Introduction

The standard treatment for T1b esophagogastric junctional adenocarcinoma (EGJAC) is surgery. However, surgery is highly invasive especially for elderly patients. Endoscopic submucosal dissection (ESD) is an alternative treatment of T1b cancer for such patients. However, the outcome and long-term prognosis of T1b EGJAC treated by ESD is unknown.

Aims & Methods

The aim of this study is to investigate the outcome and prognosis of T1b EGJAC treated by ESD.
Fifty lesions in 49 patients with pT1b EGJAC who underwent ESD from January 2000 to September 2019 were investigated the outcome and prognosis. The patients were divided into pT1bSM1 (n=30) and pT1bSM2 (n=20).
The patient characteristics were as follows. Male/female: 28/2, 18/2, median age: 69, 75 years old, macroscopic type (protuberance/depression): 5/25, 6/14, main histology (well differentiated (diff.)/ mixed poorly diff.): 22/8, 9/11, median diameter of resected specimen: 45, 39mm, median diameter of tumors: 22, 19mm, median follow-up period: 53, 64months, respectively. There was significant difference in only main histology (p=0.04).
EGJAC was defined as Siewert II, with T1bSM1 for ≤500 μm and T1bSM2 for >501 μm. R0 was defined as lateral and vertical margin negative, and lymph-vascular involvement negative. According to Japanese guidelines, additional treatment was recommended for all T1bSM2 cases and for SM1 lymph duct (ly)-positive and poorly diff. cases.

Results

1. Outcome of ESD
The en bloc resection rate is 100% for both T1bSM1 and T1bSM2. The R0 rate was 100% for T1bSM1 and 95% (19/20) for T1bSM2. Intraoperative perforation rate was 2% (1/50), delayed bleeding rate was 2% (1/50), and delayed perforation rate was 0%. Stenosis rate was 10% (5/50), and all of such complications were treated by endoscopy without surgery.
2. Long-term prognosis
A) T1bSM1 (n = 30)
a) One case (3%) was ly-positive. Additional surgical resection was performed, and lymph node metastasis (LNM) was observed. The patient is alive without recurrence.
b) Twenty-nine cases were ly/vascular (v) negative, and followed up without additional treatment (AT). Nineteen in 29 cases are alive without recurrence, and 10 in 29 cases died by other diseases.
c) Local recurrence rate was 0%, LNM rate was 3% (1/30), and distant metastasis (DM) rate was 0%.
B) T1bSM2 (n=20)
a) Six cases (30%) were ly and/or v positive. Additional surgical resection was performed in 3 cases, and no LNM was observed in any of the cases. Of the other 3 cases, 1 patient underwent only radiotherapy. LNM was found 14 months later, and chemotherapy was added, but treatment-related death occurred. One of the two cases who did not undergo AT had DM and multiple liver metastases 61 months after ESD, and died of the original disease despite of chemotherapy. Another patient died of other diseases.
b) Nine in 14 cases of ly/v negative underwent additional surgical resection, and no LNM was observed in any of the cases. 7 cases are alive without recurrence, and 2 cases died of other diseases. Four in 5 cases who did not undergo AT are alive without recurrence, and 1 patient died from other diseases.
c) R1 rate was 5% (1/20), local recurrence rate was 0%, LNM rate was 5% (1/20), DM rate was 5% (1/20), and mortality by primary cancer was 5% (1/20).

Conclusion

LNM and DM were not observed in the T1bSM1, ly0 group, however LNM and DM were observed in the T1bSM1,ly1 or T1bSM2 groups. Therefore, the current Japanese ESD criteria is appropriate. 　
The R0 resection rate for T1bSM2 was excellent at 95%, and local recurrence was 0%. ESD could be alternative treatment for patients with comorbidity.

References

None

Introduction

The Mediterranean diet identified as a priority because it contributes to the normalization of key metabolic parameters in patients with non-alcoholic fatty liver disease (NAFLD). However, it is necessary to take into account the possibilities of following such a diet and the nutrigenetic characteristics of patients in different regions of the world. Purpose: to study the effect of nutrigenetic parameters in patients with NAFLD on the effectiveness of non-drug treatment.

Aims & Methods

Purpose: to study the effect of nutrigenetic parameters in patients with NAFLD on the effectiveness of non-drug treatment. 105 patients (55 men and 50 women) with NAFLD were examined. In patients, the features of eating disorders (ED) were studied (DEBQ questionnaire), nutrigenetic parameters - 5 polymorphisms: Pro12Ala of the PPARG2 gene (rs1801282), 101027 of the ADRB2 gene (rs1042714) and Arg16Gly of the ADRB2 gene (1010282); (rs4994) and Thr54Ala of the FABP2 gene (rs1799883) associated with the risk of metabolic disorders.

Results

Two polymorphisms associated with disorders of the ED gene (Pro12Ala PPARG2 and Trp64Arg ADRB3) were identified. Sex differences in the frequency of genotypes of the Pro12Ala polymorphism of the PPARG2 gene were not found (pχ2 = 0.90). 83.6% of men and 82.0% of women were carriers of the PPARG2 gene polymorphism (OR=1.10, CI=0.24-4.99) of the Pro12Pro genotype (AB=1.10, men and 16.7% of women of the genotype Pro12Ala (OR = 0.91, OR) = 0.20 - 4.13). The distribution of genotypes and allelic variants of the ADRB3 gene polymorphism had significant differences (px2 = 0.05). The Trp64Trp genotype of the ADRB3 gene polymorphism were found in 80.0% of men and 55.0% of women, the Trp64Arg genotype in 20.0% of men and 32.5% of women, the Arg64Arg genotype was found only in women. An analysis of the distribution of 64Trp and 64Arg alleles by sex using a multiplicative model showed that the significance of the association of the minor female 64Arg allele was confirmed by an odds ratio of 3.13 (CI = 1.01–9.70) versus 0.32 (CI = 0.01–9 .70). 10 - 0.99) for the minor allele. A significant association (px2 = 0.02) of the protective minor allele 12Ala with emotionally restrictive types of ED (OR = 0.11, CI = 0.10 - 0.97) was established, while the external type of oblique ED was associated with the carriage of the main allele polymorphism 12Pro of the Pro12Ala PPARG2 gene (OR = 8.71, CI = 1.03-7.66). Analysis of the distribution of 64Trp and 64Arg alleles depending on the type of EP disorder showed a significant association (px2 = 0.006) of the metabolically unfavorable minor 64Arg allele with the external type of ED disorders (OR = 5.53, CI = 1.48-20.68), while emotional and restrictive types were associated with the carrier of the main 64Trp allele (OR = 0.18, CI = 0.05-0.68). In patients with NAFLD, the external type of ED disorder prevails (p<0.05).

Conclusion

To increase the effectiveness of non-drug treatment, one should take into account nutrigenetic features - the presence of ProARA2 polymorphisms of the PPARG2 gene and Trp64Arg of the ADRB3 gene, which are associated with the peculiarities of nutrient metabolism and malnutrition in patients with NAFLD.

Disclosure

Nothing to disclose

Introduction

Upper gastrointestinal bleeding (UGIB) is one of the main potentially life-threatening emergencies encountered in gastroenterology that requires prompt management. For this reason, numerous prognostic scores have been developed to predict the level of risk in patients. However, there are few studies in the literature comparing these scores.

Aims & Methods

The main objective of this study was to compare the performance of the pre-endoscopic scores Glasgow-Blatchford Score (GBS), Rockall Score (pRS), and quick Sequential Organ Failure Assessment (qSOFA) in predicting the severity of UGIB according to predefined criteria.
Methods: This is a retrospective descriptive and analytical study of 102 patients who presented with upper gastrointestinal bleeding at the International University Hospital Cheikh Khalifa over a period of 1 year between October 2021 and October 2022. Scores were calculated for each patient with complete medical records. The data was analyzed using the Jamovi 2.2.5 software. The accuracy of each risk score in predicting clinical criteria was evaluated by the area under the receiver operating characteristic curve (AUROC). A p-value less than 0.05 was considered statistically significant.

Results

The included patients had a mean age of 57.1 years with a slight female predominance (53.9%). GBS was found to be more predictive of the risk of admission to the Intensive care Unit (ICU) and the need for blood transfusion than pRS and qSOFA with an AUROC of 0.914 for these two criteria. GBS was also more predictive of the risk of therapeutic intervention (AUROC 0.882) compared to pRS (AUROC 0.788) and qSOFA (AUROC 0.676). Regarding endoscopic intervention, pRS and GBS were equally able to predict this criterion with moderate power (AUROC pRS 0.721) (AUROC GBS 0.747). qSOFA showed no significant performance, especially in its preferred domain of admission to the ICU (AUROC 0.735) (Table). None of the 3 scores was able to predict the need for surgical intervention.

Table: Comparison of AUC, sensitivity, specificity, PPV and NPV along with cut-off value of Glasgow-Blatchford Score, pre-Rockall, and Quick-Sofa scores to predict the clinical outcomes

Conclusion

At the end of this study, the use of GBS and pRS in daily clinical practice in emergency departments is strongly recommended to predict the need for therapeutic intervention, hospitalization, or re-bleeding episodes. However, it would be interesting to study the usefulness of these prognostic scores, especially qSOFA, through larger prospective studies.

References

1. Cúrdia Gonçalves T, Barbosa M, Xavier S, et al. Optimizing the Risk Assessment in Upper Gastrointestinal Bleeding: Comparison of 5 Scores Predicting 7 Outcomes. GE Port J Gastroenterol. nov 2018;25(6):299‑307.
2. Tham J, Stanley A. Clinical utility of pre-endoscopy risk scores in upper gastrointestinal bleeding. Expert Rev Gastroenterol Hepatol. déc 2019;13(12):1161‑7.
3. Aquarius M, Smeets FGM, Konijn HW, et al. Prospective multicenter validation of the Glasgow Blatchford bleeding score in the management of patients with upper gastrointestinal hemorrhage presenting at an emergency department. Eur J Gastroenterol Hepatol. sept 2015;27(9):1011‑6.
4. Li Y, Lu Q, Song M, Wu K, Ou X. Comparisons of six endoscopy independent scoring systems for the prediction of clinical outcomes for elderly and younger patients with upper gastrointestinal bleeding. BMC Gastroenterol. 13 avr 2022;22(1):187
5. Renukaprasad AK, Narayanaswamy S, R V. A Comparative Analysis of Risk Scoring Systems in Predicting Clinical Outcomes in Upper Gastrointestinal Bleed. Cureus. juill 2022;14(7):e26669
6. Taslidere B, Sonmez E, Özcan AB, Mehmetaj L, Keskin EB, Gulen B. Comparison of the quick SOFA score with Glasgow-Blatchford and Rockall scores in predicting severity in patients with upper gastrointestinal bleeding. Am J Emerg Med. juill 2021;45:29‑36

Disclosure

All co-authors have seen and agree with the contents of the manuscript and there is no conflict of interest.

Clinical Case Summary

Pancreatic cystic lymphangioma is extremely rare, accounting for less than 5% of pancreatic cystic neoplasms and less than 1% of all lymphangiomas.¹
A 51-year-old female presented with complaints of recurrent abdominal pain for 4 weeks. She was previously misdiagnosed as acute pancreatitis complicated with pancreatic pseudocyst. There was no history of trauma. Dynamic MRI on the abdomen revealed a large, encysted collection at the pancreatic body suggesting a query pancreatic pseudocyst. EUS examination revealed a large cystic lesion at the body of the pancreas. The cyst was multilocular with characteristic multiple thin septa within the lesion (Figure 1). EUS-FNA with 22-G needle was done with aspiration of about 20 ml of cyst fluid, divided into two bottles, one for cytology examination and mucin stain while the second bottle for Amylase, CEA and TG levels. Aspiration fluid was serous yellowish in colour, with negative string sign. Also, a large focal lesion within the spleen was also seen.The biochemical evaluation of the aspirated cystic fluid revealed low amylase and CEA, and high TG levels. Cytopathological examination showed fairly cellular material featuring numerous predominantly small lymphocytes. There were no epithelial, atypical, or malignant cells. IHC staining was positive for CD-31 and CD2-40. Alcian blue mucin stain was negative. The diagnosis of pancreatic cystic lymphangioma was made based on EUS, cytological and biochemical examination with IHC features. Since, lymphangiomas are benign tumors without malignant potential, treatment depends on the symptoms. Lymphangioma can encroach on adjacent organs and need surgical resection. To relieve symptoms of abdominal, distal pancreatectomy and splenectomy was discussed, but patient refuse surgery. Multidisciplinary team decided a conservative management with a follow-up by dynamic MRI and EUS within 6 months.

References

1. Chen D, Feng X, Lv Z, Xu X, Ding C, Wu J. Cystic lymphangioma of pancreas: A rare case report and review of the literature. Medicine (Baltimore). 2018 Jul;97(28): e11238. doi: 10.1097/MD.0000000000011238.

Introduction

It has been reported that diverticulum hemorrhage has a high spontaneous hemostasis rate of 70 to 90% and rarely leads to hemorrhagic shock, so emergency colonoscopy is not widely performed. However, there is a report that the identification rate of stigmata of recent hemorrhage increases with early colonoscopy administration, so we have performed many emergency colonoscopy within 24 hours after the ER visit.

Aims & Methods

We retrospectively examined 68 cases of diverticulum bleeding who visited our hospital from April 2011 to October 2016; 38 cases in the emergency group who underwent colonoscopy within 24 hours and 30 cases in the elective group who underwent colonoscopy after 24 hours.

Results

The age was 73 ± 11 in the emergency group and 72 ± 12 in the elective group. The blood Hb concentration at the first ER visit was 10.0 ± 3.0 g / dL in the emergency group and 10.5 ± 2.4 in the elective group, showing no significant difference. There were no significant differences in blood pressure or pulse rate at the first ER visit. The oral antiplatelet drug use was 39% in the emergency group, which was higher than that in the elective group 23%, but there was no significant difference in the oral anticoagulant and NSAIDS use. The length of hospital stay was 8.0 ± 6.1 days in the emergency group, 8.0 ± 4.6 days in the elective group, and the blood transfusion rate was 53% in the emergency group and 46% in the elective group, showing no significant differences. A significant difference was observed in the rate of identifying bleeding sources. In 26% of the emergency group and 10% of the elective group, stigmata of recent hemorrhage was identified and hemostatic treatment was performed (p<0.05). No major complications such as intestine perforation was observed in the two group.

Conclusion

Emergency colonoscopy for diverticulum bleeding does not reduce blood transfusion during hospital stay, but it identifies bleeding sources and may lead to effective treatment.

Introduction

Immunomodulators and biologicals play an essential role in current IBD management, but are associated with increased risk of infections. In light of the growing number of treatment options, the benefit-risk balance of drugs is becoming increasingly important in clinical decision making. Post-marketing surveillance studies are pivotal to assess infection risk, yet mainly focus on severe infections. As a result, data on mild and moderate infections are scarce. However, mild and moderate infections take longer to clear in immunosuppressed patients, have been reported to be the most burdensome adverse drug reaction according to patients, and can negatively influence drug adherence.

Aims & Methods

Aim: To assess the incidence of all infections and identify risk factors for the development of infections in IBD patients.
Methods: We previously developed and validated a Patient-Reported Infections Questionnaire (PRIQ), with excellent diagnostic accuracy, covering 15 infection categories with a 3-month recall period. [1] The current prospective, multicentre, observational cohort study was performed between Jun, 1 2020 and Jul, 1 2021, enrolling consecutive IBD patients and using the PRIQ which was implemented in myIBDcoach, an established telemedicine platform. Infection severity was defined as mild (self-limiting or topical treatment), moderate (oral antibiotics, antivirals or antifungals) or severe (hospitalization or IV treatment). Incidence rates (IR) were calculated for all infections, stratified for severity and subtype. Multivariable logistic regression, adjusting for relevant confounders, was performed to identify risk factors for infections.

Results

In total, 629 IBD patients (n=346 CD, n=283 UC, 58.3% female, mean age at cohort entry 48.3 years [SD 14.8], mean disease duration 12.4 years [SD 10.8]) were included which completed 2391 PRIQs during 572 person-years (PY) of follow-up. This resulted in 990 reported infections, corresponding to IRs of 17.3, 11.8, 5.1, and 0.4 per 10PY for all, mild, moderate, and severe infections, respectively. Upper respiratory tract (IR 26.9/100PY) and urinary tract infections (IR 14.8/100PY) were the most commonly reported mild and moderate infections types, respectively (Table 1). Compared to patients without treatment, patients on immunosuppressive treatment more frequently experienced infections of any severity (mild vs. no treatment: IR ratio (IRR) 1.57 [95%CI 1.21-2.06] p<0.001, moderate vs. no treatment: IRR 1.42 [95%CI 1.20-1.69] p<0.001). On multivariable logistic regression, female sex (mild adjusted odds ratio [aOR] 1.96 [95%CI 1.38-2.79]; moderate aOR 1.71 [95%CI 1.15-2.54]), smoking status (mild aOR 1.66 [95%CI 1.15-2.41]; moderate aOR 1.86 [95%CI 1.05-3.28]), higher BMI (moderate aOR 1.05 [95%CI 1.02-2.00]), and more comorbidities (mild aOR 2.41 [95%CI 1.46-3.98]; moderate aOR 1.82 [95%CI 1.07-3.11]) were all significantly associated with the development of mild and moderate infections.

Conclusion

​In this real-world prospective study, immune suppressive therapy was associated with mild and moderate infections of any kind in IBD patients. These infections particularly occur in females, smokers, patients with higher BMI and more comorbidities. This information should be considered in personalised treatment selection.

References

[1] Rezazadeh Ardabili A, van Esser D, Wintjens D, et al. Development and validation of a remote monitoring tool for assessment of mild, moderate, and severe infections in Inflammatory Bowel Disease [published online ahead of print, 2023 Feb 16]. J Crohns Colitis. 2023;jjad023. doi:10.1093/ecco-jcc/jjad023

Disclosure

M.P. reports grants and non-financial support from Falk Pharma, grants from European commission, grants from ZonMw (Dutch national research fund), grants and non-financial support from Takeda, grants and non-financial support from Johnson and Johnson, grants and non-financial support from Abbvie, non-financial support from Ferring, non-financial support from Immunodiagnostics, non-financial support from MSD, all outside the submitted work. D.J. reports grants from Top Knowledge Institute (Well on Wheat), grants from Horizon 2020 DISCOvERIE, grants from NWO-CCC Partnership program (Carbokinetics), all outside the submitted work. Z.M. reports grants form MLDS, Niels Stensen Fellowship and Galapagos. A.v.B. has served as speaker, adviser and/or principal investigator for AbbVie, Arandal, Arena, Celgene, Ferring, Galapagos, Janssen, MSD, Pfizer, Roche, Takeda, TEVA, and received research grants from Pfizer, TEVA, Eurostars funding, ZonMw, all outside the submitted work.
The other authors have nothing to disclose relevant to this publication.

Clinical Case Summary

Aims: Williams syndrome is characterized by facial dysmorphic features, mental retardation, a unique cognitive profile, hypercalcemia and connective tissue abnormalities. Gastrointestinal manifestations include feeding problems, gastroesophageal reflux, constipation, diverticulosis, chronic abdominal pain, hernias and celiac disease (Figure), while liver cirrhosis has never been reported.
Case report: We report a male patient who was diagnosed with Williams syndrome (FISH method) at the age of 2.5 years old. He developed the characteristic face with broad forehead, underdeveloped chin, short nose and full cheeks in combination with hypercalcemia, supravalvular aortic stenosis and hypothyroidism. During development, the patient showed slow physical growth, mild mental retardation, hypersociality and hearing disorders and at the age of 7 years old the patient demonstrated transaminasemia and hepatosplenomegaly. Abdominal computed tomography showed hepatomegaly with a lobular margin due to cirrhosis, splenomegaly and the presence of ectopic varices with a palpable splenoportal axis, while gastroscopy revealed Grade 1 esophageal varices. Further serology, genetic mutation and metabolic disorders tests were performed without revealing findings suggestive of the etiology of cirrhosis. Eventually, a percutaneous liver biopsy revealed extensive bridging fibrosis and the formation of incomplete regenerative nodules with macrovesicular steatosis, without specific chronic liver disease as the cause of cirrhosis. No other case of Williams syndrome with development of liver cirrhosis has been described in the literature before. Having ruled out the classic causes of cirrhosis the suspected mechanism may be genetic or metabolic in the context of the syndrome.
Conclusion: Williams syndrome is a complex disorder with variable involvement of different organs, so further research is needed to identify the factors responsible for these variations.

References

1. Morris CA, Mervis CB. Williams syndrome and related disorders. Annu Rev Genomics Hum Genet. 2000;1:461-84.
2. Pober BR. Williams-Beuren syndrome. N Engl J Med. 2010 Jan 21;362(3):239-52.
3. Kozel BA, Barak B, Kim CA, Mervis CB, Osborne LR, Porter M, Pober BR. Williams syndrome. Nat Rev Dis Primers. 2021 Jun 17;7(1):42.
4. Twite MD, Stenquist S, Ing RJ. Williams syndrome. Paediatr Anaesth. 2019 May;29(5):483-490.

Disclosure

Conflict of Interest disclosure
The authors declare that there are not conflicts of interest.