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Cystic fibrosis related diabetes

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https://www.readbyqxmd.com/read/28431569/co-colonisation-with-aspergillus-fumigatus-and-pseudomonas-aeruginosa-is-associated-with-poorer-health-in-cystic-fibrosis-patients-an-irish-registry-analysis
#1
Emma Reece, Ricardo Segurado, Abaigeal Jackson, Siobhán McClean, Julie Renwick, Peter Greally
BACKGROUND: Pulmonary infection is the main cause of death in cystic fibrosis (CF). Aspergillus fumigatus (AF) and Pseudomonas aeruginosa (PA) are the most prevalent fungal and bacterial pathogens isolated from the CF airway, respectively. Our aim was to determine the effect of different colonisation profiles of AF and PA on the clinical status of patients with CF. METHODS: A retrospective analysis of data from the Cystic Fibrosis Registry of Ireland from 2013 was performed to determine the effect of intermittent and persistent colonisation with AF or PA or co-colonisation with both microorganisms on clinical outcome measures in patients with CF...
April 21, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28273890/cftr-is-involved-in-the-regulation-of-glucagon-secretion-in-human-and-rodent-alpha-cells
#2
Anna Edlund, Morten Gram Pedersen, Andreas Lindqvist, Nils Wierup, Malin Flodström-Tullberg, Lena Eliasson
Glucagon is the main counterregulatory hormone in the body. Still, the mechanism involved in the regulation of glucagon secretion from pancreatic alpha cells remains elusive. Dysregulated glucagon secretion is common in patients with Cystic Fibrosis (CF) that develop CF related diabetes (CFRD). CF is caused by a mutation in the Cl(-) channel Cystic fibrosis transmembrane conductance regulator (CFTR), but whether CFTR is present in human alpha cells and regulate glucagon secretion has not been investigated in detail...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28254466/impaired-rib-bone-mass-and-quality-in-end-stage-cystic-fibrosis-patients
#3
Geneviève Mailhot, Natalie Dion, Delphine Farlay, Sébastien Rizzo, Nathalie J Bureau, Valérie Jomphe, Safiétou Sankhe, Georges Boivin, Larry C Lands, Pasquale Ferraro, Louis-Georges Ste-Marie
BACKGROUND: Advancements in research and clinical care have considerably extended the life expectancy of cystic fibrosis (CF) patients. However, with this extended survival come comorbidities. One of the leading co-morbidities is CF-related bone disease (CFBD), which progresses with disease severity and places patients at high risk for fractures, particularly of the ribs and vertebrae. Evidence that CF patients with vertebral fractures had higher bone mineral density (BMD) than the nonfracture group led us to postulate that bone quality is impaired in these patients...
May 2017: Bone
https://www.readbyqxmd.com/read/28245190/cystic-fibrosis-related-diabetes-cfrd-is-preceded-by-and-associated-with-growth-failure-and-deteriorating-lung-function
#4
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/28213469/insulin-signaling-via-the-pi3k-akt-pathway-regulates-airway-glucose-uptake-and-barrier-function-in-a-cftr-dependent-manner
#5
Samuel A Molina, Hannah K Moriarty, Danny T Infield, Barry R Imhoff, Rachel J Vance, Agnes H Kim, Jason M Hansen, William R Hunt, Michael Koval, Nael A McCarty
Cystic fibrosis-related diabetes (CFRD) is the most common co-morbidity associated with cystic fibrosis (CF) and correlates with increased rates of lung function decline. Since glucose is a nutrient present in the airways of patients with bacterial airway infections and since insulin controls glucose metabolism, the effect of insulin on CF airway epithelia was investigated to determine the role of insulin receptors and glucose transport in regulating glucose availability in the airway. The response to insulin by human airway epithelial cells was characterized by qPCR, immunoblot, immunofluorescence, and glucose uptake assays...
February 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28167560/nephrotoxicity-in-patients-with-or-without-cystic-fibrosis-treated-with-polymyxin-b-compared-to-colistin
#6
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 of 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 h was conducted. The primary endpoint was the incidence of kidney injury assessed by RIFLE (i.e., risk, injury, failure, loss, end-stage renal disease) criteria. Risk factors for kidney injury were evaluated using multivariate Cox regression...
April 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
#7
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
#8
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/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#9
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/28012584/severe-disease-in-cystic-fibrosis-and-fecal-calprotectin-levels
#10
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...
March 2017: 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
#11
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
#12
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/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/27752941/a-practical-approach-to-glucose-abnormalities-in-cystic-fibrosis
#17
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
https://www.readbyqxmd.com/read/27748026/a-description-of-clinician-reported-diagnosis-of-type-2-diabetes-and-other-non-type-1-diabetes-included-in-a-large-international-multicentered-pediatric-diabetes-registry-sweet
#18
Danièle Pacaud, Anke Schwandt, Carine de Beaufort, Kristina Casteels, Jacques Beltrand, Niels H Birkebaek, Myrna Campagnoli, Natasa Bratina, Catarina Limbert, Stephen Mp O'Riordan, Rogério Ribeiro, Andriani Gerasimidi-Vazeou, Lenka Petruzelkova, Rasa Verkauskiene, Iveta Dzivite Krisane
BACKGROUND: Although type 1 diabetes (T1D) remains the most frequent form of diabetes in individuals aged less than 20 years at onset, other forms of diabetes are being increasingly recognized. OBJECTIVES: To describe the population of children with other forms of diabetes (non-type 1) included in the multinational SWEET (Better control in Pediatric and Adolescent diabeteS: Working to crEate CEnTers of Reference) database for children with diabetes. METHODS: Cases entered in the SWEET database are identified by their physician as T1D, type 2 diabetes (T2D) and other types of diabetes according to the ISPAD classification...
October 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27737759/screening-for-cystic-fibrosis-related-diabetes-matching-pathophysiology-and-addressing-current-challenges
#19
REVIEW
Valérie Boudreau, Quitterie Reynaud, Catherine Lehoux Dubois, Adèle Coriati, Katherine Desjardins, Isabelle Durieu, Rémi Rabasa-Lhoret
Nearly 50% of adult patients with cystic fibrosis (CF) have diabetes. The occurrence of CF-related diabetes (CFRD) is preceded and is associated with deterioration of lung function and nutritional status. Microvascular complications can occur, but the main cause of death is respiratory failure rather than cardiovascular causes as in type 1 or type 2 diabetes. Because other methods such as glycated hemoglobin (A1C) levels are less sensitive in patients with CF, the recommended screening test is the oral glucose tolerance test (OGTT) with a 75 g glucose dose...
October 2016: Canadian Journal of Diabetes
https://www.readbyqxmd.com/read/27709245/cystic-fibrosis-a-clinical-view
#20
REVIEW
Carlo Castellani, Baroukh M Assael
Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude...
January 2017: Cellular and Molecular Life Sciences: CMLS
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