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Cystic fibrosis diabetes

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https://www.readbyqxmd.com/read/28196020/the-prevalence-and-clinicopathological-characteristics-of-high-grade-pancreatic-intraepithelial-neoplasia-autopsy-study-evaluating-the-entire-pancreatic-parenchyma
#1
Yoko Matsuda, Toru Furukawa, Shinichi Yachida, Makoto Nishimura, Atsuko Seki, Keisuke Nonaka, Junko Aida, Kaiyo Takubo, Toshiyuki Ishiwata, Wataru Kimura, Tomio Arai, Mari Mino-Kenudson
OBJECTIVE: We sought to identify clinicopathological characteristics of high-grade pancreatic intraepithelial neoplasia (PanIN)/carcinoma in situ to facilitate screening for pancreatic ductal adenocarcinoma. METHODS: We evaluated PanIN lesions in 173 consecutive autopsy cases with no evidence of pancreatic ductal adenocarcinoma and/or intraductal papillary mucinous neoplasm (mean age, 80.5 years) by submitting the entire pancreas for microscopic examination. RESULTS: PanIN-3 was found in 4% of examined cases, whereas PanIN-1 and PanIN-2 were present in 77% and 28%, respectively...
February 14, 2017: Pancreas
https://www.readbyqxmd.com/read/28167560/nephrotoxicity-in-patients-with-and-without-cystic-fibrosis-treated-with-polymyxin-b-compared-to-colistin
#2
Ryan L Crass, W Cliff Rutter, Donna R Burgess, Craig A Martin, David S Burgess
Nephrotoxicity is the primary adverse effect of the polymyxins. The relative rates of toxicity between polymyxin B and colistin have not been fully elucidated, especially in patients with cystic fibrosis (CF). A retrospective cohort study of adults treated with polymyxin B or colistin for at least 48 hours was conducted. The primary endpoint was the incidence of kidney injury by RIFLE (i.e., Risk, Injury, Failure, Loss, End-Stage Renal Disease) criteria. Risk factors for kidney injury were evaluated using multivariate Cox regression...
February 6, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28164596/genetic-expression-in-cystic-fibrosis-related-bone-disease-an-observational-transversal-cross-sectional-study
#3
Ioana M Ciuca, Liviu L Pop, Alexandru F Rogobete, Dan I Onet, Bogdan Guta-Almajan, Zoran Popa, Florin G Horhat
BACKGROUND: Cystic fibrosis (CF) is the most frequent monogenic genetic disease with autosomal recessive transmission and characterized by important clinical polymorphism and significant lethal prospective. CF related bone disease occurs frequently in adults with CF. Childhood is the period of bone formation, and therefore, children are more susceptible to low bone density. Several factors like pancreatic insufficiency, hormone imbalance, and physical inactivity contribute to CF bone disease development...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28115168/heterogeneity-in-survival-among-adult-cystic-fibrosis-patients-with-fev1-30-of-predicted-in-the-united-states
#4
Kathleen J Ramos, Bradley S Quon, Sonya L Heltshe, Nicole Mayer-Hamblett, Erika D Lease, Moira L Aitken, Noel S Weiss, Christopher H Goss
BACKGROUND: Lung transplantation (LTx) is frequently considered for patients with cystic fibrosis (CF) when FEV1 reaches <30%. This study estimated transplant-free survival for CF patients and FEV1 <30% and identified predictors of death without LTx. METHODS: We conducted a retrospective cohort study using the CF Foundation Patient Registry, 1/1/2003-12/31/2013. Adult patients (≥18 years) with FEV1 <30% prior to LTx were included. We performed Kaplan-Meier survival estimates censored at LTx...
January 20, 2017: Chest
https://www.readbyqxmd.com/read/28106773/determinants-of-serum-glycerophospholipid-fatty-acids-in-cystic-fibrosis
#5
Sławomira Drzymała-Czyż, Patrycja Krzyżanowska, Berthold Koletzko, Jan Nowak, Anna Miśkiewicz-Chotnicka, Jerzy A Moczko, Aleksandra Lisowska, Jarosław Walkowiak
The etiology of altered blood fatty acid (FA) composition in cystic fibrosis (CF) is understood only partially. We aimed to investigate the determinants of serum glycerophospholipids' FAs in CF with regard to the highest number of FAs and in the largest cohort to date. The study comprised 172 CF patients and 30 healthy subjects (HS). We assessed Fas' profile (gas chromatography/mass spectrometry), CF transmembrane conductance regulator (CFTR) genotype, spirometry, fecal elastase-1, body height and weight Z-scores, liver disease, diabetes and colonization by Pseudomonas aeruginosa...
January 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#6
REVIEW
Aisha A Aslam, Colin Higgins, Ian P Sinha, Kevin W Southern
BACKGROUND: Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein. This protein coordinates the transport of salt (and bicarbonate) across cell surfaces and the mutation most notably affects the airways. In the lungs of people with cystic fibrosis, defective protein results in a dehydrated surface liquid and compromised mucociliary clearance...
January 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28018696/alagille-syndrome-a-case-report-highlighting-dysmorphic-facies-chronic-illness-and-depression
#7
James J Bresnahan, Zachary A Winthrop, Rabia Salman, Salman Majeed
Alagille syndrome is a rare multisystem disorder affecting the liver, heart, vertebrae, eyes, and face. Alagille syndrome shares multiple phenotypic variants of other congenital or chronic childhood illnesses such as DiGeorge syndrome, Down syndrome, spina bifida, type 1 diabetes mellitus, and cystic fibrosis. All of these chronic illnesses have well-established links to psychiatric conditions. There are few community resources for Alagille patients, as it is an extremely rare condition. Despite the overlap with other chronic childhood illnesses, the psychiatric manifestations of Alagille syndrome have not been previously discussed in literature...
2016: Case Reports in Psychiatry
https://www.readbyqxmd.com/read/28012584/severe-disease-in-cystic-fibrosis-and-fecal-calprotectin-levels
#8
Giuseppe Fabio Parisi, Maria Papale, Novella Rotolo, Donatella Aloisio, Lucia Tardino, Maria Grazia Scuderi, Vincenzo Di Benedetto, Raffaella Nenna, Fabio Midulla, Salvatore Leonardi
Fecal calprotectin (FC) is used to asses the presence of intestinal inflammation also in patients with Cystic Fibrosis (CF) and recent studies showed a correlation between bowel and lung disease in these patients. The aim of this study was to analyze the levels of FC in CF and correlate them with different phenotypes of disease. We enrolled a cohort of 54 CF patients and 50 healthy controls. In these patients, calprotectin has been assayed on a stools sample using an ELISA kit. In all patients we analyzed, FC levels were elevated above the cut-off value and significantly higher than in healthy controls...
November 13, 2016: Immunobiology
https://www.readbyqxmd.com/read/27999822/glucose-200%C3%A2-mg-dl-during-continuous-glucose-monitoring-identifies-adult-patients-at-risk-for-development-of-cystic-fibrosis-related-diabetes
#9
J L Taylor-Cousar, J S Janssen, A Wilson, C G St Clair, K M Pickard, M C Jones, S J Brayshaw, C S Chacon, C M Barboa, M K Sontag, F J Accurso, D P Nichols, M T Saavedra, J A Nick
Rationale. Cystic fibrosis related diabetes (CFRD) is the most common comorbidity in patients with CF. In spite of increased screening, diagnosis, and treatment of CFRD, the mortality rate in patients with CFRD still far exceeds the mortality rate in those without CFRD. Guidelines suggest that screening for CFRD be performed annually using the 2-hour 75-gram oral glucose tolerance test (OGTT). Adherence to recommended screening has been poor, with only approximately one-quarter of adults with CF undergoing OGTT in 2014...
2016: Journal of Diabetes Research
https://www.readbyqxmd.com/read/27977404/sensitivity-and-specificity-of-different-methods-for-cystic-fibrosis-related-diabetes-screening-is-the-oral-glucose-tolerance-test-still-the-standard
#10
Catherine Mainguy, Gabriel Bellon, Véronique Delaup, Tiphanie Ginoux, Behrouz Kassai-Koupai, Stéphane Mazur, Muriel Rabilloud, Laurent Remontet, Philippe Reix
BACKGROUND: Cystic fibrosis-related diabetes (CFRD) is a late cystic fibrosis (CF)-associated comorbidity whose prevalence is increasing sharply lifelong. Guidelines for glucose metabolism (GM) monitoring rely on the oral glucose tolerance test (OGTT). However, this test is neither sensitive nor specific. The aim of this study was to compare sensitivity and specificity of different methods for GM monitoring in children and adolescents with CF. METHODS: Continuous glucose monitoring system (CGMS), used as the reference method, was compared with the OGTT, intravenous glucose tolerance test (IGTT), homeostasis model assessment index of insulin resistance (HOMA-IR), homeostasis model assessment index of β-cell function (HOMA-%B) and glycated haemoglobin A1C...
January 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27940347/proteasome-inhibitors-in-cancer-therapy-treatment-regimen-and-peripheral-neuropathy-as-a-side-effect
#11
REVIEW
Gulce Sari Kaplan, Ceyda Corek Torcun, Tilman Grune, Nesrin Kartal Ozer, Betul Karademir
Proteasomal system plays an important role in protein turnover, which is essential for homeostasis of cells. Besides degradation of oxidized proteins, it is involved in the regulation of many different signaling pathways. These pathways include mainly cell differentiation, proliferation, apoptosis, transcriptional activation and angiogenesis. Thus, proteasomal system is a crucial target for treatment of several diseases including neurodegenerative diseases, cystic fibrosis, atherosclerosis, autoimmune diseases, diabetes and cancer...
December 8, 2016: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/27934649/safety-and-effectiveness-of-arginine-in-adults
#12
REVIEW
Catherine J McNeal, Cynthia J Meininger, Deepika Reddy, Colin D Wilborn, Guoyao Wu
l-Arginine (Arg) appears to have a beneficial effect on the regulation of nutrient metabolism to enhance lean tissue deposition and on insulin resistance in humans. The observed safe level for oral administration of Arg is ∼20 g/d, but higher levels have been tested in short-term studies without serious adverse effects; however, more data are needed in both animal models and humans to fully evaluate safety as well as efficacy. The primary objective of this review is to summarize the current knowledge of the safety, pharmacokinetics, and effectiveness of oral Arg in adults...
December 2016: Journal of Nutrition
https://www.readbyqxmd.com/read/27902695/text-mining-genotype-phenotype-relationships-from-biomedical-literature-for-database-curation-and-precision-medicine
#13
Ayush Singhal, Michael Simmons, Zhiyong Lu
The practice of precision medicine will ultimately require databases of genes and mutations for healthcare providers to reference in order to understand the clinical implications of each patient's genetic makeup. Although the highest quality databases require manual curation, text mining tools can facilitate the curation process, increasing accuracy, coverage, and productivity. However, to date there are no available text mining tools that offer high-accuracy performance for extracting such triplets from biomedical literature...
November 2016: PLoS Computational Biology
https://www.readbyqxmd.com/read/27897253/hyperglycaemia-and-pseudomonas-aeruginosa-acidify-cystic-fibrosis-airway-surface-liquid-by-elevating-epithelial-monocarboxylate-transporter-2-dependent-lactate-h-secretion
#14
James Peter Garnett, Kameljit K Kalsi, Mirko Sobotta, Jade Bearham, Georgina Carr, Jason Powell, Malcolm Brodlie, Christopher Ward, Robert Tarran, Deborah L Baines
The cystic fibrosis (CF) airway surface liquid (ASL) provides a nutrient rich environment for bacterial growth including elevated glucose, which together with defective bacterial killing due to aberrant HCO3(-) transport and acidic ASL, make the CF airways susceptible to colonisation by respiratory pathogens such as Pseudomonas aeruginosa. Approximately half of adults with CF have CF related diabetes (CFRD) and this is associated with increased respiratory decline. CF ASL contains elevated lactate concentrations and hyperglycaemia can also increase ASL lactate...
November 29, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27880745/announcement-national-family-history-day-november-24-2016
#15
(no author information available yet)
In 2004, the U.S. Surgeon General declared that Thanksgiving would be National Family History Day, a day designed to encourage American families to learn about and create a written record of their family health history. Family history can identify those persons with a higher-than-average risk for many common diseases, such as heart disease, cancer, and type 2 diabetes. Having at least one first-degree relative with a disease can increase a person's risk twofold or more (1). Family history is also a determinant of less common diseases like sickle cell disease and cystic fibrosis (1)...
November 25, 2016: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/27873431/hypoglycemia-is-common-in-children-with-cystic-fibrosis-and-seen-predominantly-in-females
#16
Belma Haliloglu, Yasemin Gokdemir, Zeynep Atay, Saygin Abali, Tulay Guran, Fazilet Karakoc, Refika Ersu, Bulent Karadag, Serap Turan, Abdullah Bereket
OBJECTIVE: To determine the prevalence of hypoglycemia in children and adolescents with cystic fibrosis (CF) in 2-hour oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) under free-living conditions. RESEARCH DESIGN AND METHODS: Height, weight, body mass index (BMI), hemoglobin A1c (HbA1c), and Forced expiratory volume (FEV1%) were measured in children with CF (aged 5-18 years). Following OGTT, CGM was installed for 3 days. The total hypoglycemic and hyperglycemic time (%) during 3 days was measured...
November 22, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27789099/1-carbon-cycle-metabolites-methylate-their-way-to-fatty-liver
#17
REVIEW
Amy Karol Walker
Fatty liver is a complex disease often accompanying metabolic syndrome and Type 2 diabetes mellitus (T2DM). Hepatosteatosis may have roots in multiple metabolic abnormalities. However, metabolic dysfunction in the 1-carbon cycle (1CC), which produces the methyl donor S-adenosylmethionine (SAM) and phosphatidylcholine (PC), induces hepatic lipogenesis in model systems. Human diseases where 1CC or PC synthesis is disrupted, such as alcoholism, congenital lipodystrophy, or cystic fibrosis, often present with fatty liver...
January 2017: Trends in Endocrinology and Metabolism: TEM
https://www.readbyqxmd.com/read/27784241/a-review-on-the-pharmacology-and-toxicology-of-steviol-glycosides-extracted-from-stevia-rebaudiana
#18
Amir Abbas Momtazi-Borojeni, Seyed-Alireza Esmaeili, Elham Abdollahi, Amirhossein Sahebkar
Stevia rebaudiana Bertoni is a sweet and nutrient-rich plant belonging to the Asteraceae family. Stevia leaves contain steviol glycosides including stevioside, rebaudioside (A to F), steviolbioside, and isosteviol, which are responsible for the plant's sweet taste, and have commercial value all over the world as a sugar substitute in foods, beverages and medicines. Among the various steviol glycosides, stevioside, rebaudioside A and rebaudioside C are the major metabolites and these compounds are on average 250-300 times sweeter than sucrose...
October 21, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27753002/clinical-determinants-of-target-non-attainment-of-linezolid-in-plasma-and-interstitial-space-fluid-a-pooled-population-pharmacokinetic-analysis-with-focus-on-critically-ill-patients
#19
Iris K Minichmayr, André Schaeftlein, Joseph L Kuti, Markus Zeitlinger, Charlotte Kloft
OBJECTIVES: We aimed to assess linezolid pharmacokinetics in the plasma and interstitial space fluid (ISF) of patients with sepsis, diabetic foot infections or cystic fibrosis and healthy volunteers. The impacts of joint characteristics and disease on plasma and target-site exposure were to be identified together with the benefit of dose intensification in critically ill patients. METHODS: Rich plasma (n = 1598) and ISF concentrations in subcutaneous adipose (n = 1430) and muscle tissue (n = 1089) measured by microdialysis were pooled from three clinical trials with 51 individuals receiving 600 mg of intravenous and oral linezolid...
October 17, 2016: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/27752941/a-practical-approach-to-glucose-abnormalities-in-cystic-fibrosis
#20
REVIEW
Gregory C Jones, Christopher A R Sainsbury
Cystic fibrosis is a common genetic condition and abnormal glucose handling leading to cystic fibrosis-related diabetes (CFRD) is a frequent comorbidity. CFRD is mainly thought to be the result of progressive pancreatic damage resulting in beta cell dysfunction and loss of insulin secretion. Whilst Oral Glucose Tolerance Testing is still recommended for diagnosing CFRD, the relationship between glucose abnormalities and adverse outcomes in CF is complex and occurs at stages of dysglycaemia occurring prior to diagnosis of diabetes by World Health Organisation criteria...
December 2016: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
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