bluuebunny/crossref_metadata_2025_split huggingface dataset:
A293 PREVALENCE OF METABOLIC ASSOCIATED STEATOTIC LIVER DISEASE AND ITS IMPACT ON ADVERSE OUTCOMES IN PRIMARY BILIARY CHOLANGITIS PATIENTS
Authors: Kaviani R , Wong Y , Shreekumar D , Mason A , Montano Loza A , Lytvyak E 
Published: February 2024

Abstract

Background
Metabolic associated steatotic liver disease (MASLD) is highly prevalent among Canadians, thus making coexistence with other liver diseases inevitable. Primary biliary cholangitis (PBC) is the most common autoimmune liver disease diagnosed in one out of 1,000 women above 40. The impact of concomitant MASLD on liver outcomes in patients with PBC is unclear.

Aims
We aimed to determine the prevalence of MASLD among patients with PBC and the association between concomitant liver disease and adverse liver outcomes.

Methods
PBC patients diagnosed between 1984 and 2023 and followed up at the University of Alberta with vibration-controlled transient elastography data available were included. MASLD was diagnosed if the Controlled Attenuation Parameter ≥288 dB/min with at least one cardio-metabolic criteria, based on elevated body mass index, blood pressure, and abnormal glycemic and lipid tests. Proportions were compared using chi-square, medians – Mann-Whitney test. We utilized a Cox hazards regression model to calculate hazard ratios and determine associations between MASLD and the development of cirrhosis or decompensation, need for liver transplant, or death.

Results
A total of 115 patients (87.0% females, age at diagnosis 52.3±11.5 years) followed over a median duration of 11.0 [range 0.1-39.7] years) were included. The prevalence of MASLD (33.0% [n=38]) was higher in females than males (35.0%[n=35] vs. 20.0%[n=3]; p=0.249). The prevalence of cirrhosis at diagnosis was twice as high among PBC patients with MASLD than those without (26.3%[n=10] vs. 13.0%[n=10]; p=0.076). No difference was observed in the frequency of decompensation at diagnosis (8.8%[n=3] vs. 6.3%[n=4]; p=0.638). Cirrhosis frequency (50.0% vs. 50.6%; p=0.948) and decompensation (38.2% vs.37.1%; p=0.912) over the course of the disease were similar between PBC patients with and without MASLD. There was no association between MASLD and development of cirrhosis (HR0.51, 95%CI 0.23-1.13; p=0.095), decompensation (HR0.81, 95%CI 0.37-1.76; p=0.596), liver transplantation (HR0.72, 95%CI 0.19-2.72; p=0.628), or death (HR0.69, 95%CI 0.22-2.16; p=0.519). PBC patients with MASLD have comparable event-free (median 10.7 [3.1-30.8] vs. 10.7 [0.1-23.3] years; p=0.684) and overall survival (median 11.3 [3.2-39.7 vs. 10.7 [0.1-23.3] years; p=0.456).

Conclusions
Every third patient with PBC had MASLD, which tended to be more prevalent among females. We did not observe any significant differences in the development of adverse liver outcomes between patients with PBC and concurrent MASLD in comparison to those without. Studies with larger sample sizes are necessary to further explore the potential impact of MASLD on liver outcomes.

Funding Agencies
None
A305 ANONYMOUS LIVING LIVER DONATION IMPROVES ACCESS FOR MEDICALLY UNDERSERVED CHILDREN IN NEED OF LIVER TRANSPLANTATION
Authors: Yodoshi T , John T , Stunguris J , De Angelis M , Van Roestel K , Hensley J , Avitzur Y , Bandsma R , Jones N , Kamath B M , Ling S , Miserachs M , Ghanekar A , Cattral M , Sayed B , Ng V L 
Published: February 2024

Abstract

Background
Since our first pediatric anonymous non-directed live donor liver transplant (A-LDLT) performed in April 2005, 62 children have undergone live donor liver transplant (LDLT) with an anonymous non-directed graft. Anon-LDLT organs being allocated as per our deceased donor liver transplant (DDLT) wait list.

Aims
To evaluate clinical outcomes, recipient characteristics and social determinants of health of pediatric recipients of A-LDLT in comparison to those who received a directed LDLT (Dir-LDLT) and DDLT.

Methods
Retrospective analysis of all recipients of LDLT performed between January 2005 and March 2023. Demographic and clinical data included age, sex, race, ethnicity, single-parent households, primary diagnosis, recipient blood type, time on waiting list, post-LT intensive care unit (ICU) length of stay (LOS), time to extubation, and post-transplant comorbidities were assessed as covariates. A comparative analysis was conducted between children receiving A-LDLT, Dir-LDLT versus DDLT.

Results
A total of 422 (50% male, 57% white, 23% Asian, 7% Black, 5% indigenous) childrenunderwent LT during the study period. Biliary atresia (40%) and metabolic diseases (19%) were the commonest primary indications. A-LDLT was done in 62 children (15%) with no directed donor options. Dir-LDLT was in 174 children (41%). Of 186 DDLTs (44%), 25% were split grafts, 26% reduced, and 49% whole liver grafts. In A-LDLT recipients, the primary diseases were more frequently characterized by metabolic disorders (31%), while the incidence of acute liver failure was notably lower (0%). Recipients of Anon-LDLT were more often Indigenous recipients in comparison to Dir-LDLT or DDLT recipients (p=0.044). Anon-LDLT recipients were more frequently living in single parent households (18% vs 3% vs 10%, pampersand:003C0.001) and to require interpreter assistance (11% vs 3% vs 0.5%, pampersand:003C0.001) (Table), compared to children who received Dir-LDLT or DDLT. Median time on the wait list was longer for Anon-LDLT (92 days) compared to Dir-LDLT (62 days) and DDLT (72 days) recipients (p=0.004). Post-LT ICU LOS, time to extubation, other post-LT complications or patient prognosis were better in the A-LDLT and Dir-LDLT recipients than DDLT recipients.

Conclusions
This retrospective analysis of 422 children undergoing LT at a single institution confirms excellent patient and graft survival for A-LDLT recipients. Patients from Indigenous communities, single parent and households where English is a second language, are more frequently the beneficiaries of Anon-LDLT grafts. Anon-LDLT, as utilized in this single center analysis, benefits medically underserved pediatric patients who otherwise have limited access to the advantages of LT.

Funding Agencies
None
A307 NEUROGASTROENTEROLOGY & MOTILITY CAID (CHRONIC ATRIAL AND INTESTINAL DYSRHYTHMIA) SYNDROME: A SEVERE LATE-ONSET PEDIATRIC INTESTINAL PSEUDO-OBSTRUCTION
Authors: adouane l , Poinsot P , Castilloux J , Faure C 
Published: February 2024

Abstract

Background
Pediatric intestinal pseudo-obstruction (PIPO) is a rare, heterogeneous and severe gut motility disorder. It is associated with a high morbidity and mortality rate. The etiology of PIPO can be either primary (sporadic or familial), secondary or idiopathic. In primary forms, 50 to 75% of patients will present symptoms in the first month of life and 80% by the end of the first year of life. In 2014, Chetaille et al. described the CAID syndrome related to a recessive SGOL1 mutation as a new form of PIPO associated with cardiac dysrhythmia.

Aims
Our aim is to describe the clinical presentation and the specific digestive and nutritional features of pediatric patients with SGOL1 mutation.

Methods
Monocentric, retrospective and descriptive study. We enrolled children and adolescents aged less than 18 years. All data were collected from patients’ files.

Results
8 patients were included (5 girls), two of them were first-degree related. They all had a typical clinical presentation of PIPO (excessive abdominal distension, abdominal pain and vomiting) but with a later onset than usually seen in primary PIPO (median (range) age: 6.3 years [2-15.6]). The median age (range) at diagnosis was 7.8 years [4-15.7]. In most patients contrast studies showed massively distended small bowel and colon. Antroduodenal manometry was performed in 6 patients and was abnormal with a neuropathic pattern. Others were diagnosed using the ESPGHAN criteria (2018 Jun;66(6):991-1019). All patients needed parenteral nutrition at a median (range) age of 11.8 years [6.5-15.9] due to failure to thrive, intestinal failure and electrolytic disorders. Seven patients needed an ileostomy. One out of 8 patients was off PN at 18 years. Three patients had sinus dysfunction: one at the time of PIPO diagnosis and two sometimes after. All of them required a pacemaker. One patient died in her twenties and it wasn’t related to PIPO or its possible complications.

Conclusions
Recessive SGOL1 mutation, a new cohesinopathy affecting the gut and heart rhythm, represents a late-onset but severe form of PIPO. It is usually associated with sinus dysfunction and cardiac dysrhythmia. However, digestive symptoms appear first. Long term outcome is still to describe.

Funding Agencies
None
A308 AN UNUSUAL CASE OF MACE DYSFUNCTION: THE BENEFIT OF USING ANTEGRADE CONTRAST FOLLOW THROUGH MIMICKING HOME SETTINGS
Authors: Avelar Rodriguez D , Langer J , Gould M 
Published: February 2024

Abstract

Background
Malone Antegrade Continence Enema (MACE) is indicated in cases where medical management for constipation or faecal incontinence with laxatives or enemas has failed. MACE allows the administration of flushes with and without laxatives in an antegrade manner to mechanically empty the colon.

Aims
The aim of this case report is to illustrate an unusual case of MACE dysfunction and how it was troubleshooted.

Methods
Case report and review of the literature

Results
11-year-old female with neurogenic constipation and faecal incontinence for which MACE was being used.
She was referred to clinic for evaluation of new-onset vomiting, abdominal distention, and abdominal pain with MACE flushes. Her flushes consisted of crushed Bisacodyl, glycerin, PEG 3350 and 300 ml of saline solution. Cone enemas were also being used to completely empty her rectum, otherwise evacuation with regular flushes was not occurring, but was causing significant abdominal pain, distention and vomiting.
A contrast study was done by flushing water-soluble contrast through the MACE in an antegrade manner, while simultaneously performing a contrast enema, demonstrating good passage of contrast with no findings suggestive of obstruction.
She was admitted to hospital to complete imaging studies, bowel clean out and to better understand her MACE dysfunction. Following bowel clean out in hospital, MACE flushes were resumed, causing the same symptoms. Flushes without Bisacodyl were tried in the attempt to avoid possible colonic spasms; however symptoms re-occurred.
A second contrast study was performed (figure 1), mimicking home flushes in volume and time of administration, as well as patient’s position (sitting), completed in an antegrade fashion only. The patient developed symptoms 20 minutes after administering the contrast requiring cone enema. By the end of the study, there was poor passage of contrast through the proximal descending colon, suggesting a kink at the juncture between the sigmoid and descending colon. There was no reflux on contrast into the terminal ileum. An x-ray was done around 5 hours after, and contrast was still not seen in the rectum.
The patient underwent diagnostic laparoscopy, finding adhesions in the left upper quadrant of the descending and sigmoid colon, causing a Z-configuration hairpin obstruction, and colostomy creation. The patient has been doing well since colostomy creation with no pain.

Conclusions
MACE dysfunction can be caused by colonic adhesions distal to the MACE site Simultaneous antegrade contrast through MACE and contrast enema may miss bowel obstruction Antegrade contrast study mimicking home settings may be more helpful in situations where bowel obstruction is suspected

Funding Agencies
None
A309 THE DESCRIPTION OF NUTRITION COUNSELLING FOR IRRITABLE BOWEL SYNDROME IN THE LITERATURE IS CURRENTLY LACKING
Authors: Lau M E , Liu L W , Parker C H 
Published: February 2024

Abstract

Background
Dietary interventions are frequently recommended for the management of irritable bowel syndrome (IBS). Anecdotally, the nutrition counselling received by patients with IBS varies. In clinical practice, the optimal way to counsel these patients in regard to the application of dietary therapy remains unclear.

Aims
The aim of this scoping review is to summarize how the nutrition counselling component of dietary interventions for IBS is currently described in the literature.

Methods
A search of English language publications between January 1, 1994 and September 28th, 2023 was conducted using MEDLINE. Search results were limited to studies in humans and adults over age 18. The following combination of MeSH keywords was used in the search: irritable bowel syndrome AND diet food and nutrition OR counseling OR patient education OR practice guidelines OR nutrition therapy. Publications that involved providing instruction to patients to modify their diets were included. Titles and then abstracts were screened to determine if they potentially met inclusion criteria. Eligible publications were then reviewed in full. Those publications that met the inclusion criteria were included in this scoping review. Information regarding the description of nutrition counseling as a component of the dietary intervention was collected. Analysis is descriptive.

Results
The search strategy identified 683 potential articles. After title review, 251 abstracts were obtained and reviewed producing 31 publications that were reviewed in full. All 31 publications were included in the scoping review. Nutrition counselling was mentioned in all 31 publications, but the description of the components of nutrition counselling varied (Figure 1). No publication included all important components of nutrition counselling in the description of the dietary intervention.

Conclusions
Patients with IBS frequently report symptoms triggered by food. Counselling is an important component to the delivery of dietary interventions in IBS. However, the nutrition counselling components of these interventions for IBS is not well described in the literature in a reliable or standardized way. This limits the ability to provide these therapies to patients in an optimal way. Furthermore, the impact of different nutrition counselling strategies on the efficacy of dietary interventions remains unknown.

Figure 1: Results of Scoping Review.

Funding Agencies
None
A313 DISCONNECTED PANCREATIC DUCT SYNDROME: A RETROSPECTIVE OBSERVATIONAL STUDY REVIEWING AN IMPORTANT BUT OVERLOOKED COMPLICATION OF NECROTIZING PANCREATITIS
Authors: Kim H , Ashrafinia N , Fetz A , Tam L , Akhtar D , Aboalfaraj N , Gan S 
Published: February 2024

Abstract

Background
Disconnected pancreatic duct syndrome (DPDS) is a complication of acute necrotizing pancreatitis (ANP) characterized by a complete disruption of the main pancreatic duct, leading to the loss of continuity between the pancreatic duct and the gastrointestinal tract (figure 1). Due to unopposed leakage of residual pancreatic fluid, patients with DPDS often experience a prolonged disease course with significant morbidity, including complications such as pancreatic fistulas. However, information about the epidemiology, clinical characteristics, and outcomes of DPDS remains scarce.

Aims
To assess clinical outcomes, diagnostic delay, burden of endoscopic and surgical interventions, and complications related to DPDS.

Methods
A retrospective review was conducted for all patients diagnosed with DPDS who underwent either endoscopic ultrasound (EUS) or endoscopic retrograde cholangiopancreatography (ERCP) at our tertiary center between January 2010 and December 2022.

Results
Fifteen patients with DPDS following ANP were identified. The median age of patients at DPDS diagnosis was 56 years (range 33-86 years), with 47% being female. The most common etiology of ANP was biliary (40%), followed by idiopathic (33%) and alcoholic (27%). The median time between ANP diagnosis and DPDS diagnosis was 265 days (range 86-2023 days). Patients underwent a median of 3 (1-9) endoscopic interventions and 1 (0-5) surgical intervention between the initial ANP episode and DPDS diagnosis. 40% of patients experienced intra-abdominal sepsis secondary to infected pancreatic fluid collections. 20% developed pancreatic fistulas (two small bowel fistulas and one cutaneous fistula). 20% developed new-onset diabetes, and 13% had significant worsening of pre-existing diabetes. No mortalities were reported.

Conclusions
There is a significant delay in diagnosing DPDS following ANP, and a substantial burden of endoscopic interventions is required. Frequent complications of DPDS were pancreatic fistulas, recurrent pancreatic fluid collections, and pancreatogenic diabetes. Developing a predictive model for assessing the risk of DPDS development following ANP could aid in earlier diagnosis and potentially reduce morbidity and complications associated with DPDS.

Figure 1. Contrast enhanced CT demonstrating typical finding in DPDS in coronal (A) and axial (B) views. CT demonstrates 3cm area of necrosis involving pancreatic neck/body (asterisk) with viable upstream parenchyma in the remainder of the body and tail (white arrow). Pancreatic head/uncinate parenchyma and main duct appear intact (black arrow). B, the distal pancreatic duct is not dilated. DPDS, disconnected pancreatic duct syndrome.

Funding Agencies
None
A316 ACUTE NECROTIZING PANCREATITIS COMPLICATED BY PULMONARY EMBOLISM: A CASE REPORT AND REVIEW OF LITERATURE
Authors: Shamatutu C , Barclay R 
Published: February 2024

Abstract

Background
Acute pancreatitis (AP) is an inflammatory condition associated with local and systemic complications. Severe pancreatitis can include the development of peripancreatic fluid collections and necrotizing pancreatitis (NP). Vascular complications are one of the causes of morbidity and mortality in patients with AP. Splanchnic thrombosis has been well described in AP and can been seen in up to 24% of patients. Extra splanchnic vessel thrombosis, including pulmonary embolism (PE), is rare.

Aims
Describe a case of acute NP with peripancreatic fluid collection complicated with bilateral PE and review the relevant literature.

Methods
We performed a literature review for AP complicated by PE.

Results
Case: A 60-year-old man presented to the hospital with one day of abdominal pain, nausea and vomiting. His medical history was significant for obesity with a BMI of 40. He was not on any medications. He did not drink alcohol. On exam his heart rate was 58, blood pressure 149/94 mmHG, oxygen saturation was 95% on room air, he was afebrile. His abdomen was tender in the epigastric region. His labs revealed a white blood cell count of 14 x 109/L, lipase ampersand:003E 3,000 U/L, Bilirubin 40 umol/ L, AST 613 U/L, ALT 527 U/L, GGT 394 U/L, ALP 94 U/L, Creatinine 159 mmol/L. CT abdomen/pelvis revealed cholelithiasis without choledocholithiasis or biliary dilation. The pancreas was inflammed with necrosis throughout. There were two peripancreatic fluid collections (60mm x 41mm, and 148mm x 89mm). He was diagnosed with acute necrotizing pancreatitis thought secondary to gallstones. He was fluid resuscitated, started on antibiotics and unfractionated heparin (UFH) for deep vein thrombosis (DVT) prophylaxis. His oral intake remained low. Imaging on day 30 showed a large walled off necrotic collection (25cm x15cm). Vascular strucutres were patent. On day 36 he underwent endoscopic ultrasound guided drainage via lumen-apposing metal stent (LAMS). On day 43 he developed new tachycardia. CT chest revealed bilateral pulmonary emboli (Image 1). Doppler ultrasound revealed bilateral occlusive thrombus in the calf veins. He was treated with therapeutic UFH.
Literature review: We identified 10 case reports describing AP complicated by PE. The age amonst reports was variable (21 to 68). 4 cases reported additional thrombosis outside of the pulmonary vasculature. No cases which described splanchnic venous thrombosis prior. 3 cases reported pancreatic necrosis. The use of DVT prophylaxis was not reported. There is one case of PE 2 days after LAMS insertion in a patient with known thromboembolic disease.

Conclusions
PE is a rare complication of AP. Novel to this case was the recent deployment of a LAMS, the significance of which is unknown to date. Improved documentation of variables in future reports is needed to further characterize this event.

Image 1: Acute Pulmonary Embolism.

Funding Agencies
None
Total en bloc vertebrectomy and immunochemotherapy for chondrosarcoma colliding with intraosseous lymphoma
Authors: Palacios-Diaz Luis , Aguilar Martínez Antonio J , Pérez Rodríguez Francisco J , Penedo Agustín , Sánchez-Márquez Jose M , Fernández-Baillo Nicomedes 
Published: February 2024

Abstract
A 59-year-old woman diagnosed with a Grade I chondrosarcoma in T7 underwent total en bloc vertebrectomy. Analysis of the surgical piece established diagnosis of a Grade 1 chondrosarcoma confined to T7. Surprisingly, an infiltration with diffuse large B-cell lymphoma was found. Systemic disease was ruled out and diagnosis was established as intracompartmental Grade 1 chondrosarcoma colliding with intraosseous extranodal diffuse large B-cell lymphoma. Resection of chondrosarcoma was considered complete and treatment with four cycles of RCHOP was indicated. Two years after surgery, the patient remains at complete metabolic response. To date, this is the first reported case of chondrosarcoma colliding with lymphoma. Although Grade 1 chondrosarcoma is typically managed with local control through complete surgical resection, the mentioned finding of the lymphoma indicated the need for systemic treatment with immunochemotherapy.
Extending the usefulness of the Stryker Growing Prosthesis in pediatric patients
Authors: Sakamoto Akio , Noguchi Takashi , Matsuda Shuichi 
Published: February 2024

Abstract
Osteosarcoma is a highly invasive primary bone tumor that predominantly occurs in childhood and adolescence. The Stryker Growing Prosthesis provides a means of reconstructing large bone defects resulting from bone resection in skeletally immature patients. This device can be expanded as the patient grows. The possible length of extension depends on the length of the prosthesis. Because further expansion was not possible, by turning the adjustable part of the extension back to zero and adding a new permanent extension allow the prosthesis to be further adjusted as growth ensues. Using this method/device only, a separate endoprosthesis was required to be attached onto the extension. Therefore, the applicable cases are limited, because of the fact that extensive resection usually means total femoral replacement is best indicated. However, this method is still useful for reducing the number of revision surgeries in such cases. This reduces costs and increases savings for insurers/countries.
Management of retroperitoneal appendiceal perforation: a case report
Authors: Li Jinliang , Li Feng , Li Jun , Wang Chunlei , Sun Yubao , Bian Xiaowei 
Published: February 2024

Abstract
Retroperitoneal appendiceal perforation presents unique challenges in surgical management due to the complex nature of the retroperitoneal space. We present a case of a 57-year-old male with retroperitoneal appendiceal perforation, characterized by the presence of a large amount of gas in the retroperitoneal space. Emergent laparoscopic surgery was performed to address the retroperitoneal involvement. In retroperitoneal appendiceal perforation, surgical intervention and postoperative drainage are of great significance to prevent septic shock. The interconnectedness of the retroperitoneal space with other body regions is highlighted, underscoring the potential for severe complications. This case emphasizes the need for a tailored approach to managing retroperitoneal appendiceal perforation, preventing potential complications associated with this condition.