Pancreatic Enzyme Supplementation Research Articles

Dietary intervention for a growing Australian Kelpie with intestinal lipid malabsorption

AbstractA 12‐week‐old male Australian Kelpie was referred for nutritional consultation after being diagnosed with autosomal recessively inherited intestinal lipid malabsorption (ILM). At the timepoint of referral, the dog's bodyweight was below the ideal growth curve, the faecal quality was poor and the patient received a pancreatic enzyme supplement. The patient's diet at referral was based on a commercial dry food for all age groups and contained approximately 20% fat in dry matter (DM). We formulated a highly digestible homemade diet with a low‐fat content (10% DM) while meeting the recommendations for linoleic acid, α‐linolenic acid, eicosapentaenoic acid and docosahexaenoic acid, which supported the nutritional requirements of a growing dog with impaired fat absorption. The diet was adapted according to the different growth phases.To our knowledge, this is the first case report highlighting the nutritional management of an Australian Kelpie with ILM. After diet adaptation, the patient reached his growth potential.

Investigation of lipid digestion by fungal and porcine lipase supplements for severe pancreatic insufficiency using the in vitro dynamic TIM model

Exocrine pancreaticinsufficiency (EPI) is caused by insufficient secretion or limited activity ofpancreatic enzymes, especially lipase. Chronic pancreatitis (CP) is the mostcommon EPI disease. CP patients suffer from gastrointestinal symptoms such asdiarrhea, abdominal pain, and decreased uptake of nutrients as a consequence ofinadequate food digestion. To improve their nutritional status, pancreaticenzyme supplements can be orally administered, known as pancreatic enzymereplacement therapy (PERT). PERT is commonly based on oral porcinepreparations, despite their pH sensitivity which results in a limited timeframefor optimal activity. Instead, fungal enzymes could be used to prolong enzymeactivity duration, since they are active over a wide pH range. The performanceof fungal enzymes was compared to porcine enzymes by measuring total fattyacids under severe pancreatic insufficiency conditions in the tiny-TIMsgdynamic in vitro gastrointestinal model. Administration of 14.000 fungallipase units (Nortase) led to similar lipid digestion compared to 20.000porcine lipase units (Kreon and Pangrol). Additionally, opening the Nortasecapsules had no negative effect on fungal lipase activity. This workdemonstrates that fungal lipases, administered as capsules or powder, can beused as an alternative treatment for EPI patients.

ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis

Nutritional status is paramount in Cystic Fibrosis (CF) and is directly correlated with morbidity and mortality. The first ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with CF were published in 2016. An update to these guidelines is presented. The study was developed by an international multidisciplinary working group in accordance with officially accepted standards. Literature since 2016 was reviewed, PICO questions were discussed and the GRADE system was utilized. Statements were discussed and submitted for on-line voting by the Working Group and by all ESPEN members. The Working Group updated the nutritional guidelines including assessment and management at all ages. Supplementation of vitamins and pancreatic enzymes remains largely the same. There are expanded chapters on pregnancy, CF-related liver disease, and CF-related diabetes, bone disease, nutritional and mineral supplements, and probiotics. There are new chapters on nutrition with highly effective modulator therapies and nutrition after organ transplantation.

Exocrine pancreatic insufficiency in dogs and cats.

Exocrine pancreatic insufficiency (EPI) is a malabsorptive syndrome caused by insufficient secretion of digestive enzymes from pancreatic acini. The most common causes of EPI in dogs and cats are pancreatic acinar atrophy and chronic pancreatitis. EPI is diagnosed by measurement of species-specific immunoassays for serum trypsin-like immunoreactivity, the concentration of which directly reflects the mass of functioning pancreatic acinar tissue. EPI is treated by pancreatic enzyme replacement therapy, nutritional management (low-residue diets with moderate fat content), and supplementation of cobalamin. Some dogs and cats have persistent clinical signs despite these treatments. Growing evidence suggests that these clinical signs may be due to enteric microbiota dysbiosis or the presence of concurrent diseases such as chronic enteropathies. Management of these abnormalities may improve outcome in dogs and cats with EPI. The long-term prognosis for dogs and cats with EPI is generally good if high-quality medical therapy is provided. Future studies are needed to further understand the causes of persistent dysbiosis in animals with EPI following initiation of pancreatic enzyme replacement therapy and assess the efficacy of treatments to ameliorate these abnormalities.

553 Effect of low- versus high-dose pancreatic enzyme supplement on BMI in people with cystic fibrosis

553 Effect of low- versus high-dose pancreatic enzyme supplement on BMI in people with cystic fibrosis

A-340 CFTR Modulator Therapy Increases Serum/Plasma Concentrations of Vitamin a in People with Cystic Fibrosis

Abstract Introduction Pancreatic insufficiency (PI) is common in people with Cystic Fibrosis (PwCF), contributing to malabsorption of fat and fat-soluble vitamins. Thus, PI PwCF are prescribed pancreatic enzyme replacement therapy (PERT) and supplementation with fat-soluble vitamins. The circulating vitamin levels are monitored to guide dietary therapy. Currently, there are multiple cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies available that have shown significant improvements in pulmonary function, anthropometric parameters, and life expectancy in PwCF, but the effects on serum/plasma fat-soluble vitamin levels remain unknown. The goal of this study was to determine how modulator therapy affects serum/plasma vitamin A and E levels in individuals with CF. Methods The analysis included 1139 serum/plasma samples from 157 children and adolescents with CF: 74 males, 83 females, between the ages of 2–17 years. Most individuals were homozygous or compound heterozygous for the F508del variant (n = 81 and n = 72, respectively). 141 individuals (90%) were diagnosed with PI (fecal elastase <200 μg/g stool). 76 individuals (48%) received different CFTR modulator therapies for at least 1 month. Vitamin A (retinol and retinyl palmitate) and Vitamin E (α-tocopherol and γ-tocopherol) were measured using a high-performance liquid chromatography assay. Data analyses were performed in Prism software (La Jolla, CA), and statistical significance was defined as P < 0.05. The study was approved by the IRB. Results Overall, vitamin deficiency was rarely observed in PwCF prescribed PERT and vitamin supplements; <3% of them had low concentrations of Vitamin A and/or E. The proportion of individuals with Vitamin A and/or E above the reference range was larger; 10.9% and 30.8% for Vitamin A and E, respectively, most likely reflecting supplementation. There was no correlation of plasma vitamin A or E results with sex, weight-for-age, height-for-age, or BMI-for-age. However, pancreatic status affected vitamin levels in individuals not taking modulators: samples from PI PwCF (n = 367) had significantly lower Vitamin A and E than those from PS PwCF (n = 71). In contrast, we have observed higher concentrations of Vitamin A retinol in individuals on modulators: 0.49 ± 0.13 mg/L on modulators vs 0.43 ± 0.12 mg/L without modulators. Pairwise analysis of samples from 76 individuals before and after modulator therapy confirmed this finding, showing an average 16% increase in serum/plasma retinol concentration (P < 0.0001). No changes in retinol were observed between different measurements in PwCF who were not taking modulators (n = 82). Vitamin E levels were not impacted by modulator therapy. Conclusions Advances in clinical care improved fat-soluble vitamin status in people with CF. However, even when supplemented and on PERT, PI PwCF not on modulators tend to have lower fat-soluble vitamin concentrations than PS PwCF. CFTR modulator therapy increases serum/plasma concentrations of vitamin A. Thus, individuals receiving modulators and vitamin supplementation should be monitored to avoid Vitamin A toxicity. This is especially true for PS PwCF who already have higher baseline vitamin levels.

How macronutrients and pancreatic enzyme supplements dose variability affect fat, protein and starch absorption in children with cystic fibrosis.

low evidence on the dose of enzymatic supplements used in pancreatic enzyme replacement therapy (PERT) is available. assessing if fat, protein and starch absorption could be related to the dose of the enzymatic supplement, the intra-patient variability in the dose and macronutrient intake. Four-day food records and 3-day faecal samples were prospectively collected in 69 children with cystic fibrosis. Pearson correlations between enzyme dose and macronutrient absorption, and beta regression models were applied to explain the results. the supply of protease units per protein intake (PU/g protein) in relation to lipase units per fat intake (LU/g fat) was low and the intra-patient variability in the dose of enzymes was ±1331 LU/g fat. Fat and starch absorption was >90% while for protein it was 81.5%. The coefficient of fat absorption was associated with an interaction between the dose of LU/g fat and its variability among different days. Lipid and protein intake were also determinants of the coefficient of fat absorption. the dose of PERT should be re-adjusted to the amount of dietary fat of every meal (constant LU/g fat) to minimize variability and increase fat absorption. Also, the supply of protease should be increased to prevent from protein malabsorption.

Selective use of pancreatic duct occlusion during pancreaticoduodenectomy in patients with a small-size duct and atrophic parenchyma in the distal pancreas: A retrospective study.

Despite the advancements in surgical techniques, postoperative pancreatic fistula (POPF) remains a potentially life-threatening complication of pancreaticoduodenectomy (PD). Pancreatic duct occlusion (PDO) without anastomosis has also been proposed to alleviate the clinical consequences of POPF in selected patients after PD. To assess the safety and effectiveness of PDO with mechanical closure after PD in patients with an atrophic pancreatic body-tail and a small pancreatic duct. We retrospectively identified two female and two male patients from April 2019 to October 2020 through preoperative computed tomography of the abdomen. Among them, three patients underwent PDO with mechanical closure after PD, and one underwent PDO after pylorus-preserving PD. In addition, patients' medical records and medium-and long-term follow-up data were analyzed. Postoperative histological examination revealed a solid pseudopapillary tumor in two patients, pancreatic ductal adenocarcinoma in one patient, and chronic pancreatitis with pancreatic duct stones in one patient. However, none of the patients developed biochemical or clinically relevant POPF, with no postpancreatectomy hemorrhage, biliary leakage, delayed gastric emptying, intra-abdominal abscess, or chyle leakage. Among the four patients, three developed new-onset diabetes mellitus, and one had impaired glucose tolerance. Furthermore, three patients received pancreatic enzyme supplementation at a dose of 90,000 Ph. Eur. units/d, and one was prescribed a higher dose of 120,000 Ph. Eur. units/d. PDO with mechanical closure is an alternative approach for patients with an atrophic pancreatic body-tail and a small pancreatic duct after PD. Therefore, further evidence should evaluate the potential benefits of selective PDO in these patients.

Pancreatic enzyme supplementation versus placebo for improvement of gastrointestinal symptoms in non-responsive celiac disease: A cross-over randomized controlled trial.

Pancreatic Exocrine Insufficiency (PEI) is a possible cause of recurrent/persistent symptoms in celiac disease. Although pancreatic enzyme supplementation may be used to treat non-responsive celiac disease (NRCD) in clinical practice, clinical outcomes are variable and there is limited and low quality evidence to support this practice. The aim of this study was to assess the efficacy of pancreatic enzyme supplements (PES) for improvement of gastrointestinal symptoms in NRCD. Prospective, randomized, placebo-controlled, double-blind, cross-over trial in adults with NRCD examining Celiac Disease-Gastrointestinal Symptom Rating Scale (CeD-GSRS) scores on PES (pancrelipase co-administered with omeprazole) versus placebo (omeprazole only) during a 10-day treatment period. The study was registered under the clinical trials registry (https://clinicaltrials.gov/ number, NCT02475369) on 18 Jun 2015. Twelve participants (nine female) were included in the per-protocol analysis; one participant had low fecal elastase-1. Pancrelipase was not associated with significant change in CeD-GSRS compared to placebo (-0.03 versus -0.26; P = 0.366). There was a significant decrease in mean values of total CeD-GSRS scores (3.58 versus 2.90, P = 0.004), abdominal pain (2.92 versus 2.42, P = 0.009), and diarrhea sub-scores (3.44 versus 2.92, P = 0.037) during the run-in period with omeprazole. In this prospective, cross-over randomized, placebo-controlled study, PES did not improve symptoms in patients with NRCD. It is unclear whether this is a trial effect or related to administration of omeprazole.

Kreon® (Creon®) vs. Lipancrea®: In Vitro Comparison of Two Encapsulated Pancreatin Preparations.

Kreon® (Creon®) and Lipancrea® are pancreatic enzyme supplements indicated in the treatment of exocrine pancreatic insufficiency. In order to determine their interchangeability, an in vitro comparison of their physical properties and enzymatic activity was carried out. Capsule fill weight and particle size were also determined in order to establish their physical properties. Amylase, lipase and protease activities, lipase release at different pHs and the dissolution time of pellets were assessed for enzymatic analysis. The length range of Kreon® and Lipancrea® pellets was 1.1-2.2 mm and 1.5-2.8 mm, respectively. Protease activity was below the label claim for Lipancrea® and above for Kreon® presentations. Lipase and amylase activity were equal to or higher than the label claim in both preparations. In dissolution experiments simulating the stomach passage, significant release of lipase activity was observed for Lipancrea® (% actual activity: 41% for Lipancrea® 8000; 21% for Lipancrea® 16000) after 60 min at pH 5.0. No release of lipase activity was observed for Kreon® at that particular pH. Enzyme release for Lipancrea® at pH 6.0 was generally slower than for Kreon® and seemed to be influenced by the preceding incubation at lower pH. More than 85% of Kreon® and Lipancrea® dissolved in a pH 6.0 phosphate buffer within 20 min. Despite the similarities of the enzyme content on the respective labels, Kreon® and Lipancrea® differ in pellet size, enzymatic activity and release. This may impact their therapeutic efficacy and, therefore, may limit their interchangeability.

S2009 Chronic Diarrhea Due to Campylobacter Infection in an Immunocompetent Host: A Case Report

Introduction: Campylobacter infection is a foodborne illness transmitted by ingestion of contaminated food or water. It is responsible for roughly 1.5 million infections annually in the U.S. Typical symptoms include acute diarrhea (< 2 weeks) that can be bloody or non-bloody, periumbilical pain, fever, and body aches. The enteritis is often self-limited in immunocompetent hosts. Subacute or chronic diarrhea can be observed in immunocompromised patients, but rarely in the immunocompetent host. Case Description/Methods: A 58-year-old male with a history of Korsakoff syndrome and anoxic brain injury was admitted with chronic diarrhea of 2 months, severe metabolic acidosis and acute kidney injury. Patient had 5 to 6 episodes of large volume watery diarrhea daily. There was serum leukocytosis with 17.2 K/mcL. Fecal immunochemical test was negative for blood. Stool culture was positive for Campylobacter antigen. CT abdomen showed numerous mildly enlarged mesenteric root lymph nodes without pericolonic fat stranding (Figure). Serum and stool inflammatory markers were elevated (Table). Stool testing for other infectious agents was unremarkable. Diarrhea did not improve with fasting. Patient did have underlying chronic pancreatitis due to prior alcohol abuse, but had been abstinent for 3 years. He was not previously on pancreatic enzyme and vitamin supplementation, which was started but did not improve his symptoms. Screening for celiac disease, HIV, and viral hepatitis was also unremarkable. It was concluded that in addition to chronic pancreatic insufficiency, Campylobacter was the culprit for his chronic diarrhea, which was treated with IV azithromycin for 10 days. Patient’s diarrhea resolved after completion of antibiotics. Repeat CT abdomen showed near-complete resolution of previously seen lymphadenopathy. Discussion: Chronic diarrhea due to Campylobacter infection is rare in immunocompetent hosts. Long-term Campylobacter colonization has been described in immunocompromised hosts. Colonization, or chronic campylobacteriosis, is confirmed by persistent Campylobacter-positive feces in the setting of prolonged diarrhea. It is typically treated with oral azithromycin or ciprofloxacin for 3 days. However, our patient required an intravenous administration and longer course of treatment since his symptoms were refractory to oral antibiotics. This is an uncommon case of complicated Campylobacter infection resulting in chronic diarrhea in an immunocompetent patient.Figure 1.: Left: CT Abdomen without contrast (axial) showing mesenteric lymphadenopathy, lymph node measuring 1.2cm x 2.4cm (arrow). Right: Resolution of lymphadenopathy after completion of 10-day course of azithromycin. Table 1. - Stool and serum laboratory profile of the patient Test Result Reference Range Stool Studies Stool culture Campylobacter sp Negative WBC Positive Negative Calprotectin 447 Normal < 50 mcg/g Pancreatic Elastase 1 < 15 Normal >200 mcg/g C. Difficile PCR Negative Negative Giardia antigen Negative Negative Fecal sodium 114 N/A Fecal potassium 14.0 N/A Fecal chloride 73 N/A Fecal Osmolality 437 275-295 mOsm/kg* Fecal Osmotic Gap 34 Secretory diarrhea: < 50 mOsm/kg Osmotic diarrhea: >75 mOsm/kg Fecal Fat (Qualitative) Abnormal (positive) Normal Ova and parasites Negative Negative Serum Studies WBC 17.2 4.8-10.8 K/mcL TSH 1.5 0.55-4.78 uIU/mL ESR 62 < 20 mm/hr for men over 50 years CRP 95 0-7.5 mg/L Lipase 7 22-51 U/L HIV 1 and 2 antibody Nonreactive Nonreactive Celiac panel (tTg IgA) Negative Negative Total IgA 293 47-310 mg/dL Blood cultures Negative Negative *Stool osmolality greater than 290 and up to 600 are common because of bacterial metabolism of fecal carbohydrate during storage of the stool sample.Abbreviations: WBC: white blood cells, CRP: C-reactive protein, HIV: human immunodeficiency virus, tTg: tissue transglutaminase, IgA: Immunoglobulin A.

Fat-Soluble Vitamin Deficiencies and Disruption of the Immune System in Pancreatic Cancer: A Vicious Cycle.

Pancreatic ductal adenocarcinoma (PDAC) is currently an increasing contributor to cancer-related mortality. Despite advances in cancer treatment, PDAC survival rates have remained roughly unchanged over the years. Specifically, late diagnosis and insensitivity to currently available therapeutic regimens have been identified as the main causes for its poor survival. Pancreatic exocrine insufficiency (PEI) is a typical complication associated with PDAC diagnosis and pancreatic surgery. Pancreatic exocrine insufficiency, a major contributor to maldigestion in PDAC, is often not treated because it remains undetected because of lack of overt signs and symptoms. In this review, we will focus on the major consequences of PEI, including the inadequacy of lipase excretion, which results in deficiency of fat-soluble vitamins. Because PDAC is known for its immune-high jacking mechanisms, we describe key features in which deficiencies of fat-soluble vitamins may contribute to the aggressive biological behavior and immune evasion in PDAC. Because PEI has been shown to worsen survival rates in patients with PDAC, detecting PEI and the related fat-soluble vitamin deficits at the time of PDAC diagnosis is critical. Moreover, timely supplementation of pancreatic enzymes and fat-soluble vitamins may improve outcomes for PDAC patients.

MIRAGE Syndrome Enteropathy Responding to Pancrelipase Despite Normal Pancreatic Fecal Elastase: A Case Report

Patient: MaLe, newborn Final Diagnosis: MIRAGE syndrome Symptoms: Adrenal insufficiency • ambiguous genitalia • colonic dilatation • dry eyes • enteropathy • hypothyroidism Medication: — Clinical Procedure: — Specialty: Endocrinology and Metabolic • Gastroenterology and Hepatology • Genetics • Pediatrics and Neonatology Objective: Rare disease Background:Major findings of myelodysplasia, infection, restriction of growth, adrenal hypoplasia, genital phenotypes, and enteropathy (MIRAGE) syndrome is a rare genetic condition caused by a gain-of-function mutation in the SAMD9 gene. It acts as a growth repressor expressed in the endothelial cells. Pathogenic variants in the SAMD9 gene lead to profound growth-restricting activity intrinsic to the protein, which further reduces cellular proliferation and instigates this growth-limiting condition. Gastrointestinal features include chronic diarrhea, severe diaper rash, and colonic dilatation. Until now, there has been no description of exocrine pancreatic insufficiency as a possible cause of enteropathy in MIRAGE syndrome.Case Report:We report a case of MIRAGE syndrome affecting multiple systems in an infant who had severe enteropathy which responded well to porcine-derived pancreatic enzyme supplements despite normal pancreatic fecal elastase level. The infant is being followed up by multidisciplinary teams in our outpatient department.Conclusions:Porcine-derived pancreatic enzyme is beneficial in enteropathy due to MIRAGE syndrome and is worth considering.

Glycemic Variability Assessment in Newly Treated Exocrine Pancreatic Insufficiency With Type 1 Diabetes.

Thirty-nine percent of people with type 1 diabetes may have lowered pancreatic elastase levels, correlated with exocrine pancreatic insufficiency (EPI or PEI). EPI is treated with oral supplementation of pancreatic enzymes. Little is known about the glycemic impact of pancreatic enzyme replacement therapy (PERT) in people with diabetes. This article demonstrates a method of assessing glycemic variability (GV), glycemic outcomes, and other changes in an individual with type 1 diabetes using open-source automated insulin delivery (AID). Macronutrient, PERT intake, and EPI-related symptoms were self-tracked; diabetes data were collected automatically via an open-source AID system. Diabetes data were uploaded via Nightscout to Open Humans and downloaded for analysis alongside self-tracked data (food, PERT). Glycemic outcomes, macronutrients, PERT dosing, and a variety of GV metrics following meals were evaluated for one month before and one month after PERT commencement. Breakfast was assessed independently across both time periods. In an n = 1 individual using an open-source AID, time in range was already above goal and improved further after PERT commencement. Glucose rate of change and excursions >180 mg/dL were reduced; mean high blood glucose index was reduced overall and more so specifically at breakfast following PERT commencement. GV can aid in assessing response to new-onset medications, as was demonstrated in this article for n = 1 individual with type 1 diabetes (using an open-source AID) after commencing PERT for newly identified EPI. GV may be useful for evaluating the efficacy of new-onset medications for people with insulin-requiring diabetes.

Continuous glucose monitoring to assess glucose variability in type 3c diabetes.

AimThe effectiveness of continuous glucose monitoring (CGM) in maintaining glycaemic control in type 1 diabetes mellitus and type 2 diabetes mellitus has been well demonstrated. However, the degree of glycaemic variability (GV) in people with type 3c diabetes mellitus has not been fully explored using CGM. This study aims to evaluate GV in type 3c diabetes mellitus participants and compare it to type 1 diabetes mellitus and type 2 diabetes mellitus.MethodsParticipants were grouped according to type of diabetes. GV, defined as percentage coefficient of variation (%CV), and other glycaemic indices were obtained using CGM (FreeStyle Libre, Abbott, Australia) from 82 participants across all three cohorts over a 14‐day period. Comparison of baseline characteristics and GV were performed across all groups. Correlation of GV with C‐peptide values, and whether pancreatic supplementation had an effect on GV were also assessed in the type 3c diabetes mellitus cohort.ResultsGV of type 3c diabetes mellitus participants was within the recommended target of less than %CV 36% (p = 0.004). Type 3c diabetes mellitus participants had the lowest GV among the three groups (p = 0.001). There was a trend for lower C‐peptide levels to be associated with higher GV in type 3c diabetes mellitus participants (p = 0.22). Pancreatic enzyme supplementation in type 3c diabetes mellitus participants did not have an effect on GV (p = 0.664).ConclusionsAlthough type 3c diabetes mellitus participants were the least variable, they had the highest mean glucose levels and estimated HbA1c, which suggests that the concept of ‘brittle’ diabetes in type 3c diabetes mellitus is not supported by the results of CGM in this study and may be leading to poorer glycaemic control.

Nonalcoholic fatty liver disease developed after pancreatoduodenectomy for solid pseudopapillary neoplasm in a 10-year-old girl: a case report

BackgroundSolid pseudopapillary neoplasms of the pancreas are rare. Moreover, pancreatoduodenectomy (PD) and postoperative care are not common in pediatric surgery. Herein, we report a case of PD and nonalcoholic fatty liver disease (NAFLD) after PD and present a literature review.Case presentationA 10-year-old girl with a suspected liver tumor was referred to our hospital. Echography, enhanced computed tomography and magnetic resonance imaging showed that the tumor coexisted with the solid and cystic parts of the pancreatic head. Since the patient was a young woman and the imaging findings were consistent with that of pancreatic solid pseudopapillary neoplasms (SPNs), we diagnosed her with pancreatic SPN. Thereafter, PD was performed, and she was discharged 10 days after the operation. Although her postoperative course was mostly uneventful, she experienced few episodes of abdominal pain and diarrhea before hospital discharge. These symptoms subsequently became more frequent and severe. The patient was urgently readmitted to the hospital for watery steatorrhea and lower abdominal colic pain. Her serum aspartate aminotransferase and alanine aminotransferase levels were elevated, and a fatty liver was detected on echography. The patient was diagnosed with steatorrhea, peristaltic pain, and NAFLD after PD. Pancrelipase (containing pancreatic digestive enzymes), antidiarrheal agents, and probiotics were started. Dosage increase of these drugs reduced the defecation frequency and abdominal pain and switched diarrhea to loose stools. However, more lipids in meals or more meals caused diarrhea and abdominal pain. Therefore, the doses of these drugs were further increased, and another antidiarrheal agent, loperamide hydrochloride, was added. Exocrine pancreatic enzymes supplementation and careful follow-up should prevent NAFLD progression after PD. At present, the patient has occasional abdominal pain, but has tangible soft stools once or twice a day. Although echography still shows a mottled fatty liver, her hepatic enzymes are only mildly elevated.ConclusionsPediatric PD is rare, and residual pancreatic function is usually sufficient, unlike in adult cases. However, we experienced a case of NAFLD after PD for a pediatric pancreatic SPN, in which pancreatic enzyme supplementation effectively improved this condition. Further attention must be paid to worsening of NAFLD that can develop nonalcoholic steatohepatitis.

ROLE OF ORAL PANCREATIC ENZYME SUPPLEMENTATION IN PANCREATIC EXOCRINE DEFICIENCY

Objectives: The present study was carried out to study the role of oral pancreatic enzyme supplementation in pancreatic exocrine deficiency. Methods: This study included 50 consecutive cases of pancreatic exocrine deficiency. Diagnosis of pancreatic exocrine deficiency was made based on history, clinical examination, and contrast-enhanced computed tomography findings. Each patient was supplied with oral pancreatic enzyme supplements. Each patient was followed up for 1 year with three visits (3 months, 6 months, and 12 months) to assess changes in clinical features of pancreatic exocrine deficiency, change in nutritional status of the patient, and compliance with therapy. Results: At first follow-up visit (3 months), abdominal discomfort reduced in 17 previously symptomatic patients. Mean stool frequency reduced from 3.18 per day to 2.34 per day. Stool consistency improved with only 18 patients (36%) having liquid consistency stool in comparison to 76% at the time of initial presentation. Average body mass index (BMI) improved from baseline value of 20.648 kg/sqm to 20.674 kg/sqm. Average hemoglobin improved from 10.40 g/dL to 10.52 g/dL and average serum albumin remained static at 3.0 g/dL. At second follow-up visit (6 months), abdominal discomfort reduced in 20 previously symptomatic patients. Mean stool frequency reduced from 3.18 per day (primary survey) to 1.7 per day. Stool consistency improved with only 12 patients (24%) having liquid consistency stool in comparison to 76% at the time of initial presentation. Average BMI improved from baseline value of 20.648 kg/sqm to 21.062 kg/sqm. Average hemoglobin improved from 10.40 g/dL to 10.69 g/dL and average serum albumin improved from 3.0 g/dL at primary survey to 3.1 g/dL. At third follow-up visit (12 months), abdominal discomfort reduced in 30 previously symptomatic patients. Mean stool frequency reduced from 3.18 per day (primary survey) to 1.6 per day. Stool consistency improved with only 9 patients (18%) having liquid consistency stool in comparison to 76% at the time of initial presentation. Average BMI improved from baseline value of 20.648 kg/sqm to 21.402 kg/sqm. Average hemoglobin improved from 10.40 g/dL to 10.76 g/dL and average serum albumin improved from 3.0 g/dL at primary survey to 3.3 g/dL. Conclusion: In follow-up visits, there was an improvement in symptoms over 12 months. Abdominal discomfort and stool frequency reduced. Stool consistency improved. Nutritional parameters showed statistically significant improvement. Mean BMI of the study sample, mean hemoglobin, and serum albumin increased. The study provides rationale for using these clinical symptoms as surrogate markers for the efficacy of Pancreatic Enzyme Replacement Therapy in patients with pancreatic exocrine insufficiency.

P-OGC03 Pancreatic enzyme supplementation improves quality of life in patients following surgery for upper GI cancer

Abstract Background Unpleasant abdominal symptoms are common following surgery for upper gastrointestinal (UGI) cancer and may occur secondary to pancreatic exocrine insufficiency (EPI). This study investigated symptoms of EPI in patients following surgery and assessed the effect of pancreatic enzyme supplementation (PERT) on these symptoms and the effect of supplementation on quality of life. Methods Patients were assessed for symptoms of EPI using a novel questionnaire. Patients who reported two or more symptoms suggestive of EPI were prescribed PERT. Abdominal symptoms were reassessed following treatment. Quality of life (QoL) was studied using the SF-36 questionnaire before and after treatment. Faecal elastase was also measured in a patient subgroup. Results Fifty-six out of 57 patients (98%) reported at least two symptoms of EPI. Following PERT every patient reported fewer abdominal symptoms; median 5 symptoms before treatment reduced to two symptoms following treatment (p &amp;lt; 0.0001; Wilcoxon rank). Reduced faecal elastase concentration was associated with more frequent abdominal symptoms; median 5 symptoms versus 3 symptoms (p = 0.043; Mann Whitney U test). PERT increased quality of life scores for every patient in each of the 5 principle health domains. Conclusions Symptoms of EPI are common among patients following UGI cancer surgery. PERT reduces unpleasant abdominal symptoms and this leads to significant improvements in quality of life across global health domains. PERT should be offered to all post-operative UGI cancer patients with symptoms suggestive of EPI.

Incidence and risk factors of nonalcoholic fatty liver disease after total pancreatectomy: A first multicenter prospective study in Japan.

In the present study we aimed to prospectively assess the current prevalence and risk factors of nonalcoholic fatty liver disease (NAFLD) after total pancreatectomy (TP). Between August 2015 and December 2017, we prospectively collected data from 68 Japanese centers on 148 consecutive patients who underwent TP whose computed tomography (CT) attenuation values were evaluated for 12months. We defined post-TP NAFLD as a liver parenchyma CT value of less than 40 Hounsfield units (HU). Data on perioperative variables were retrieved from all patients and evaluated using univariate and multivariate analyses to identify the perioperative risk factors of NAFLD. In this prospective cohort study, supplementation of pancreatic exocrine enzymes was provided to all 148 patients, and 97% of them were treated with high-titer pancrelipase (median dosage: 1800mg) postoperatively. Indeed, 29 patients (19.6%) developed NAFLD within a year after TP. Multivariate analysis revealed that female sex (P=.002), higher body mass index (BMI) (P=.001), and postoperative diarrhea (P=.038) were independent risk factors for post-TP NAFLD. However, post-TP NAFLD ameliorated in 11 patients (37.9%) at 12months after surgery. Among patients with risk factors such as female sex, higher BMI, and postoperative diarrhea, attention should be paid to the occurrence of NAFLD after TP.

Indocyanine green fluorescence to ensure perfusion in middle segment-preserving pancreatectomy: a case report

BackgroundMiddle segment-preserving pancreatectomy (MSPP) is an alternative to total pancreatectomy that allows for the preservation of the endocrine and exocrine functions of the pancreas. However, maintaining perfusion to the pancreatic remnant is of critical importance. We describe the first case to our knowledge in which indocyanine green (ICG) fluorescence was used to confirm perfusion to the pancreatic remnant during MSPP.Case presentationA 79-year-old man with diabetes mellitus was referred to our hospital for treatment of a pancreatic tumor. Computed tomography revealed a hypovascular mass in the uncus of the pancreas and dilatation of the main pancreatic duct, measuring 13 mm in the tail of the pancreas. He was diagnosed with cancer of the pancreatic uncus via endoscopic ultrasound and fine-needle aspiration revealed a mixed-type intraductal papillary mucinous neoplasm (IPMN), along with high-risk stigmata in the tail of the pancreas. We performed MSPP and the length of the pancreatic remnant was 4.6 cm. The dorsal pancreatic artery was preserved and perfusion to the pancreatic remnant was confirmed by ICG fluorescence. Histopathological examination showed a pancreatic ductal adenocarcinoma in the uncus (pT1cN1M0, pStage 2B) and IPMN in the tail of the pancreas. The postoperative course was complicated by a grade B pancreatic fistula, but this was successfully treated with conservative management. The patient was transferred to a hospital 33 days after surgery. Insulin administration was necessary, but C-peptide was detectable and blood glucose was relatively well-controlled. He did not exhibit any exocrine dysfunction when pancreatic enzyme supplementation was administered.ConclusionICG fluorescence can be used to evaluate perfusion to the pancreatic remnant during MSPP.