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exocrine insufficiency

Norman Schöffel, Diana Rubin, David A Groneberg, Ralf-Marco Liehr
No abstract text is available yet for this article.
October 2016: MMW Fortschritte der Medizin
E Bellon, J R Izbicki, M Bockhorn
Chronic pancreatitis (CP) is an irreversible, inflammatory process, which is characterized by progressive fibrosis of the pancreas and leads to abdominal pain, endocrine and exocrine insufficiency. Surgical therapy is indicated by the absence of pain relief and local complications. The target of the surgical approach is to relieve the pancreatic and bile ducts and resection of the fibrotic and calcified parenchyma. Drainage procedures, such as the Partington-Rochelle method, are used in patients with isolated congestion of the pancreatic duct without further organ complications, such as inflammatory processes of the pancreatic head; however, patients with CP often have an inflammatory swelling of the pancreatic head...
October 18, 2016: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
Dorota Gliwicz, Irena Jankowska, Aldona Wierzbicka, Anna Miśkiewicz-Chotnicka, Aleksandra Lisowska, Jarosław Walkowiak
Alagille syndrome (AGS) is often associated with symptoms of maldigestion, such as steatorrhea, hypotrophy and growth retardation. Exocrine pancreatic insufficiency was proposed as the underlying cause. We aimed to assess the exocrine pancreatic function with the use of different methods in AGS patients. Concentrations of fecal elastase-1 (FE1) and fecal lipase (FL) activities were measured in 33 children with AGS. The C-mixed triglyceride breath test (MTBT) in a subgroup comprising 15 patients. In all patients studied, FE1 concentrations and FL activities were normal...
October 17, 2016: Scientific Reports
Ryuta Shintakuya, Kenichiro Uemura, Yoshiaki Murakami, Naru Kondo, Naoya Nakagawa, Kazuhide Urabe, Keisuke Okano, Kazuo Awai, Toru Higaki, Taijiro Sueda
BACKGROUND/OBJECTIVES: The loss of skeletal muscle mass (sarcopenia) is associated with the poor prognosis of pancreatic cancer. It has been reported pancreatic exocrine insufficiency (PEI) is associated with serum nutritional markers in chronic pancreatitis. However, there has been no report about the relationship between sarcopenia and PEI. The aim of this study is to determine whether body composition, including skeletal muscle (SM), subcutaneous adipose tissue (SAT), visceral adipose tissue (VAT), intramuscular adipose tissue content (IMAC), and serum nutritional markers are associated with pancreatic exocrine function in patients with pancreatic disease...
October 11, 2016: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
Geert Roeyen, Miet Jansen, Laure Ruyssinck, Thiery Chapelle, Aude Vanlander, Bart Bracke, Vera Hartman, Dirk Ysebaert, Frederik Berrevoet
OBJECTIVE: Recently, pancreaticogastrostomy (PG) has attracted renewed interest as a reconstruction technique after pancreaticoduodenectomy (PD), as it may imply a lower risk of clinical pancreatic fistula than reconstruction by pancreaticojejunostomy (PJ). We hypothesise that pancreatic exocrine insufficiency (PEI) is more common during clinical follow-up after PG than it is after PJ. RESEARCH DESIGN AND METHODS: This study compares the prevalence of PEI in patients undergoing PD for malignancy with reconstruction by PG versus reconstruction by PJ...
October 7, 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Jean A Donet, Frank Czul, Nathalie A Peña, Jamie S Barkin
Autoimmune pancreatitis (AIP) is an uncommon disease that represents a diagnostic challenge unless it is considered as a cause of acute pancreatitis, pancreatic exocrine insufficiency and a pancreatic mass. This entity is under diagnosed and successful medical therapy is available. In this paper, we will describe a case of a 59 year-old, Hispanic woman diagnosed with autoimmune pancreatitis, a disease previously believed to affect typically older men. We will review the definition, types, clinical manifestations, radiological features, serology, histopathological findings, treatment strategies and diagnostic criteria of autoimmune pancreatitis...
July 2016: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Marie Szymanowski, Maria Salomon Estebanez, Raja Padidela, Bing Han, Karolina Mosinska, Adam Stevens, Lena Damaj, Florence Pihan-Le Bars, Emilie Lascouts, Rachel Reynaud, Catherine Ferreira, Claire Bansept, Pascale de Lonlay, Cécile Saint-Martin, Mark J Dunne, Indraneel Banerjee, Jean-Baptiste Arnoux
CONTEXT: Congenital hyperinsulinism (CHI) is the commonest cause of persistent hypoglycemia in neonates and infants. In medically unresponsive CHI, subtotal pancreatectomy is performed to achieve euglycaemia with consequent diabetes in later life. Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor has been reported to obviate the need for pancreatectomy, but experience is limited. OBJECTIVE: We have investigated the efficacy and adverse effect profile of mTOR inhibitors in the treatment of severe CHI...
October 3, 2016: Journal of Clinical Endocrinology and Metabolism
Anne Mößeler, Marion Schmicke, Martin Höltershinken, Martin Beyerbach, Josef Kamphues
Pancreatic exocrine insufficiency (PEI) is a disease of diverse aetiology-e.g., majority of patients suffering from cystic fibrosis (CF) show PEI congenitally. Malnutrition and malabsorption of nutrients impair growth and nutritional status. As reduced fat digestion leads to a deficiency of fat-soluble vitamins the supplementation is standard, but absorption is a critical point in PEI-patients. The pancreatic duct ligated (PL) pig is an established model for PEI in humans and has been proven to be a suitable model to compare different vitamin additives for supplementation...
2016: International Journal of Molecular Sciences
Jennifer A Campbell, David S Sanders, Katherine A Francis, Matthew Kurien, Sai Lee, Hatim Taha, Arvind Ramadas, Diamond Joy, Andrew D Hopper
BACKGROUND AND AIMS: Pancreatic exocrine insufficiency may be under recognised in gastroenterological practice. We aimed to identify the prevalence of pancreatic insufficiency in secondary care gastroenterology clinics and determine if co-morbidity or presenting symptoms could predict diagnosis. A secondary aim was to assess response to treatment. METHODS: A dual centre retrospective analysis was conducted in secondary care gastroenterology clinics. Patients tested for pancreatic exocrine insufficiency with faecal elastase-1 (FEL-1) between 2009 and 2013 were identified in two centres...
September 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
Emma Martínez-Moneo, Serena Stigliano, Aleksandra Hedström, Aleksandra Kaczka, Marko Malvik, Alexander Waldthaler, Patrick Maisonneuve, Peter Simon, Gabriele Capurso
BACKGROUND AND AIMS: Chronic pancreatitis (CP) patients are at risk for fat-soluble vitamins (A, D, E, K) deficiency, but available studies are small and heterogeneous. We conducted a systematic review and meta-analysis to determine the prevalence of fat-soluble vitamins deficiency in CP patients. METHODS: Medline was searched up to January 2016 for case series and case-control studies reporting prevalence of fat-soluble vitamin deficiency in CP patients. The prevalent deficiency rate was pooled for included studies, and deficiency rate between CP and controls, with relative odds ratio (OR) and 95% confidence interval (CI) calculated for case-control studies...
September 20, 2016: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
Tsukasa Ikeura, Hideaki Miyoshi, Masaaki Shimatani, Kazushige Uchida, Makoto Takaoka, Kazuichi Okazaki
Autoimmune pancreatitis (AIP) has been considered a favorable-prognosis disease; however, currently, there is limited information on natural course of AIP during long-term follow-up. Recently published studies regarding the long-term outcomes of AIP has demonstrated the developments of pancreatic stone formation, exocrine insufficiency, and endocrine insufficiency are observed in 5%-41%, 34%-82%, and 38%-57% of patients having the disease. Furthermore, the incidence rate of developing pancreatic cancer ranges from 0% to 4...
September 14, 2016: World Journal of Gastroenterology: WJG
Bo Kong, Philipp Bruns, Nora A Behler, Ligong Chang, Anna Melissa Schlitter, Jing Cao, Andreas Gewies, Jürgen Ruland, Sina Fritzsche, Nataliya Valkovskaya, Ziying Jian, Ivonne Regel, Susanne Raulefs, Martin Irmler, Johannes Beckers, Helmut Friess, Mert Erkan, Nikola S Mueller, Susanne Roth, Thilo Hackert, Irene Esposito, Fabian J Theis, Jörg Kleeff, Christoph W Michalski
OBJECTIVE: The initial steps of pancreatic regeneration versus carcinogenesis are insufficiently understood. Although a combination of oncogenic Kras and inflammation has been shown to induce malignancy, molecular networks of early carcinogenesis remain poorly defined. DESIGN: We compared early events during inflammation, regeneration and carcinogenesis on histological and transcriptional levels with a high temporal resolution using a well-established mouse model of pancreatitis and of inflammation-accelerated Kras(G12D)-driven pancreatic ductal adenocarcinoma...
September 19, 2016: Gut
P G Xenoulis, D L Zoran, G T Fosgate, J S Suchodolski, J M Steiner
BACKGROUND: Little information is available about the clinical presentation and response to treatment of cats with exocrine pancreatic insufficiency (EPI). OBJECTIVES: To describe the signalment, clinical signs, concurrent diseases, and response to treatment of cats with EPI. ANIMALS: One hundred and fifty cats with EPI. METHODS: Retrospective case series. RESULTS: Questionnaires were sent to 261 veterinarians, and 150 (57%) were returned with data suitable for statistical analysis...
September 19, 2016: Journal of Veterinary Internal Medicine
Sergio Alfieri, Giuseppe Quero, Fausto Rosa, Dario Di Miceli, Antonio Pio Tortorelli, Giovanni Battista Doglietto
Severe post-operative complications after pancreaticoduodenectomy (PD) are largely due to pancreatic fistula onset. The occlusion of the main pancreatic duct using synthetic glue may prevent these complications. Aim of this study is to describe this technique and to report short- and long-term results as well as the post-operative endocrine and exocrine insufficiency. Two hundred and four patients who underwent PD with occlusion of the main pancreatic duct in a period of 15 years were retrospectively analyzed...
September 8, 2016: Updates in Surgery
Peter Colin Gregory, Katrin Hoffmann, Josef Kamphues, Anne Möeler
Modern therapy of pancreatic exocrine insufficiency (PEI) using pancreatic enzyme replacement therapy (PERT) has largely been very effective and has greatly helped in improving the nutritional status of patients with PEI and in increasing the life expectancy in cystic fibrosis. It is believed that the use of predictable large animal models could play an important role in assessing and developing new therapies. This article reviews the pancreatic duct ligated (adult) minipig as a chronic model of total PEI, with a detailed look at the influence of PEI and response to PERT on prececal compared to fecal digestibility, to directly investigate effects on protein and starch digestion and absorption...
October 2016: Pancreas
R El-Khairi, L Vallier
Heterozygous mutations in the gene that encodes the transcription factor hepatocyte nuclear factor 1β (HNF1B) result in a multi-system disorder. HNF1B was initially discovered as a monogenic diabetes gene; however, renal cysts are the most frequently detected feature. Other clinical features include pancreatic hypoplasia and exocrine insufficiency, genital tract malformations, abnormal liver function, cholestasis and early-onset gout. Heterozygous mutations and complete gene deletions in HNF1B each account for approximately 50% of all cases of HNF1B-associated disease and may show autosomal dominant inheritance or arise spontaneously...
September 2016: Diabetes, Obesity & Metabolism
Kara L Raphael, Field F Willingham
Hereditary pancreatitis (HP) is a rare cause of acute, recurrent acute, and chronic pancreatitis. It may present similarly to other causes of acute and chronic pancreatitis, and often there has been a protracted evaluation prior to the diagnosis of HP. Since it was first described in 1952, multiple genetic defects that affect the action of digestive enzymes in the pancreas have been implicated. The most common mutations involve the PRSS1, CFTR, SPINK1, and CTRC genes. New mutations in these genes and previously unrecognized mutations in other genes are being discovered due to the increasing use of next-generation genomic sequencing...
2016: Clinical and Experimental Gastroenterology
Lucia Nacci, Roberto Valli, Rita Maria Pinto, Marco Zecca, Marco Cipolli, Jacopo Morini, Simone Cesaro, Emanuela Boveri, Vittorio Rosti, Paola Corti, Maura Ambroni, Francesco Pasquali, Cesare Danesino, Emanuela Maserati, Antonella Minelli
Shwachman-Diamond syndrome (SDS) (OMIM 260400) is a rare autosomal recessive disease characterized by exocrine pancreatic insufficiency, skeletal and haematological abnormalities and bone marrow (BM) dysfunction. Mutations in the SBDS gene cause SDS. Clonal chromosome anomalies are often present in BM, i(7)(q10) and del(20)(q) being the most frequent ones. We collected 6 SDS cases with del(20)(q): a cluster of imprinted genes, including L3MBTL1 and SGK2 is present in the deleted region. Only the paternal allele is expressed for these genes...
August 24, 2016: Genes, Chromosomes & Cancer
Jörg Kleeff, Christian Stöß, Julia Mayerle, Lynne Stecher, Matthias Maak, Peter Simon, Ulrich Nitsche, Helmut Friess
BACKGROUND: If conservative treatment of chronic pancreatitis is unsuccessful, surgery is an option. The choice of the most suitable surgical method can be difficult, as the indications, advantages, and disadvantages of the available methods have not yet been fully documented with scientific evidence. METHODS: In April 2015, we carried out a temporally unlimited systematic search for publications on surgery for chronic pancreatitis. The target parameters were morbidity, mortality, pain, endocrine and exocrine insuffi - ciency, weight gain, quality of life, length of hospital stay, and duration of urgery...
July 25, 2016: Deutsches Ärzteblatt International
Alexander Brock, Ingo Aldag, Stella Edskes, Marcus Hartmann, Torsten Herzog, Waldemar Uhl, Juergen Schnekenburger
AIM AND OBJECTIVES: Exocrine pancreatic insufficiency caused by inflammation or pancreatic tumors results in nutrient malfunction by a lack of digestive enzymes and neutralization compounds. Despite satisfactory clinical results with current enzyme therapies, a normalization of fat absorption in patients is rare. An individualized therapy is required that includes high dosage of enzymatic units, usage of enteric coating, and addition of gastric proton pump inhibitors. The key goal to improve this therapy is to identify digestive enzymes with high activity and stability in the gastrointestinal tract...
November 2016: European Journal of Gastroenterology & Hepatology
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