Recurrent Episodes Of Pancreatitis Research Articles

Potential Predictors of Severe and Recurrent Pancreatitis in Children: A Single-Center Experience.

Severe pancreatitis often requires intensive care; therefore, early detection is important. This study aimed to evaluate the possible predictors of pancreatitis severity in children. Furthermore, we evaluated the prevalence of pancreatitis recurrence and related factors. We retrospectively analyzed the medical records of patients aged younger than 18 years who were diagnosed with acute pancreatitis between January 2017 and June 2022. Acute pancreatitis was diagnosed and classified based on the revised Atlanta criteria. A total of 64 patients were enrolled, and severe pancreatitis was observed in 10 (15.6%) patients. Patients with severe pancreatitis were younger and had higher C-reactive protein levels than those with mild pancreatitis. The areas under the receiver operating characteristic curves of C-reactive protein levels at admission and 48 hours after diagnosis were 0.612 (95% confidence interval, 0.379-0.844) and 0.873 (95% confidence interval, 0.537-0.983), respectively. Fourteen patients (21.9%) experienced further episodes of recurrent pancreatitis. Patients with recurrent pancreatitis were older (13.7 vs 11.4 years, P = 0.022) and prominently boys (85.7 vs 52%, P = 0.022) compared with those without recurrence. Obesity was observed in 4 (0.6%) patients, which was confirmed in all recurrence groups and was statistically significant. This study demonstrated the feasibility of C-reactive protein in the early risk assessment of patients with acute pancreatitis and highlighted the potential risk of recurrence in patients with obesity.

Volanesorsen and triglyceride levels in familial chylomicronemia syndrome: Long-term efficacy and safety data from patients in an open-label extension trial

Familial chylomicronemia syndrome (FCS) is a rare, autosomal recessive genetic disorder characterized by a marked increase in plasma triglyceride (TG) levels and recurrent episodes of pancreatitis. The response to conventional TG-lowering therapies is suboptimal. Volanesorsen, an antisense oligonucleotide that targets hepatic apoC-III mRNA, has been shown to significantly reduce TGs in patients with FCS. To further evaluate the safety and efficacy of extended treatment with volanesorsen in patients with FCS. This phase 3 open-label extension study evaluated the efficacy and safety of extended treatment with volanesorsen in three groups of patients with FCS: Those who had previously received volanesorsen or placebo in the APPROACH and COMPASS studies, and treatment-naive patients not participating in either study. Key endpoints included change in fasting TG and other lipid measurements, and safety over 52 weeks. Volanesorsen treatment resulted in sustained reductions in plasma TG levels in previously treated patients from the APPROACH and COMPASS studies. Volanesorsen-treated patients from the three populations studied had mean decreases in fasting plasma TGs from index study baseline to months 3, 6, 12 and 24 as follows: decreases of 48%, 55%, 50%, and 50%, respectively (APPROACH); decreases of 65%, 43%, 42%, and 66%, respectively (COMPASS); and decreases of 60%, 51%, 47%, and 46%, respectively (treatment-naive). Common adverse events were injection site reactions and platelet count decrease, consistent with previous studies. Extended open-label treatment with volanesorsen in patients with FCS resulted in sustained reductions of plasma TG levels and safety consistent with the index studies.

Acute pancreatitis secondary to hypercalcemia in a patient with primary hyperparathyroidism: An uncommon association

An uncommon cause of acute pancreatitis, primary hyperparathyroidism accounts for less than 1% of cases. A 41-year-old male with acute pancreatitis and hypercalcemia is described in this case. Primary hyperparathyroidism was discovered during the work-up for hypercalcemia. During the first 24 hours after his hospitalization, the patient was monitored in the intensive care unit, and after a positive outcome, he was discharged. Pancreatitis is a rare presentation of hyperparathyroidism. The first documented case of this association was by Erdheim in 1903 on a post-mortem study (2). Hyperparathyroidism is often only discovered after two or three episodes of recurrent pancreatitis (5), thankfully, in this case, the patient has been diagnosed from its first episode and eventually treated to prevent any other ones. hypercalcaemia leads to increase calcium in the pancreatic responsible for aggression of the pancreatic parenchyma and ducts, Other authors suggest that the pancreatic secretion in patients with hypercalcaemia is lower than normal, but the enzyme activity remains normal, resulting in the formation of protein plugs in the pancreatic ducts leading to their obstruction and self-digestion. Hypercalcemia can cause acute pancreatitis. This report describes rare case of a patient with acute pancreatitis caused by hyperparathyroidism.

LPL Gene Mutation in Type 1 Familial Triglyceridemia Presenting as Recurrent Pancreatitis and Complicated by COVID19

AbstractA 7-year-old girl with recurrent episodes of pancreatitis with risk factor of poorly controlled hyperglyceridemia presented with an acute episode of pancreatitis. She was managed conservatively and underwent whole exome sequencing which showed a likely pathogenic LPL gene mutation. Incidentally, she was diagnosed with COVID-19 on screening, which we hypothesize to have triggered the recent episode. On further examination, she was found to have bilateral cataracts. Her hypercholesterolemia was effectively managed with dietary therapy, high dose omega 3, and gemfibrozil. Our case report sensitizes the clinician to use a modern diagnostic tool such as whole exome sequencing in children with recurrent pancreatitis where hypertriglyceridemia is a known risk factor. This child is the first case of LPL mutation reported in India.

S2808 Diagnostic Dilemma: Utilizing Endoscopic Ultrasound (EUS) Guided Biopsy to Assist With the Diagnosis of the Duodenal Duplication Cyst

Introduction: Duodenal duplication cyst (DDC) is a rare congenital anomaly that often presents in the neonatal period with presentations later in life less common. Duplication cysts can develop anywhere from the mouth to the anus with only 4-12% are located in the duodenum. DDC is an uncommon cause of acute pancreatitis but the diagnosis is difficult to make in adults because the symptoms are non-specific. We present a case of diagnostic dilemma of a 53 years old female patient with recurrent acute pancreatitis (RAP), attributed to DDC. Case Description/Methods: Her first index episode of pancreatitis was 12 years prior to her presentation. Patient was found to have a large cyst in the 2nd portion of the duodenum that appeared to be separate from the biliary tree and pancreas on EGD (Figure a), EUS (Figure b) and MRI (Figure c), abutting the major papilla. The initial EUS showed a 20 mm x 13.4 mm anechoic cyst with layering and debris seen in the cavity. Aspiration of the cyst was performing, returning with elevated CEA at 817 ng/mL. No features of chronic pancreatitis were seen. Given elevated CEA, there was a concern for mucinous cyst, prompting a repeat EUS. EUS guided biopsy of the internal cyst wall was performed and histology was consistent with small bowel suggestive of a duplication cyst. Discussion: Our patient presented with recurrent episodes of pancreatitis that could be explained by the intermittent occlusion of the pancreatic duct by debris and sludge found within the cyst. The elevated CEA and inconclusive radiographic results initially suggested possible mucinous cyst, presenting a diagnostic dilemma. Therefore, EUS guided biopsies were essential to confirm the diagnosis and allowing for further endoscopic management. The presence of a smooth muscle coat is absolutely essential for the diagnosis of a duplication cyst which was the case for our patient. DDC are covered both inside and outside by duodenal mucosa containing a distinct layer of smooth muscle. Given its malignancy potential, resection of these cysts is indicated. Endoscopic resection is minimally invasive and has been proposed as an alternative to surgery in selected patients especially given the close proximity of major papilla. Resection techniques using standard polypectomy, or large incision of the roof of the duplication cyst has been discussed in the literature. In our case, endoloop was applied around the base of the cyst, leading to strangulation and necrosis for obliteration of the cyst.Figure 1.: (a) shows a large cyst in the 2nd portion of the duodenum that appeared to be separate from the biliary tree and pancreas on EGD abutting the major papilla, (b) shows the cysts on EUS and (c) shows the cyst on MRI.

43 The Value of Endoscopic Ultrasound (EUS) in the Investigative Pathway of Acute Idiopathic Pancreatitis (AIP)

Abstract Introduction The British Society of Gastroenterology (BSG) guidelines on the management of acute idiopathic pancreatitis (AIP) state the incidence of idiopathic cases to be no more than 30%. However, before a firm diagnosis of AIP is made, Endoscopic Ultrasound (EUS) may be used to determine occult causes. This approach may help prevent recurrent attacks which may evolve into chronic pancreatitis. Method Retrospective analysis over a one-year period of cases of AIP in a tertiary referral centre was performed to see the incidence of AIP and the role of EUS. Patients with an identifiable cause for pancreatitis were excluded, leaving only those who had received a diagnosis of AIP and the diagnostic value of EUS was examined. Results Of the 101 patients diagnosed with AIP, 19% (n=19) underwent an EUS successfully. 79% (n=15) had no underlying cause of pancreatitis identified on EUS. In the remaining 21% of cases (n=4), microlithiasis, ductal stones, and pus requiring drainage were common findings. Of these patents, only 1 was referred for surgery. In the patient group with negative findings on EUS, 4% were unaffected, 2% were referred for surgery, 2% died and 7% had recurrent episodes of pancreatitis. Conclusions The results of this study show that EUS is a valuable modality in patients with suspected AIP, with a positive diagnostic rate of 21%. Therefore, we propose EUS needs to be included in the investigative pathway of all suspected AIP. Although EUS is a relatively scarce resource, further research is required to establish guidelines for the investigation of suspected AIP.

P-EUS02 The value of Endoscopic Ultrasound (EUS) in the investigative pathway of Acute Idiopathic Pancreatitis (AIP)

Abstract Background The British Society of Gastroenterology (BSG) guidelines on the management of acute idiopathic pancreatitis (AIP) state the incidence of idiopathic cases to be no more than 30%. However, before a firm diagnosis of AIP is made, Endoscopic Ultrasound (EUS) may be used to determine occult causes. This approach may help prevent recurrent attacks which may evolve into chronic pancreatitis. Methods Retrospective analysis over a one-year period of cases of AIP in a tertiary referral center was performed to see the incidence of AIP and the role of EUS. Patients with an identifiable cause for pancreatitis were excluded, leaving only those who had received a diagnosis of AIP and the diagnostic value of EUS was examined. Results Of the 101 patients diagnosed with AIP, 19% (n = 19) underwent an EUS succesfully. 79% (n = 15) had no underlying cause of pancreatitis identified on EUS. In the remaining 21% of cases (n = 4), microlithiasis, ductal stones, and pus requiring drainage were common findings. Of these patents, only 1 was referred for surgery. In the patient group with negative findings on EUS, 4% were unaffected, 2% were refered for surgery, 2% died and 7% had recurrent episodes of pancreatitis. Conclusions The results of this study show that EUS is a valuable modality in patients with suspected AIP, with a positive diagnostic rate of 21%. Therefore, we propose EUS needs to be included in the investigative pathway of all suspected AIP. Although EUS is a relatively scarce resource, further research is required to establish guidelines for the investigation of suspected AIP.

51-Year-Old Man With Ascites and Abdominal Pain

51-Year-Old Man With Ascites and Abdominal Pain

Sleeve Gastrectomy Attenuates the Severity of Cerulein-Induced Acute Pancreatitis in Obese Rats.

Obesity is one of the most important risk factors for acute pancreatitis. Based on the effect of sleeve gastrectomy (SG) on improving body weight and blood lipids, we investigated whether SG is beneficial in improving pancreatitis in obese rats. Two studies were used to evaluate the effect of SG on the first onset of pancreatitis and acute episodes of recurrent pancreatitis in obese rats. A high-fat diet (HFD) for 8 weeks resulted in obesity in rats. Study 1: Obese rats were treated with SG and sham surgery. Pancreatitis was induced by intraperitoneal injection of cerulein at 6 weeks after surgery. The severity of pancreatitis was assessed by histological examination, cytokines, and infiltration of inflammatory cells. Study 2 performed the same procedure as in study 1, except that rats were intraperitoneally injected with a small dose of cerulein three times a week for 6 weeks before surgery to induce recurrent pancreatitis. The body weight, food intake, and blood lipids of SG rats in study 1 and study 2 were significantly lower than those of sham rats during the 6 weeks after surgery. Compared with sham rats, SG rats in both studies had fewer inflammatory cytokines, inflammatory cell infiltration, and pathological injury in the pancreas after cerulein-induced acute pancreatitis. SG reduces the severity of the first onset of pancreatitis and the seriousness of acute episodes of recurrent pancreatitis. The improvement of lipid metabolism and body weight by SG may play an important role in this effect.

Multiple Concomitant Episodes of Diabetic Ketoacidosis and Acute Pancreatitis in a Pediatric Patient With Type 1 Diabetes

Introduction: Abdominal pain is a common presenting symptom in diabetic ketoacidosis (DKA). Correction of the acidosis usually leads to resolution of the abdominal pain. In some instances, the pain may persist due to additional etiologies presenting alongside DKA. Though uncommon, there has been shown to be an association between DKA and acute pancreatitis (AP). In these rare cases, AP was secondary to the hypertriglyceridemia (HTG) state induced by DKA. We report a 13-year-old female known with type 1 diabetes (T1D) who presented with multiple concomitant episodes of DKA and AP and normal triglyceride levels. Case Presentation: The patient is a 13-year-old female with T1D who presented with two days of hyperglycemia, nausea, and diffuse abdominal pain. Initial laboratory evaluation was remarkable for point-of-care glucose of >500 mg/dL (60-99), venous pH of 7.006 (7.330-7.430), bicarbonate of < 5 mmol/L (20-28), beta-hydroxybutyrate of 5.6 mmol/L (0.0-0.8); consistent with severe DKA. She received normal saline bolus fluids and then started on the DKA protocol with improvement of acidosis, though with the persistence of abdominal pain. Due to concern for other causes of her abdominal pain, additional workup was done, notable for elevated lipase of 624 U/L (10-52), amylase of 434 U/L (25-100), and triglyceride of 121 mg/dL (30-149). An abdominal ultrasound showed findings consistent with AP, lipase levels peaked at 1753 U/L before down-trending to 959 U/L, and amylase decreased to 389 U/L. After several days abdominal pain resolved, and the patient was discharged home. The patient was readmitted six weeks and again one year later for laboratory and symptoms, including abdominal pain consistent with DKA. Both lipase and amylase were elevated during both admissions with normal triglyceride levels. Magnetic resonance cholangiopancreatography was significant for findings compatible with acute pancreatitis with no evidence of cholelithiasis or choledocholithiasis. The patient underwent genetic testing, including normal PRSS1, SPINK1, CFTR, CPA1, and CTRC. A variant of unknown clinical significance was identified in the CTRC gene (c.550G>A), which was not thought to be the cause of her recurrent pancreatitis. Interestingly, since her hemoglobin A1c has been in a better range for the past year, she did not have any recurrent episodes of pancreatitis. Conclusion: The insulin-deficient state associated with DKA can lead to moderate to severe HTG, which in turn can cause AP. Even though abdominal pain is a common symptom in patients presenting in DKA, one should think about other causes when the abdominal discomfort is out of proportion or not improving as acidosis resolves. Our patient had recurrent pancreatitis for unknown etiology; however, she has not had any pancreatitis episodes in the last year since her diabetes has been under better control.

Derivation and validation of the ED-SAS score for very early prediction of mortality and morbidity with acute pancreatitis: a retrospective observational study

BackgroundExisting scoring systems to predict mortality in acute pancreatitis may not be directly applicable to the emergency department (ED). The objective of this study was to derive and validate the ED-SAS, a simple scoring score using variables readily available in the ED to predict mortality in patients with acute pancreatitis.MethodsThis retrospective observational study was performed based on patient data collected from electronic health records across 2 independent health systems; 1 was used for the derivation cohort and the other for the validation cohort. Adult patients who were eligible presented to the ED, required hospital admission, and had a confirmed diagnosis of acute pancreatitis. Patients with chronic or recurrent episodes of pancreatitis were excluded. The primary outcome was 30-day mortality. Analyses tested and derived candidate variables to establish a prediction score, which was subsequently applied to the validation cohort to assess odds ratios for the primary and secondary outcomes.ResultsThe derivation cohort included 599 patients, and the validation cohort 2011 patients. Thirty-day mortality was 4.2 and 3.9%, respectively. From the derivation cohort, 3 variables were established for use in the predictive scoring score: ≥2 systemic inflammatory response syndrome (SIRS) criteria, age > 60 years, and SpO2 < 96%. Summing the presence or absence of each variable yielded an ED-SAS score ranging from 0 to 3. In the validation cohort, the odds of 30-day mortality increased with each subsequent ED-SAS point: 4.4 (95% CI 1.8–10.8) for 1 point, 12.0 (95% CI 4.9–29.4) for 2 points, and 41.7 (95% CI 15.8–110.1) for 3 points (c-statistic = 0.77).ConclusionAn ED-SAS score that incorporates SpO2, age, and SIRS measurements, all of which are available in the ED, provides a rapid method for predicting 30-day mortality in acute pancreatitis.

Long-term outcome of patients with biliary pancreatitis not undergoing cholecystectomy. A retrospective study.

most acute pancreatitis cases are of biliary origin and cholecystectomy is recommended to prevent recurrence. However, some patients will never be referred for surgery. In this study, the long-term follow-up of this group of patients was reviewed. all new cases of biliary pancreatitis from January 2015 to December 2017 that did not undergo cholecystectomy were analyzed. Epidemiologic data and Charlson's comorbidity index (CCI) were recorded. Recurrent episodes of pancreatitis or biliary events and mortality during the follow-up period were recorded. a total of 104 patients were included in the study (30.4 % of all biliary pancreatitis cases) and the median age was 82 years (range, 27-96). Average CCI was 5 (range, 0-18) and the median follow-up period was 37 months (range, 1-70). A total of 41 patients (39.4 %) had gallstone-related complications. Twenty-three patients (22,1 %) had recurrent pancreatitis and 34 (32,7 %) developed biliary events. Twenty-five patients died during follow-up (24 %) but only in 6 (5,8 %) was death due to gallstone-related complications. Non-related mortality was 15.5 % in patients who refused surgery and 25 % in multiple-comorbidity patients. patients who did not undergo cholecystectomy were at high risk for biliary events and pancreatitis recurrence. Conservative treatment and surgical abstention should be individualized and reserved for patients with multiple comorbidities with a short life expectancy.

Long-term outcome of patients with biliary pancreatitis not undergoing cholecystectomy: a retrospective study

Background and objective Most acute pancreatitis are of biliary origin and undergoing a cholecystectomy is recommended to prevent recurrence. However, some patients will never be referred to surgery. We reviewed the long-term follow-up of these group of patients Methods All cases of biliary pancreatitis presented from January 2015 to December 2017 that did not receive a cholecystectomy were analyzed. Epidemiologic data and Charlson comorbidity Index (CCI) were recorded. Recurrent episodes of pancreatitis or biliary events and mortality during the follow-up period was recorded. Results A total of 104 patients were included in the study (30.4% of all biliary pancreatitis). Median age was 82 years (range 27-96). Average CCI was 5 (range 0-18). The median follow-up period was 37 months (range 1-70). A total of 41 patients (39.4%) had gallstone-related complications. Twenty-three patients (22,1%) had recurrent pancreatitis and 34 (32,7%) developed biliary events. Decease occurred in 25 patients during follow-up (24%) but only 6 (5,8%) were due to gallstone-related complications. Non-related mortality was 15.5% in patients who refused surgery and 25% if high comorbidity patients. Conclusion Patients that are not cholecystectomized are at high risk for biliary event and pancreatitis recurrence. Conservative treatment and surgical abstention should be individualized and reserved to high comorbid patients with short life expectancy.

Management of a pregnant patient with chylomicronemia from a novel mutation in GPIHBP1: a case report

BackgroundFamilial chylomicronemia syndrome (FCS) is a rare autosomal recessive lipid disorder often associated with recurrent episodes of pancreatitis. It is documented in most cases with FCS due to the mutations of key proteins in lipolysis, including LPL, APOC2, APOA5, LMF1 and GPIHBP1.Case presentationWe report the successful management of a 35-year-old pregnant woman carrying a novel homozygous frameshift mutation c.48_49insGCGG (p.P17A fs*22) in the GPIHBP1 gene with previous severe episodes of acute pancreatitis triggered by pregnancy, resulting in adverse obstetrical outcomes. With careful monitoring, the patient underwent an uneventful pregnancy and delivered a baby with no anomalies.ConclusionsThe case report contributes to the understanding of GPIHBP1-deficient familial chylomicronemia syndrome (FCS) and highlights gestational management of FCS patient.

Pancreatitis in pre-adolescent children: a 10\u2009year experience in the pediatric emergency department

BackgroundThe diagnosis of pediatric pancreatitis has been increasing over the last 15 years but the etiology of this is uncertain. The population of pre-adolescent patients with pancreatitis in the emergency department has not been specifically described. Our objective was to determine the characteristics of these patients to illuminate this population and disease in order to better identify them and avoid a delay in diagnosis and treatment.MethodsThis was a retrospective descriptive study of consecutive pediatric patients under the age of 13 years between 2006 and 2016 who presented to our pediatric emergency department with a diagnosis of atraumatic pancreatitis. Patient characteristics, lab and imaging results, identified etiology of pancreatitis, and recurrence rates were recorded and evaluated.ResultsThere were 139 visits, of which 85 were for a first episode of acute pancreatitis, and 54 were patients with an episode of recurrent pancreatitis. The median age for all visits was 8 years (IQ range 5–11). Of the acute cases, 26% had uncertain or undetermined etiologies of which half were thought to likely be viral related; 20% had systemic inflammatory or autoimmune diseases; 19% were associated with medications, with the most common being valproic acid; 16% were cholelithiasis-related; and 15% were found to have a genetic, congenital or structural etiology. No patients had elevated triglycerides. Those with cholelithiasis and genetic or structural defects were found to have a higher recurrence rate than those with other etiologies. There were only four patients diagnosed with chronic pancreatitis.ConclusionsThe etiology of pancreatitis in pre-adolescent children has a different distribution than in adolescents and adults, with gallstone disease less frequent and concurrent contributing illness more common. Patients on pancreatitis-causing medications or with known genetic risk or structural pancreatic problems should be tested for pancreatitis if presenting with concerning symptoms. Hypertriglyceridemia and chronic pancreatitis with evidence of pancreatic exocrine insufficiency is uncommon in this population.

2792 A Retrospective Study of Probiotic Use in Patients With Chronic Pancreatitis

INTRODUCTION: Chronic pancreatitis is a debilitating condition that develops following recurrent episodes of pancreatitis. It increases morbidity, decreases quality of life and leads to high utilization of medical resources. There is no definitive medical treatment. Studies have shown that gut microbiota may play a role in pancreatitis as bacterial and endotoxin translocation can contribute to severity. Bacteria such as lactobacillus, and bifidobacterium have been noted to be lower in chronic pancreatitis while enterobacteriaceae species were identified at higher levels in those. Probiotics can increase concentrations of bifidobacteria, lactobacilli and streptococci, preventing overgrowth of non-commensals. We are focused on researching new strategies to prevent disease progression and decrease length of hospital stay. METHODS: Retrospective chart review of a database of 86 chronic pancreatitis patients and probiotic use. All patients were cohorted based on severity of disease as rated on EUS using a scale of 1-5 with grades 1-3 grouped as “non-severe” and 4-5 as “severe.” We chose a null of 0.05 with a 95% confidence interval for statistical significance. Relative risk assessments were conducted on the rate of severe chronic pancreatitis in users of probiotics versus negative control. RESULTS: Our initial results revealed that 34% of patients had used probiotics since diagnosis of chronic pancreatitis on EUS. The rate of severe pancreatitis for the cohort of probiotic users was 0.241, and that for negative control was 0.298. A relative risk assessment for our population found a relative risk of 0.81 (95% CI, [0.379-1.727]; P = 0.2922) for the rate of severe chronic pancreatitis given exposure to probiotics. The result suggested a potential effect from probiotics on decreasing the risk of having severe chronic pancreatitis but was not statistically significant. CONCLUSION: There was no significant association between probiotic use and severity of chronic pancreatitis. We cannot exclude that probiotics may have a protective or therapeutic immunomodulatory effect on the microbiome of patients with chronic pancreatitis. This study is limited to a chart review in order to determine the extent of probiotic use in patients and cannot verify compliance with probiotics. We cannot report a correlation between probiotic use and severity of chronic pancreatitis.

Volanesorsen and Triglyceride Levels in Familial Chylomicronemia Syndrome

BackgroundFamilial chylomicronemia syndrome is a rare genetic disorder that is caused by loss of lipoprotein lipase activity and characterized by chylomicronemia and recurrent episodes of pancreatitis. There are no effective therapies. In an open-label study of three patients with this syndrome, antisense-mediated inhibition of hepatic APOC3 mRNA with volanesorsen led to decreased plasma apolipoprotein C-III and triglyceride levels.MethodsWe conducted a phase 3, double-blind, randomized 52-week trial to evaluate the safety and effectiveness of volanesorsen in 66 patients with familial chylomicronemia syndrome. Patients were randomly assigned, in a 1:1 ratio, to receive volanesorsen or placebo. The primary end point was the percentage change in fasting triglyceride levels from baseline to 3 months.ResultsPatients receiving volanesorsen had a decrease in mean plasma apolipoprotein C-III levels from baseline of 25.7 mg per deciliter, corresponding to an 84% decrease at 3 months, whereas patients receiving placebo had an increase in mean plasma apolipoprotein C-III levels from baseline of 1.9 mg per deciliter, corresponding to a 6.1% increase (P<0.001). Patients receiving volanesorsen had a 77% decrease in mean triglyceride levels, corresponding to a mean decrease of 1712 mg per deciliter (19.3 mmol per liter) (95% confidence interval [CI], 1330 to 2094 mg per deciliter [15.0 to 23.6 mmol per liter]), whereas patients receiving placebo had an 18% increase in mean triglyceride levels, corresponding to an increase of 92.0 mg per deciliter (1.0 mmol per liter) (95% CI, −301.0 to 486 mg per deciliter [–3.4 to 5.5 mmol per liter]) (P<0.001). At 3 months, 77% of the patients in the volanesorsen group, as compared with 10% of patients in the placebo group, had triglyceride levels of less than 750 mg per deciliter (8.5 mmol per liter). A total of 20 of 33 patients who received volanesorsen had injection-site reactions, whereas none of the patients who received placebo had such reactions. No patients in the placebo group had platelet counts below 100,000 per microliter, whereas 15 of 33 patients in the volanesorsen group had such levels, including 2 who had levels below 25,000 per microliter. No patient had platelet counts below 50,000 per microliter after enhanced platelet-monitoring began.ConclusionsVolanesorsen lowered triglyceride levels to less than 750 mg per deciliter in 77% of patients with familial chylomicronemia syndrome. Thrombocytopenia and injection-site reactions were common adverse events. (Funded by Ionis Pharmaceuticals and Akcea Therapeutics; APPROACH Clinical Trials.gov number, NCT02211209.)

Eluxadoline-induced Recurrent Pancreatitis in a Young Female without a Gallbladder: A Case Report and Literature Review

Eluxadoline is a mixed opioid receptor agonist and antagonist approved for the treatment of diarrhea-predominant irritable bowel syndrome (IBS). It is believed to decrease visceral hypersensitivity without completely inhibiting intestinal motility. Pooled safety data from two phase three randomized trials have reported few cases of pancreatitis especially in patients with sphincter of Oddi (SO) dysfunction and cholecystectomy patients. We present a rare case of eluxadoline-induced recurrent pancreatitis in a 31-year-old female without a gallbladder. Her medical history was significant for irritable bowel syndrome with diarrhea (IBS-D), cholecystectomy, and depression. She was started on 75 mg of eluxadoline (the recommended dose for IBS-D patients without a gallbladder) three weeks prior to the first episode of pancreatitis. She had a recurrent episode of pancreatitis after few weeks and her symptoms and lipase levels improved significantly two days after stopping eluxadoline. The exact mechanism of eluxadoline to cause pancreatitis is unknown but it is believed to increase SO contractions. The absence of gallbladder prevents cholecystokinin mediated relaxation of the SO thus contributing more to spasms with eluxadoline. Few cases of severe pancreatitis and death have been reported even with the reduced dose of eluxadoline recommended for cholecystectomy patients. This case highlights the importance of considering drug-induced pancreatitis and avoidance of eluxadoline even in reduced doses in patients without a gallbladder.

Hereditary chronic pancreatitis in a patient with type 1 diabetes mellitus

In this case report, we present a young patient with type 1 diabetes who had hereditary chronic pancreatitis. We evaluated him for the cause of pancreatitis, but it was inconclusive and finally the genetic testing was done for him, which revealed heterozygous missense mutation in exon 3 of the PRSS1 gene (protease serine 1 gene) on chromosome 7. Hence, we were able to make the diagnosis of hereditary chronic pancreatitis. Chronic pancreatitis secondary to any cause can lead to permanent diabetes, which is typically difficult to control. However, in this case, the episodes of recurrent pancreatitis were present after the onset of type 1 diabetes as compared to the usual presentation of diabetes after the advancement of chronic pancreatitis.

Diffuse Large B-Cell Lymphoma Presenting as Acute Pancreatitis and Mediastinal Mass

SESSION TITLE: Disorders of the Mediastinum 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Acute pancreatitis is an extremely rare presentation of diffuse large B-cell lymphoma (DLBCL). We present the case of a 19 year old female with recurrent pancreatitis secondary to DLBCL. CASE PRESENTATION: A 19 year old female with a prior history of cholecystectomy and no alcohol use presented to the emergency department with epigastric pain and vomiting. She had recently been admitted twice for acute pancreatitis with no etiology identified. Lipase was 3,334 U/L (normal 23-300 U/L). Ultrasound showed no biliary duct dilation. Magnetic resonance cholangiopancreatography five days prior had shown pancreatic edema but no common bile duct obstruction. A CT had shown only pancreatic edema. Extensive workup for the etiology of her pancreatitis was done: calcium, triglycerides, hepatitis panel, EBV, CMV and HIV serology, alpha-1 antitrypsin, ANA, and IgG4 levels were normal. On review of systems, she had a one-month history of dry cough and dyspnea on exertion. Chest X-ray revealed a right upper lobe (RUL) infiltrate. CT chest/abdomen/pelvis was done and showed a 13 x 3.4 cm mediastinal mass (Fig. 1) causing compression of the superior vena cava and RUL bronchus, right hilar adenopathy, and pericardial and bilateral pleural effusions. The pancreas was diffusely swollen with peripancreatic induration and retroperitoneal adenopathy. Pathology from CT-guided biopsy of the mediastinal mass revealed DLBCL. She was treated with 6 cycles of R-CHOP and has been in remission for five years with no further episodes of pancreatitis. DISCUSSION: Primary pancreatic lymphoma often presents as pancreatitis, but it is very rare for DLBCL to present as acute pancreatitis from secondary involvement of the pancreas. Our case is even more unusual as there was no discrete pancreatic mass on imaging. To our knowledge, only two other cases of DLBCL involving the pancreas have been reported in which there was no discrete mass1,2. Notably, in all cases reported in the literature, imaging has demonstrated either lymphadenopathy or masses involving other organs in addition to the pancreas. Therefore, these findings should raise suspicion for malignant etiologies in the patient with otherwise routine-appearing pancreatitis. Our case demonstrates the value of repeat imaging upon recurrent episodes of pancreatitis when no etiology has been found, as her lymphadenopathy and masses did not appear on initial imaging. CONCLUSIONS: Lymphoma must be considered in the differential diagnosis of acute pancreatitis after the usual causes are excluded. Repeat imaging is valuable in cases of recurrent pancreatitis of unknown etiology since new findings may develop rapidly and lead to the diagnosis. Reference #1: To C, et. al. Masquerade without a mass: an unusal cause of severe acute pancreatitis. J Gastrointest Oncol 2013;4(1):114-117. Reference #2: Saif M, et al. Secondary pancreatic involvement by a diffuse large B-cell lymphoma presenting as acute pancreatitis. World J Gastroenterol 2007;13(36):4909-4911. DISCLOSURE: The following authors have nothing to disclose: Zachary Wolfe, Lydia Winnicka, Bradley Lash No Product/Research Disclosure Information