.041), and evenness (Pielou’s evenness, p = 0.045) between the two treatment modes. No differences were identified in richness (Chao1) in UC participants.

Conclusion
Conclusion: Baseline biological indices were mostly similarly distributed between the two treatment options in remissive IBD patients, however biological therapy significantly influenced longitudinal trends of some microbiome dynamics, especially in UC. This suggests an underlying interrelationship between mode of treatment and biopsychological trajectories which might overshadow response to the treatment, requiring further assessment.
P510 Postoperative enterocoutaneous fistula after surgery for Crohn’s disease performed in a tertiary centre over 15 years: analysis of the long-term outcomes and predictors of fistula closure
Authors: Parlanti1 D , Poggioli G , Cardelli S , Tanzanu M , Boschi L , Rottoli M 
Published: May 2021

Abstract

Background
Patients with Crohn’s disease (CD) have an increased risk to develop enterocoutaneous fistula (ECF) after surgery. Although conservative therapy could be advisable, in some cases surgery is unavoidable, despite there might be greater risks of intestinal failure associated with redo surgery.

Methods
CD patients affected by postoperative ECF (within 90 days from surgery) between 2004 and 2020, and followed for at least 3 months after the onset of the ECF, were retrospectively included. Variables were presented as median (range) or number (%).Chi-square, Fisher’s exact and Wilcoxon rank sum tests were used as appropriate. The Kaplan-Meier method was performed to analyse the rate of ECF closure over the follow-up time, and to compare the outcome between subgroups of patients.

Results
Eighteen patients were included in the study. The perioperative variables are reported in Figure 1.

The median follow-up time was 29.5 months (range 0–204), and the median time to ECF closure was 104 days (24–954), although a clinical remission (defined as an ECG non requiring hospitalization) was obtained after a median time of 41 days (15–768). The comparison of the rates of ECF closure between subgroups of patients over the follow-up are reported in Figure 2 and 3.

Conclusion
The ECF output is the only variable which is significantly associated with the rate of ECF closure. The origin of ECF from an ileo-colic or a colo-colic anastomosis seems to be associated with a faster progression towards fistula healing as compared to an origin from the small bowel (ECF closure at 3 and 6 months: 56.3% vs 22.2% and 70.8% vs 33.3%, respectively; p=0.19). The use of NPWT and, when feasible, an early surgical treatment, might increase the chance of ECF closure within 3 months (55.5% vs 12.5%, p=0.43 and 66.7% vs 29.9%, p=0.32, respectively).
Although postoperative ECF represents a challenging complication in CD, the present study shows that a complete closure is obtainable in the long-term in all patients treated in a referral centre.
P558 Medication Preference of 400 patients with mild to moderate Ulcerative Colitis
Authors: Mackenzie-Smith L , Wüthrich H , Laoun R 
Published: May 2021

Abstract

Background
Patient adherence to his/her medication is a major challenge in the successful management of any chronic disease and it is a major challenge in Ulcerative Colitis (UC). Patient adherence is thought to be closely related to patient preference of medication and formulation used.
We wanted to investigate the patient preference between tablets and granules of mesalazine and compare it to the physician perception.

Methods
We conducted an online survey between November and December 2020 in 4 EU countries (France, Germany, Spain, and the UK). In addition to patients and physicians from these 4 countries, the questionnaire was also addressed to IBD nurses from the UK. Patients were included if they had mild or moderate UC.

Results
400 patients, 160 physicians, 20 nurses participated in this survey. 68% patients were taking tablets and 32% granules. 62% of the patients who answered the survey admitted their preference for tablet while only 38% for granules (figure 1). 63.9% of the physician would prefer to prescribe tablets for their patients.

Physicians stated that patients tend to be more adherent to tablets than granules (69% vs. 31%) and patients tend to find this the most convenient formulation.
When patients were asked about some positive attributes of tablets, the highest agreement was with “good size, easy to see and handle” (7.6/10) while for granules “no problem to take granules in public” (8.4/10).
When asked about some negative attributes of tablets, the highest agreement was with “I would like to take fewer each day” (6.1/10) and “I wish I could take fewer at a time” (5.4/10) while for granules “you have to drink a lot of liquid for them to go down” (6.6/10) and “I wish I could take fewer” (5.1/10).
50 patients were switched to tablets after trying granules first and 45 to granules after trying tablets. Treatment changes were initiated mainly by the physician (57% of the time). Patients switching to granules almost all agreed that they wanted to take fewer tablets each day (8.1/10). Patients switching to tablets complained about the need to drink a lot of water with granules (6.5/10) or to mix them with food (6.3/10).
This is also reflected in the way patients take tablets (figure 2) or granules (figure 3). While 71% of the patients swallow the tablets whole only 35% of the patients swallow all the granules at once. 37% mix the granules with food or liquid, 20% divide the portions to be able to swallow them and 8% chew the granules.

Asked about adherence, only 21% (85 patients) admitted taking less than 80% of prescribed medication.

Conclusion
The majority of the patients prefer the tablet formulation. A high strength tablet overcoming the pill burden could be a good solution to address patient expectations.
P614 A strategy to solve complexity of physician diagnosis using a mixture of Patient Reports and Provider Reports: A Case Study of Crohn’s Disease Phenotypes
Authors: Zhang L , Fan Y , Thompson J , Bohn R , Negar O , Brodovicz K , Parakkal D 
Published: May 2021

Abstract

Background
The IBD Plexus® Registry, established by the US Crohn’s & Colitis Foundation, combines patient and provider reported survey data collected from doctors’ visits and historical data in electronic medical record (EMR) systems. This multi-resource dataset presents data discrepancies in assessing phenotypes of Crohn’s disease (CD). The aim of this analysis is to describe a strategic process to solve data challenges related to CD phenotype definitions based on the IBD Plexus data.

Methods
This cross-sectional, multicenter, US based study used data from 11/2016 to 06/2020 from the IBD Plexus® Sparc program to assess demographics, symptoms and treatments among CD patients. Data discrepancies between CD phenotype status reported and as described in historical EMRs were identified. Physicians may use the Montreal classification with/without the Paris modification for phenotype evaluation. To resolve this discrepancy, we explored and evaluated several study phenotype definitions: 1) using phenotypes at visits, the registry suggested method; 2) using phenotype and history of fistula/abscess or stricture in EMR; and 3) using definition 2 without anal stricture. The implementation included two steps: 1) severe conditions (penetrating, stricturing or both) were considered irreversible and defined using the data at any time before 30 days after the registry consent date; 2) the inflammatory condition was positive in the absence of any other reported severe condition during the entire study period.

Results
The frequency results by phenotypes show small differences across definitions (Figure 1). The discrepancy in frequency by definition1 demonstrated the phenotypes recorded at visits contradicted phenotypes in the EMR. For instance, 0.1%-3.2% or 0.1–0.7% of CD inflammatory patients had subtypes of stricture and subtypes of fistula/abscess, respectively. About 0.5% of CD stricturing patients had intra-abdominal abscess or other fistula (Figure 2). Among CD penetrating patients, 32.0% had history of ileal stricture (Figure 3). Including EMR phenotype variables in definition 2, all discrepancies were resolved. With verification, anal strictures are due to perianal disease which should not be used in the stricturing definition; therefore, the anal stricture was exempt from definition 3. All subtype phenotypes showed 0% discrepancy with study phenotype. A small percentage of positive anal stricture patients was allowed in definition 3.

Figure 1.

Figure 2.

Figure 3.

Figure 4.

Conclusion
When handling the mixture of patient reported and provider-reported data, data discrepancies have many causes. Clarifying the clinical rationale is a key process to resolve discrepancies and accurately define measures of interest.
P617 Trends in Pediatric Inflammatory Bowel Disease-attributable direct costs: A Canadian population-based analysis
Authors: El-Matary W , Nugent Z , Witt J , Bernstein C 
Published: May 2021

Abstract

Background
Background: In addition to its symptomatic burden, inflammatory bowel disease (IBD) has a major financial burden on patients and healthcare systems. However, there is a paucity of evidence on IBD-attributable costs in children. In a population-based analysis, we determined the trends of IBD-attributable direct costs over 22 years.

Methods
Data were extracted from Manitoba Health Provider Claims, and other population registry datasets from 1995 to 2017. Children with IBD were matched by age, sex, and location with children without IBD. IBD-attributable direct costs were calculated using utilization counts from the administrative data and cost estimates from different sources. Inpatient hospitalization and outpatient procedure costs were estimated using the resource intensity weight (RIW) that is attached to each record in the data. Costs were expressed in 2018 Canadian dollars.

Results
We included 733 (428 with Crohn’s disease) prevalent cases who were diagnosed with IBD before the age of 18 years and were followed for 2450 person-years. An age, sex and geography matched control group of 6763 persons without IBD who were followed for 21558 person-years was included. The median annual physician’s billing fees increased from $381 (IQR 215–1064) in 1995 to $936 (IQR 579–1932) in 2017 (p&lt;.001). The annual ambulatory care days for persons with IBD also significantly increased from a median of 8 days per year (IQR 5–13) in 1995 to 12 days per year (IQR 8–17) in 2017 (p &lt; 0.0001) while they remained stable (median 2 days per year) in the control group (p=0.12). The annual medication costs per patient increased from a median of $270.9 in 1995 to $7944 in 2017 (p&lt;.0001). Between 2012 and 2017, the median annual medication costs for all persons on anti-tumor necrosis factor (TNF) medications were $28,582 (IQR 21854–36736)compared to $861for persons with IBD not on an anti-TNF agent (IQR 355–1,714) (p&lt;0.0001) with a relatively stable costs over the 5-year period. The median annual direct costs per patient with IBD was $1810.716 in 2004 as compared to $14791.55 (p&lt;.0001) in 2017.

Conclusion
Over 2 decades, there was a significant increase in the pediatric IBD-attributable direct costs mainly driven by medication costs mainly anti-TNF biologics.