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pancreatic exocrine insufficiency

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https://www.readbyqxmd.com/read/28436541/pdx1-mody-and-dorsal-pancreatic-agenesis-new-phenotype-of-a-rare-disease
#1
L A Caetano, L S Santana, A D Costa-Riquetto, A M Lerario, M Nery, G F Nogueira, C D Ortega, M S Rocha, Aal Jorge, M G Teles
Maturity-Onset Diabetes of the Young (MODY) type 4 or PDX1-MODY is a rare form of monogenic diabetes caused by heterozygous variants in PDX1. Pancreatic developmental anomalies related to PDX1 are reported only in neonatal diabetes cases. Here, we describe dorsal pancreatic agenesis in two patients with PDX1-MODY. The proband presented with diabetes since 14 years of age and maintained regular glycemic control with low doses of basal insulin and detectable C-peptide levels after 30 years with diabetes. A diagnosis of MODY was suspected...
April 24, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28406793/population-level-incidence-and-predictors-of-surgically-induced-diabetes-and-exocrine-insufficiency-after-partial-pancreatic-resection
#2
Irmina A Elliott, Irene Epelboym, Megan Winner, John D Allendorf, Philip I Haigh
CONTEXT: Endocrine and exocrine insufficiency after partial pancreatectomy affect quality of life, cardiovascular health, and nutritional status. However, their incidence and predictors are unknown. OBJECTIVE: To identify the incidence and predictors of new-onset diabetes and exocrine insufficiency after partial pancreatectomy. DESIGN: We retrospectively reviewed 1165 cases of partial pancreatectomy, performed from 1998 to 2010, from a large population-based database...
2017: Permanente Journal
https://www.readbyqxmd.com/read/28405150/comparing-acid-steatocrit-and-faecal-elastase-estimations-for-use-in-m-annheim-staging-for-pancreatitis
#3
M Ganesh Kamath, C Ganesh Pai, Asha Kamath, Annamma Kurien
AIM: To compare two tests for exocrine pancreatic function (EPF) for use in M-ANNHEIM staging for pancreatitis. METHODS: One hundred and ninety four consecutive patients with acute pancreatitis (AP; n = 13), recurrent acute pancreatitis (RAP; n = 65) and chronic pancreatitis (CP; n = 116) were enrolled. EPF was assessed by faecal elastase-1 (FE-1) estimation and stool fat excretion by the acid steatocrit method. Patients were classified as per M-ANNHEIM stages separately based on the results of the two tests for comparison...
March 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28393988/-chronic-pancreatitis-retrospective-review-of-121-cases
#4
Zoltán Berger F, Carla Mancilla A
BACKGROUND: Chronic pancreatitis (CP) is a rare disease in Chile, without a clear explanation for this low prevalence. AIM: To analyze the characteristics of our patients with pancreatitis. MATERIAL AND METHODS: Retrospective analysis of a database of patients with pancreatitis of a clinical hospital. Morphological proof of diagnosis (calcifications/calculi, alterations of ducts, local complication or histology) was obtained for every patient...
December 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28384265/growth-deficits-in-cystic-fibrosis-mice-begin-in-utero-prior-to-igf-1-reduction
#5
Rebecca Darrah, Ilya Bederman, Megan Vitko, Dana M Valerio, Mitchell L Drumm, Craig A Hodges
Growth deficits are common in cystic fibrosis (CF), but their cause is complex, with contributions from exocrine pancreatic insufficiency, pulmonary complications, gastrointestinal obstructions, and endocrine abnormalities. The CF mouse model displays similar growth impairment despite exocrine pancreatic function and in the absence of chronic pulmonary infection. The high incidence of intestinal obstruction in the CF mouse has been suggested to significantly contribute to the observed growth deficits. Previous studies by our group have shown that restoration of the cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium prevents intestinal obstruction but does not improve growth...
2017: PloS One
https://www.readbyqxmd.com/read/28344786/united-european-gastroenterology-evidence-based-guidelines-for-the-diagnosis-and-therapy-of-chronic-pancreatitis-hapaneu
#6
REVIEW
J Matthias Löhr, Enrique Dominguez-Munoz, Jonas Rosendahl, Marc Besselink, Julia Mayerle, Markus M Lerch, Stephan Haas, Fatih Akisik, Nikolaos Kartalis, Julio Iglesias-Garcia, Jutta Keller, Marja Boermeester, Jens Werner, Jean-Marc Dumonceau, Paul Fockens, Asbjorn Drewes, Gürlap Ceyhan, Björn Lindkvist, Joost Drenth, Nils Ewald, Philip Hardt, Enrique de Madaria, Heiko Witt, Alexander Schneider, Riccardo Manfredi, Frøkjer J Brøndum, Sasa Rudolf, Thomas Bollen, Marco Bruno
BACKGROUND: There have been substantial improvements in the management of chronic pancreatitis, leading to the publication of several national guidelines during recent years. In collaboration with United European Gastroenterology, the working group on 'Harmonizing diagnosis and treatment of chronic pancreatitis across Europe' (HaPanEU) developed these European guidelines using an evidence-based approach. METHODS: Twelve multidisciplinary review groups performed systematic literature reviews to answer 101 predefined clinical questions...
March 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28332731/undiagnosed-pancreatic-exocrine-insufficiency-and-chronic-pancreatitis-in-functional-gi-disorder-patients-with-diarrhea-or-abdominal-pain
#7
N J Talley, G Holtmann, N Q Nguyen, P R Gibson, P Bampton, M Veysey, J Wong, S Philcox, N Koloski, L Bunby, M Jones
BACKGROUND & AIMS: A previous UK study showed that 6.1% of patients with diarrhea-predominant IBS (IBS-D) had evidence of severe pancreatic exocrine insufficiency (PEI), but these findings need replication. We aimed to identify the prevalence of PEl based on faecal elastase stool testing in consecutive outpatients presenting with chronic unexplained abdominal pain and/or diarrhea and/or IBS-D. METHODS: Patients aged over 40 years presenting to hospital outpatient clinics from 6 sites within Australia with unexplained abdominal pain and/or diarrhea for at least 3 months and/or IBS-D were studied...
March 23, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28332032/qualitative-assessment-of-the-symptoms-and-impact-of-pancreatic-exocrine-insufficiency-pei-to-inform-the-development-of-a-patient-reported-outcome-pro-instrument
#8
Colin D Johnson, Rob Arbuckle, Nicola Bonner, Gary Connett, Enrique Dominguez-Munoz, Philippe Levy, Doris Staab, Nicola Williamson, Markus M Lerch
BACKGROUND: Pancreatic exocrine insufficiency (PEI) affects patients with chronic pancreatitis (CP) and cystic fibrosis (CF) who produce insufficient digestive pancreatic enzymes. Common symptoms include steatorrhoea, diarrhea, and abdominal pain. OBJECTIVE: The objective of the study was to develop and test the content validity of a patient-reported outcome (PRO) instrument assessing PEI symptoms and their impact on health-related quality of life. METHODS: Instrument development was supported by a literature review, expert physician interviews (n = 10: Germany 4, UK 3, France 3), and exploratory, qualitative, concept-elicitation interviews with patients with CF and CP with PEI (n = 61: UK 29, Germany 18, France 14) and expert physicians (n = 10)...
March 22, 2017: Patient
https://www.readbyqxmd.com/read/28331068/mutations-in-efl1-an-sbds-partner-are-associated-with-infantile-pancytopenia-exocrine-pancreatic-insufficiency-and-skeletal-anomalies-in-a-shwachman-diamond-like-syndrome
#9
Polina Stepensky, Montserrat Chacón-Flores, Katherine H Kim, Omar Abuzaitoun, Arnulfo Bautista-Santos, Natalia Simanovsky, Dritan Siliqi, Davide Altamura, Alfonso Méndez-Godoy, Abril Gijsbers, Adeeb Naser Eddin, Talia Dor, Joel Charrow, Nuria Sánchez-Puig, Orly Elpeleg
BACKGROUND: For the final step of the maturation of the ribosome, the nascent 40S and 60S subunits are exported from the nucleus to the cell cytoplasm. To prevent premature association of these ribosomal subunits, eukaryotic initiation factor 6 (eIF6) binds the 60S subunit within the nucleus. Its release in the cytoplasm requires the interaction of EFL1 and SDBS proteins. In Shwachman-Diamond syndrome (SDS), a defective SDBS protein prevents eIF6 eviction, inhibiting its recycle to the nucleus and subsequent formation of the active 80S ribosome...
March 22, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28315875/systematic-review-of-exocrine-pancreatic-insufficiency-after-gastrectomy-for-cancer
#10
Jennifer Straatman, Jim Wiegel, Nicole van der Wielen, E P Jansma, Miguel A Cuesta, Donald L van der Peet
BACKGROUND: Survival rates after a total gastrectomy with adequate lymphadenectomy are improving, leading to a shift in outcomes of interest from survival to postoperative outcomes and symptoms. In this systematic review, we investigate gastrointestinal symptoms that occur after a gastrectomy in relation to exocrine pancreatic insufficiency and the effect of pancreatic exocrine enzyme supplementation on these symptoms. METHODS: Online databases PubMed, Embase, and Cochrane Library were systematically searched in accordance with the PRISMA guidelines...
March 18, 2017: Digestive Surgery
https://www.readbyqxmd.com/read/28315028/potential-for-screening-for-pancreatic-exocrine-insufficiency-using-the-fecal-elastase-1-test
#11
REVIEW
J Enrique Domínguez-Muñoz, Philip D Hardt, Markus M Lerch, Matthias J Löhr
The early diagnosis of pancreatic exocrine insufficiency (PEI) is hindered because many of the functional diagnostic techniques used are expensive and require specialized facilities, which prevent their widespread availability. We have reviewed current evidence in order to compare the utility of these functional diagnostic techniques with the fecal elastase-1 (FE-1) test in the following three scenarios: screening for PEI in patients presenting with symptoms suggestive of pancreatic disease, such as abdominal pain or diarrhea; determining the presence of PEI in patients with an established diagnosis of pancreatic disease, such as chronic pancreatitis or cystic fibrosis; determining exocrine status in disorders not commonly tested for PEI, but which have a known association with this disorder...
May 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28291656/diagnosis-of-exocrine-pancreatic-insufficiency-in-chronic-pancreatitis-13-c-mixed-triglyceride-breath-test-versus-fecal-elastase
#12
Víctor González-Sánchez, Rahma Amrani, Victoria González, Celia Trigo, Antonio Picó, Enrique de-Madaria
BACKGROUND: Pancreatic enzyme replacement therapy (PERT) is indicated in case of clinically relevant exocrine pancreatic insufficiency (EPI). Clinical trials addressing PERT have used the coefficient of fat absorption (CFA) to define EPI but this test is cumbersome to perform. Our aim was to compare two easier-to-perform tests to detect clinically relevant EPI: Fecal Elastase-1 (FE-1) and (13)C-Mixed Triglyceride Breath Test (TGBT). METHODS: We prospectively included 54 patients with chronic pancreatitis (CP), 24...
March 6, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28281169/complications-of-chronic-pancreatitis
#13
REVIEW
Mitchell L Ramsey, Darwin L Conwell, Phil A Hart
Chronic pancreatitis is a disease that leads to irreversible changes in the pancreatic morphology and function. The loss of function can lead to diabetes mellitus and exocrine pancreatic insufficiency. The inflammation and fibrosis can also lead to other complications including a chronic abdominal pain syndrome, metabolic bone disease, and pancretic cancer. This article reviews our current understanding of the mechanisms and management of these complications of chronic pancreatitis.
March 9, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28272344/nutritional-and-metabolic-derangements-in-pancreatic-cancer-and-pancreatic-resection
#14
REVIEW
Taylor M Gilliland, Nicole Villafane-Ferriol, Kevin P Shah, Rohan M Shah, Hop S Tran Cao, Nader N Massarweh, Eric J Silberfein, Eugene A Choi, Cary Hsu, Amy L McElhany, Omar Barakat, William Fisher, George Van Buren
Pancreatic cancer is an aggressive malignancy with a poor prognosis. The disease and its treatment can cause significant nutritional impairments that often adversely impact patient quality of life (QOL). The pancreas has both exocrine and endocrine functions and, in the setting of cancer, both systems may be affected. Pancreatic exocrine insufficiency (PEI) manifests as weight loss and steatorrhea, while endocrine insufficiency may result in diabetes mellitus. Surgical resection, a central component of pancreatic cancer treatment, may induce or exacerbate these dysfunctions...
March 7, 2017: Nutrients
https://www.readbyqxmd.com/read/28253873/extended-clinical-features-associated-with-novel-glis3-mutation-a-case-report
#15
K A Alghamdi, A B Alsaedi, A Aljasser, A Altawil, Naglaa M Kamal
BACKGROUND: Mutations in the GLI-similar 3 (GLIS3) gene encoding the transcription factor GLIS3 are a rare cause of neonatal diabetes and congenital hypothyroidism with 12 reported patients to date. Additional features, previously described, include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay, facial dysmorphism, osteopenia, sensorineural deafness, choanal atresia, craniosynostosis and pancreatic exocrine insufficiency. CASE PRESENTATION: We report a new case for consanguineous parents with homozygous novel mutation in GLIS3 gene who presented with neonatal diabetes mellitus, severe resistant congenital hypothyroidism, cholestatic liver disease, bilateral congenital glaucoma and facial dysmorphism...
March 2, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28248878/surgical-strategies-in-the-treatment-of-chronic-pancreatitis-an-updated-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#16
REVIEW
Xin Zhao, Naiqiang Cui, Ximo Wang, Yunfeng Cui
BACKGROUND: Chronic pancreatitis (CP) is a common and frequently occurring disease. Pancreaticoduodenectomy (PD), pylorus-preserving pancreaticoduodenectomy (PPPD), and duodenum-preserving pancreatic head resection (DPPHR) are important treatment options for patients with chronic pancreatitis. The Beger and Frey procedures are 2 main duodenum-preserving techniques in duodenum-preserving pancreatic head resection (DPPHR) strategies. We conducted this systematic review and meta-analysis to compare the clinical efficacy of DPPHR versus PD, the Beger procedure versus PD, the Frey procedure versus PD, and the Beger procedure versus the Frey procedure in the treatment of pancreatitis...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28245190/cystic-fibrosis-related-diabetes-cfrd-is-preceded-by-and-associated-with-growth-failure-and-deteriorating-lung-function
#17
Nicolas Terliesner, Mandy Vogel, Anna Steighardt, Ruth Gausche, Constance Henn, Julia Hentschel, Thomas Kapellen, Sabine Klamt, Julia Gebhardt, Wieland Kiess, Freerk Prenzel
BACKGROUND: Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents. METHODS: A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed...
February 28, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28244497/quality-of-life-in-chronic-pancreatitis-is-determined-by-constant-pain-disability-unemployment-current-smoking-and-associated-co-morbidities
#18
Jorge D Machicado, Stephen T Amann, Michelle A Anderson, Judah Abberbock, Stuart Sherman, Darwin L Conwell, Gregory A Cote, Vikesh K Singh, Michele D Lewis, Samer Alkaade, Bimaljit S Sandhu, Nalini M Guda, Thiruvengadam Muniraj, Gong Tang, John Baillie, Randall E Brand, Timothy B Gardner, Andres Gelrud, Christopher E Forsmark, Peter A Banks, Adam Slivka, C Mel Wilcox, David C Whitcomb, Dhiraj Yadav
OBJECTIVES: Chronic pancreatitis (CP) has a profound independent effect on quality of life (QOL). Our aim was to identify factors that impact the QOL in CP patients. METHODS: We used data on 1,024 CP patients enrolled in the three NAPS2 studies. Information on demographics, risk factors, co-morbidities, disease phenotype, and treatments was obtained from responses to structured questionnaires. Physical and mental component summary (PCS and MCS, respectively) scores generated using responses to the Short Form-12 (SF-12) survey were used to assess QOL at enrollment...
April 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28241470/pancreatic-exocrine-insufficiency-in-pancreatic-cancer
#19
REVIEW
Miroslav Vujasinovic, Roberto Valente, Marco Del Chiaro, Johan Permert, J-Matthias Löhr
Abstract: Cancer patients experience weight loss for a variety of reasons, commencing with the tumor's metabolism (Warburg effect) and proceeding via cachexia to loss of appetite. In pancreatic cancer, several other factors are involved, including a loss of appetite with a particular aversion to meat and the incapacity of the pancreatic gland to function normally when a tumor is present in the pancreatic head. Pancreatic exocrine insufficiency is characterized by a deficiency of the enzymes secreted from the pancreas due to the obstructive tumor, resulting in maldigestion...
February 23, 2017: Nutrients
https://www.readbyqxmd.com/read/28223257/the-fecal-microbiome-of-dogs-with-exocrine-pancreatic-insufficiency
#20
Anitha Isaiah, Joseph Cyrus Parambeth, Jörg M Steiner, Jonathan A Lidbury, Jan S Suchodolski
Exocrine pancreatic insufficiency (EPI) in dogs is a syndrome of inadequate synthesis and secretion of pancreatic enzymes. Small intestinal bacterial dysbiosis occurs in dogs with EPI, and is reversed with pancreatic enzyme therapy. However, there are no studies evaluating the fecal microbiome of dogs with EPI. The objective of this study was to evaluate the fecal microbiome of dogs with EPI. Three day pooled fecal samples were collected from healthy dogs (n = 18), untreated (n = 7) dogs with EPI, and dogs with EPI treated with enzyme replacement therapy (n = 19)...
February 18, 2017: Anaerobe
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