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Pancreatic enzyme replacement therapy

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https://www.readbyqxmd.com/read/30487519/a-preclinical-model-of-chronic-pancreatitis-driven-by-trypsinogen-autoactivation
#1
Andrea Geisz, Miklós Sahin-Tóth
Inflammatory diseases of the pancreas have no specific therapy. Discovery of the genetic basis of chronic pancreatitis identified the digestive enzyme trypsin as a therapeutic target. Preclinical testing of trypsin inhibition has been hampered by the lack of animal models. Here we report the T7D23A knock-in mouse, which carries a heterozygous p.D23A mutation in mouse cationic trypsinogen (isoform T7). This trypsinogen mutant autoactivates to trypsin 50-fold faster than wild type. T7D23A mice develop spontaneous acute pancreatitis with edema, necrosis and serum amylase elevation at an early age followed by progressive atrophic chronic pancreatitis with acinar cell loss, fibrosis, dilated ducts and adipose replacement...
November 28, 2018: Nature Communications
https://www.readbyqxmd.com/read/30397535/challenges-in-the-management-of-pancreatic-exocrine-insufficiency
#2
EDITORIAL
Benjamin Myles Shandro, Rani Nagarajah, Andrew Poullis
Pancreatic exocrine insufficiency (PEI) occurs when the insufficient secretion or function of pancreatic enzymes leads to maldigestion, most commonly as a result of chronic pancreatitis and pancreatic cancer. The condition is associated with significant morbidity and reductions in quality of life, even in milder forms. The challenges in approaching this condition include the non-specific presentation of mild to moderate PEI, and the lack of a convenient, accurate diagnostic test in this cohort. Classical symptoms appear late in the disease, and the diagnosis should be considered before steatorrhoea develops...
October 25, 2018: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/30385188/enzyme-replacement-improves-survival-among-patients-with-pancreatic-cancer-results-of-a-population-based-study
#3
K J Roberts, C A Bannister, H Schrem
Pancreatic exocrine insufficiency (PEI) and malnutrition are prevalent among patients with pancreatic adenocarcinoma. Pancreatic enzyme replacement therapy (PERT) can correct PEI but its use among patients with pancreatic cancer is unclear as are effects upon survival. This population-based study sought to address these issues METHODS: Subjects with pancreatic adenocarcinoma were identified from the UK Clinical Practice Research Datalink (CPRD). Propensity score matching generated matched pairs of subjects who did and did not receive PERT...
October 24, 2018: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/30308536/clinical-characteristics-and-genetic-causes-of-infantile-exocrine-pancreatic-insufficiency-in-chinese-patients-study-from-a-tertiary-care-center
#4
Ziqing Ye, Ying Zhou, Ying Huang, Hua Sun, Huijun Wang, Bingbing Wu
OBJECTIVE: Infantile exocrine pancreatic insufficiency is a rare disease. We examined phenotypes and performed genetic sequencing in children with this disorder. METHODS: We enrolled 4 infants with exocrine pancreatic insufficiency. Patients were characterized by phenotypes and radiologic findings. Genetic sequencing was performed. RESULTS: Average age of disease onset was 2 months. Average (standard deviation [SD]) age at diagnosis was 11...
November 2018: Pancreas
https://www.readbyqxmd.com/read/30193081/fecal-fat-and-energy-loss-in-pancreas-exocrine-insufficiency-the-role-of-pancreas-enzyme-replacement-therapy
#5
Friedemann Erchinger, Anne Kristine N Øvre, Marita Malene Aarseth, Trond Engjom, Ingeborg Brønstad, Georg Dimcevski, Oddrun Anita Gudbrandsen, Erling Tjora
BACKGROUND: Chronic pancreatitis (CP) can lead to severe pancreatic exocrine insufficiency (PEI). Pancreatic enzyme replacement therapy (PERT) is well established, but knowledge of the physiological response to increasing doses on fecal fat- and energy loss is scarce. METHODS: We included 10 patients with CP and established PEI and 12 healthy controls for a prospective interventional study. Subjects received no PERT in the first week followed by four weeks PERT incrementally increasing doses every week...
September 2018: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/30151918/diabetes-of-the-exocrine-pancreas
#6
REVIEW
Katie Wynne, Benedict Devereaux, Anne Dornhorst
Diabetes of the exocrine pancreas (DEP) is a form of diabetes that occurs due to pancreatic disease. It is far more common than has been previously considered, with a recent study showing 1.8% of adults with new-onset diabetes should have been classified as DEP. The majority is misdiagnosed as type 2 diabetes mellitus (T2DM). Patients with DEP exhibit varying degrees of exocrine and endocrine dysfunction. Damage to the islet of Langerhans effects the secretion of hormones from the β, α, and pancreatic polypeptide cells; the combination of low insulin, glucagon, and pancreatic polypeptide contributes to rapid fluctuations in glucose levels...
August 27, 2018: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/30133868/serum-concentrations-of-lipid-soluble-vitamins-in-dogs-with-exocrine-pancreatic-insufficiency-treated-with-pancreatic-enzymes
#7
Patrick C Barko, David A Williams
BACKGROUND: In humans, exocrine pancreatic insufficiency (EPI) is associated with deficiencies in lipid-soluble vitamins. Little is reported regarding lipid-soluble vitamin status in dogs with EPI. HYPOTHESIS/OBJECTIVES: Compare serum concentrations of retinol, 25-hydrocholecalciferol (25OHD), and α-tocopherol among dogs with EPI, those with subclinical EPI (sEPI), and healthy dogs. Detect associations between serum concentrations of lipid-soluble vitamins and residual clinical signs in treated dogs with EPI and sEPI...
September 2018: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/30101991/nutrition-and-inflammatory-biomarkers-in-chronic-pancreatitis-patients
#8
Julia B Greer, Phil Greer, Bimaljit S Sandhu, Samer Alkaade, C Mel Wilcox, Michelle A Anderson, Stuart Sherman, Timothy B Gardner, Michele D Lewis, Nalini M Guda, Thiruvengadam Muniraj, Darwin Conwell, Gregory A Cote, Christopher E Forsmark, Peter A Banks, Gong Tang, Kim Stello, Andres Gelrud, Randall E Brand, Adam Slivka, David C Whitcomb, Dhiraj Yadav
BACKGROUND: Chronic pancreatitis (CP) patients frequently experience malabsorption and maldigestion, leading to micronutrient and macronutrient deficiencies. Comorbid diabetes and lifestyle habits, such as alcohol consumption, may impact nutrition status. METHODS: We compared micronutrient antioxidant, bone metabolism, serum protein, and inflammatory marker levels in 301 CP patients and 266 controls with no known pancreatic disease. We analyzed serum prealbumin and retinol binding protein; vitamins A, D, E, and B12; osteocalcin; tumor necrosis factor-α; and C-reactive protein (CRP)...
August 13, 2018: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/30094008/exocrine-pancreatic-dysfunction-is-common-in-hepatocyte-nuclear-factor-1%C3%AE-associated-renal-disease-and-can-be-symptomatic
#9
Rhian L Clissold, Jon Fulford, Michelle Hudson, Beverley M Shields, Timothy J McDonald, Sian Ellard, Andrew T Hattersley, Coralie Bingham
Background: Heterozygous mutations in the HNF1B gene are the most common monogenic cause of developmental kidney disease. Extrarenal phenotypes frequently occur, including diabetes mellitus and pancreatic hypoplasia; the latter is associated with subclinical exocrine dysfunction. We measured faecal elastase-1 in patients with HNF1B-associated disease regardless of diabetes status and assessed the degree of symptoms associated with pancreatic exocrine deficiency. Methods: Faecal elastase-1 was measured in 29 patients with a known HNF1B mutation...
August 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/30077689/critical-care-of-the-adult-patient-with-cystic-fibrosis
#10
REVIEW
Christopher S King, A Whitney Brown, Shambhu Aryal, Kareem Ahmad, Scott Donaldson
Cystic fibrosis (CF) is a multiorgan, genetic disease resulting from dysfunction of the CF transmembrane conductance regulator. The primary clinical manifestations are bronchiectasis, chronic pulmonary infections with recurrent exacerbations, and pancreatic insufficiency. Advances in CF care have led to improved survival, with more than one-half of those affected being adults. As a result, adult pulmonary and critical care physicians increasingly will be involved in the care of these patients. Patients with CF are at risk for numerous conditions that require ICU admission, including respiratory failure, massive hemoptysis, pneumothorax, hepatic failure, and bowel obstruction...
August 2, 2018: Chest
https://www.readbyqxmd.com/read/30074926/exocrine-pancreatic-insufficiency-and-malnutrition-in-chronic-pancreatitis-identification-treatment-and-consequences
#11
May Min, Boskey Patel, Samuel Han, Lisa Bocelli, Joan Kheder, Aditya Vaze, Wahid Wassef
OBJECTIVES: The purpose of this study was to examine the impact of exocrine pancreatic insufficiency (EPI) on chronic pancreatitis (CP) patients and to identify challenges with its diagnosis and treatment. METHODS: Ninety-one patients with CP diagnosed with endoscopic ultrasound were identified and assessed for symptoms of EPI, fat-soluble vitamin levels, dual-energy x-ray absorptiometry scan T-scores, and treatment with pancreatic enzyme replacement therapy. All patients were also screened with the Malnutrition Universal Screening Test...
September 2018: Pancreas
https://www.readbyqxmd.com/read/30052568/relationship-of-initial-pancreatic-enzyme-replacement-therapy-dose-with-weight-gain-in-infants-with-cystic-fibrosis
#12
Michael S Schechter, Suzanne Michel, Shufang Liu, B Woun Seo, Mudra Kapoor, Rupal Khurmi, Mark Haupt
OBJECTIVE: The aim of the study is to test the hypothesis of a positive relationship between initial dose of pancreatic enzyme replacement therapy (PERT) in infants with cystic fibrosis (CF) and optimal weight gain over the first 2 years of life. METHODS: Using the CF Foundation Patient Registry, we identified 502 children born in 2010 and used multivariable models to compare as our primary analysis their 2-year changes in weight-for-age z score (WAZ) and as our secondary analysis weight-for-length percentile (W/L%) by initial PERT dose...
October 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/30029989/nutritional-support-and-therapy-in-pancreatic-surgery-a-position-paper-of-the-international-study-group-on-pancreatic-surgery-isgps
#13
Luca Gianotti, Marc G Besselink, Marta Sandini, Thilo Hackert, Kevin Conlon, Arja Gerritsen, Oonagh Griffin, Abe Fingerhut, Pascal Probst, Mohammed Abu Hilal, Giovanni Marchegiani, Gennaro Nappo, Alessandro Zerbi, Antonio Amodio, Julie Perinel, Mustapha Adham, Massimo Raimondo, Horacio J Asbun, Asahi Sato, Kyoichi Takaori, Shailesh V Shrikhande, Marco Del Chiaro, Maximilian Bockhorn, Jakob R Izbicki, Christos Dervenis, Richard M Charnley, Marc E Martignoni, Helmut Friess, Nicolò de Pretis, Dejan Radenkovic, Marco Montorsi, Michael G Sarr, Charles M Vollmer, Luca Frulloni, Markus W Büchler, Claudio Bassi
BACKGROUND: The optimal nutritional therapy in the field of pancreatic surgery is still debated. METHODS: An international panel of recognized pancreatic surgeons and pancreatologists decided that the topic of nutritional support was of importance in pancreatic surgery. Thus, they reviewed the best contemporary literature and worked to develop a position paper to provide evidence supporting the integration of appropriate nutritional support into the overall management of patients undergoing pancreatic resection...
November 2018: Surgery
https://www.readbyqxmd.com/read/30027527/diagnosis-and-management-of-exocrine-pancreatic-insufficiency
#14
REVIEW
Chris E Forsmark
PURPOSE OF REVIEW: Recent studies have documented that many patients with pancreatic exocrine insufficiency (EPI) are not identified and are not treated with appropriate dosages of pancreatic enzyme replacement therapy. This review will summarize the approach to diagnosis, treatment, and monitoring for treatment effect and complications in patients with exocrine insufficiency. RECENT FINDINGS: While chronic pancreatitis is the most commonly identified cause of EPI, pancreatic cancer and pancreatic surgery are increasingly important...
September 2018: Current Treatment Options in Gastroenterology
https://www.readbyqxmd.com/read/29946424/chronic-pancreatitis-review-and-update-of-etiology-risk-factors-and-management
#15
REVIEW
Angela Pham, Christopher Forsmark
Chronic pancreatitis is a syndrome involving inflammation, fibrosis, and loss of acinar and islet cells which can manifest in unrelenting abdominal pain, malnutrition, and exocrine and endocrine insufficiency. The Toxic-Metabolic, Idiopathic, Genetic, Autoimmune, Recurrent and Severe Acute Pancreatitis, Obstructive (TIGAR-O) classification system categorizes known causes and factors that contribute to chronic pancreatitis. Although determining disease etiology provides a framework for focused and specific treatments, chronic pancreatitis remains a challenging condition to treat owing to the often refractory, centrally mediated pain and the lack of consensus regarding when endoscopic therapy and surgery are indicated...
2018: F1000Research
https://www.readbyqxmd.com/read/29908691/pancreatic-enzyme-replacement-therapy-following-surgery-for-pancreatic-cancer-an-exploration-of-patient-self-management
#16
Lisa Dunleavy, Ahmed Al-Mukhtar, Vanessa Halliday
BACKGROUND: For those diagnosed with pancreatic cancer, ill-addressed pancreatic exocrine insufficiency (PEI) following surgery can result in malnutrition related complications that may impact on predict mortality and morbidity. The use of pancreatic enzyme replacement therapy (PERT) is recommended and often demands a degree of patient self-management. Understanding more about how this treatment is managed is fundamental to optimising care. OBJECTIVE: This study aimed to explore patient self-management of PERT following surgery for pancreatic cancer...
August 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29889111/diagnosis-and-treatment-of-pancreatic-exocrine-insufficiency
#17
J Enrique Dominguez-Muñoz
PURPOSE OF REVIEW: Pancreatic exocrine insufficiency (PEI), defined as a secretion of pancreatic enzymes and bicarbonate insufficient to maintain a normal digestion, is a frequent but frequently underdiagnosed and undertreated condition. PEI may be secondary to different pancreatic diseases and extrapancreatic conditions. Recent data support the high clinical relevance of PEI and its treatment. RECENT FINDINGS: Together with symptoms of maldigestion, PEI is associated with nutritional deficiencies leading to osteoporosis, low-trauma fractures, sarcopenia and increased mortality...
September 2018: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/29851751/a-multicenter-open-label-randomized-controlled-trial-of-pancreatic-enzyme-replacement-therapy-in-unresectable-pancreatic-cancer
#18
Tomotaka Saito, Yousuke Nakai, Hiroyuki Isayama, Kenji Hirano, Kazunaga Ishigaki, Ryunosuke Hakuta, Tsuyoshi Takeda, Kei Saito, Gyotane Umefune, Dai Akiyama, Takeo Watanabe, Kaoru Takagi, Naminatsu Takahara, Tsuyoshi Hamada, Rie Uchino, Suguru Mizuno, Dai Mouri, Hiroshi Yagioka, Hirofumi Kogure, Osamu Togawa, Saburo Matsubara, Yukiko Ito, Natsuyo Yamamoto, Minoru Tada, Kazuhiko Koike
OBJECTIVE: Exocrine pancreatic insufficiency may impair the nutritional status in pancreatic cancer (PC), but the role of pancreatic enzyme replacement therapy (PERT) is not fully evaluated. Therefore, we conducted this multicenter open-label randomized controlled trial to evaluate the role of PERT in PC patients. METHODS: Patients with unresectable PC receiving chemotherapy were randomly assigned to pancrelipase and nonpancrelipase groups. Patients in the pancrelipase group took oral pancrelipase of 48,000 lipase units per meal...
August 2018: Pancreas
https://www.readbyqxmd.com/read/29804317/shwachman-diamond-syndrome-nationwide-survey-and-systematic-review-in-japan
#19
REVIEW
Tamaki Ikuse, Takahiro Kudo, Katsuhiro Arai, Yoshimitsu Fujii, Shinobu Ida, Tomohiro Ishii, Sotaro Mushiake, Kouji Nagata, Hiroshi Tamai, Akira Toki, Takeshi Tomomasa, Kosuke Ushijima, Tadahiro Yanagi, Takeo Yonekura, Tomoaki Taguchi, Toshiaki Shimizu
BACKGROUND: Shwachman-Diamond syndrome (SDS) is a rare multisystem disorder associated with exocrine pancreatic insufficiency. The present study reports the results of a nationwide survey and a systematic review on SDS to develop consensus guidelines for intractable diarrhea including SDS. METHODS: Questionnaires were sent to 616 departments of pediatrics or of pediatric surgery in Japan in a nationwide survey. A second questionnaire was sent to doctors who had treated SDS patients and included questions on clinical information...
August 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29737796/pancreatic-enzyme-replacement-therapy-pert-in-children-with-persistent-diarrhea-avoidance-of-elemental-diet-need-accessibility-and-costs
#20
Ariani Dewi Widodo, Rianto Setiabudy, Ina S Timan, Saptawati Bardosono, Widdy Winarta, Agus Firmansyah
BACKGROUND AND OBJECTIVES: Persistent diarrhea has been proven to cause pancreatic exocrine insufficiency, due to decreased stimulation to the pancreas caused by prolonged mucosal injury. Pancreatic enzyme replacement therapy (PERT) given in conjunction to regular treatment is thought to be beneficial in replacing this pancreatic enzyme deficiency, avoiding the need of elemental diet. This study aims to evaluate the benefit of PERT in chil-dren with persistent diarrhea. METHODS AND STUDY DESIGN: This is a randomized, two double-blind parallel group, placebo-controlled clinical trial to evaluate the effects of pancreatic enzyme supplementation in persistent diar-rhea...
2018: Asia Pacific Journal of Clinical Nutrition
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