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Pancreatic insufficiency

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https://www.readbyqxmd.com/read/28337084/therapeutic-drug-monitoring-of-continuous-infusion-doripenem-in-a-pediatric-patient-on-continuous-renal-replacement-therapy
#1
Jeffrey J Cies, Wayne S Moore, Susan B Conley, Paul Shea, Adela Enache, Arun Chopra
An 11-year-old African American male with severe combined immunodeficiency variant, non-cystic fibrosis bronchiectasis, pancreatic insufficiency, chronic mycobacterium avium-intracellulare infection, chronic sinusitis, and malnutrition presented with a 1-week history of fevers. He subsequently developed respiratory decompensation and cefepime was discontinued and doripenem was initiated. Doripenem was the carbapenem used due to a national shortage of meropenem. By day 7 the patient (24.7 kg) had a positive fluid balance of 6925 mL (28% FO), and on days 7 into 8 developed acute kidney injury evidenced by an elevated serum creatinine of 0...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28332731/undiagnosed-pancreatic-exocrine-insufficiency-and-chronic-pancreatitis-in-functional-gi-disorder-patients-with-diarrhea-or-abdominal-pain
#2
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
#3
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
#4
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/28326749/a-case-of-autoimmune-polyendocrine-syndrome-type-i-with-strong-positive-gad-antibody-titer-followed-up-with-glucose-tolerance-measured-by-oral-glucose-tolerance-test
#5
(no author information available yet)
A 26-year-old Japanese woman presented with adrenal insufficiency, and treatment was started with cortisone and fludrocortisone in 1975. A few years later, she presented with hypoparathyroidism and was diagnosed with autoimmune polyendocrine syndrome type I (APS I), and treatment with calcium and alfacalcidol was started. She was found to have subacute thyroiditis and relative adrenal failure in 2006. Her condition remained stable under treatment with cortisone, fludrocortisone, levothyroxine, calcium lactate, precipitated calcium carbonate and alfacalcidol...
January 22, 2017: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28318089/a-cacna1d-mutation-in-a-patient-with-persistent-hyperinsulinaemic-hypoglycaemia-heart-defects-and-severe-hypotonia
#6
S E Flanagan, F Vairo, M B Johnson, R Caswell, T W Laver, H Lango Allen, K Hussain, S Ellard
Congenital hyperinsulinaemic hypoglycaemia (HH) can occur in isolation or it may present as part of a wider syndrome. For approximately 40%-50% of individuals with this condition, sequence analysis of the known HH genes identifies a causative mutation. Identifying the underlying genetic aetiology in the remaining cases is important as a genetic diagnosis will inform on recurrence risk, may guide medical management and will provide valuable insights into β-cell physiology. We sequenced the exome of a child with persistent diazoxide-responsive HH, mild aortic insufficiency, severe hypotonia, and developmental delay as well as the unaffected parents...
March 20, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28315875/systematic-review-of-exocrine-pancreatic-insufficiency-after-gastrectomy-for-cancer
#7
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
#8
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...
March 17, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28303682/ultrastructural-alterations-of-pancreatic-beta-cells-in-human-diabetes-mellitus
#9
Matilde Masini, Luisa Martino, Lorella Marselli, Marco Bugliani, Ugo Boggi, Franco Filipponi, Piero Marchetti, Vincenzo De Tata
AIMS: Both types of diabetes are characterized by beta-cell failure and death, leading to insulin insufficiency. Very limited information is currently available about the ultrastructural alterations of beta cells in human diabetes. Our aim was to provide a comprehensive ultrastructural analysis of human pancreatic islets in type 1 and type 2 diabetic patients. METHODS: We performed a morphometric electron microscopy evaluation of beta cells obtained from the pancreas of 8 non-diabetic (ND), 5 type 1 (T1D) and 8 type 2 (T2D) diabetic organ donors...
March 16, 2017: Diabetes/metabolism Research and Reviews
https://www.readbyqxmd.com/read/28294170/exploring-inter-organ-crosstalk-to-uncover-mechanisms-that-regulate-%C3%AE-cell-function-and-mass
#10
REVIEW
J Shirakawa, D F De Jesus, R N Kulkarni
Impaired β-cell function and insufficient β-cell mass compensation are twin pathogenic features that underlie type 2 diabetes (T2D). Current therapeutic strategies continue to evolve to improve treatment outcomes in different ethnic populations and include approaches to counter insulin resistance and improve β-cell function. Although the effects of insulin secretion on metabolic organs such as liver, skeletal muscle and adipose is directly relevant for improving glucose uptake and reduce hyperglycemia, the ability of pancreatic β-cells to crosstalk with multiple non-metabolic tissues is providing novel insights into potential opportunities for improving β-cell function and/or mass that could have beneficial effects in patients with diabetes...
March 15, 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28291656/diagnosis-of-exocrine-pancreatic-insufficiency-in-chronic-pancreatitis-13-c-mixed-triglyceride-breath-test-versus-fecal-elastase
#11
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
#12
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/28277226/nasal-polyposis-in-cystic-fibrosis-follow-up-of-children-and-adolescents-for-a-3-year-period
#13
Silke Anna Theresa Weber, Renata Mizusaki Iyomasa, Camila de Castro Corrêa, Wellington Novais Mafra Florentino, Giesela Fleischer Ferrari
INTRODUCTION: Nasal polyposis is often found in patients with cystic fibrosis. OBJECTIVE: To assess the incidence of nasal polyposis, the response to medical treatment, recurrence and the need for surgical intervention in children and adolescents with cystic fibrosis during a three-year follow-up. METHODS: Clinical symptoms (pulmonary, pancreatic insufficiency, malnutrition, nasal obstruction), two positive sweat chloride tests, and genotype findings in 23 patients with cystic fibrosis were analyzed...
October 17, 2016: Brazilian Journal of Otorhinolaryngology
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/28254727/predicting-the-incidence-of-portosplenomesenteric-vein-thrombosis-in-patients-with-acute-pancreatitis-using-classification-and-regression-tree-algorithm
#15
Yang Fei, Kun Gao, Jian Hu, Jianfeng Tu, Wei-Qin Li, Wei Wang, Guang-Quan Zong
BACKGROUND AND OBJECTIVE: The accurate prediction of portosplenomesenteric vein thrombosis (PVT) in patients with acute pancreatitis(AP) is very important but may also be difficult because of our insufficient understanding of the characteristics of AP-induced PVT. The purpose of this study is to design a decision tree model that provides critical factors associated with PVT using an approach that makes use of classification and regression tree (CART) algorithm. METHODS: The analysis included 353 patients with AP who were admitted between January 2011 and December 2015...
February 12, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28253873/extended-clinical-features-associated-with-novel-glis3-mutation-a-case-report
#16
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
#17
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/28247763/n-n-dimethyl-tertiary-amino-group-mediated-dual-pancreas-and-lung-targeting-therapy-against-acute-pancreatitis
#18
Shi Luo, Peiwen Li, Sha Li, Zhengwu Du, Xun Hu, Yao Fu, Zhirong Zhang
Acute pancreatitis (AP) is a sudden inflammation of the pancreas with high-mortality rate worldwide. As a severe complication to AP, acute lung injury has been the major cause of death among patients with AP. Poor penetration across the blood pancreas barrier (BPB), and insufficient drug accumulation at the target site often result in poor therapeutic outcome. Our previous work successfully demonstrated a dual-specific targeting strategy to pancreas and lung using a phenolic propanediamine moiety. Inspired by this, a simplified ligand structure, N, N-dimethyl tertiary amino group, was covalently conjugated to celastrol (CLT) to afford tertiary amino conjugates via either an ester (CP) or an amide linkage (CTA)...
March 1, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28246469/restoration-of-energy-level-in-the-early-phase-of-acute-pediatric-pancreatitis
#19
REVIEW
Dóra Mosztbacher, Nelli Farkas, Margit Solymár, Gabriella Pár, Judit Bajor, Ákos Szűcs, József Czimmer, Katalin Márta, Alexandra Mikó, Zoltán Rumbus, Péter Varjú, Péter Hegyi, Andrea Párniczky
Acute pancreatitis (AP) is a serious inflammatory disease with rising incidence both in the adult and pediatric populations. It has been shown that mitochondrial injury and energy depletion are the earliest intracellular events in the early phase of AP. Moreover, it has been revealed that restoration of intracellular ATP level restores cellular functions and defends the cells from death. We have recently shown in a systematic review and meta-analysis that early enteral feeding is beneficial in adults; however, no reviews are available concerning the effect of early enteral feeding in pediatric AP...
February 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28245190/cystic-fibrosis-related-diabetes-cfrd-is-preceded-by-and-associated-with-growth-failure-and-deteriorating-lung-function
#20
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
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