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Cystic fibrosis related diabetes

Kayani Kayani, Raihan Mohammed, Hasan Mohiaddin
Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian populations. Individuals with CF have seen significant increases in life expectancy in the last 60 years. As a result, previously rare complications are now coming to light. The most common of these is cystic fibrosis-related diabetes (CFRD), which affects 40-50% of CF adults. CFRD significantly impacts the pulmonary function and longevity of CF patients, yet a lack of consensus on the best methods to diagnose and treat CFRD remains...
2018: Frontiers in Endocrinology
Barbara Giordani, Annalisa Amato, Fabio Majo, Gianluca Ferrari, Serena Quattrucci, Laura Minicucci, Rita Padoan, Giovanna Floridia, Gianna Puppo Fornaro, Domenica Taruscio, Marco Salvatore
INTRODUCTION: The Italian Cystic Fibrosis Registry (ICFR) is based on a new agreement about the data flow towards the Registry signed on October, 4th 2016 by the Centre for Rare Diseases of the Italian National Institute of Health (NIH), the clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, the Paediatric Hospital "Bambino Gesù" (Rome), the Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis. OBJECTIVES: The aim of the present Report is to improve the knowledge on cystic fibrosis (CF) through the epidemiological description of Italian patients...
January 2018: Epidemiologia e Prevenzione
Jenny H Conviser, Sheehan D Fisher, Susanna A McColley
OBJECTIVE: Pediatric chronic illnesses (CI) can affect a child's mental health. Chronic illnesses with treatment regimens that specify a therapeutic diet may place the child at increased risk for disordered eating and specific eating disorders (ED). The aim of this review is to examine the relation between diet-treated CI and disordered eating and to determine the order of onset to infer directionality. Diet-treated CI is hypothesized to precede and to be associated with disordered eating...
February 22, 2018: International Journal of Eating Disorders
Rebecca L Hull, Ronald L Gibson, Sharon McNamara, Gail H Deutsch, Corinne L Fligner, Charles W Frevert, Bonnie W Ramsey, Srinath Sanda
OBJECTIVE: Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis (CF), increasing patient morbidity and mortality. Poor understanding of CFRD pathogenesis limits the development of targeted therapies to treat and/or prevent the disease. The aim of this study was to evaluate islet pathology, specifically, inflammation, amyloid deposition, and endocrine cell composition in subjects with CF with diabetes and with CF without diabetes. RESEARCH DESIGN AND METHODS: A retrospective analysis of archived pancreas tissue collected at autopsy was conducted using pancreas tissue from subjects with CF and diabetes (CFRD) ( n = 18) and CF without diabetes (CF-no DM) ( n = 17)...
February 1, 2018: Diabetes Care
Fares Ayoub, Cesar Trillo-Alvarez, Giuseppe Morelli, Jorge Lascano
AIM: To investigate the clinical, biochemical and imaging characteristics of adult cystic fibrosis (CF) patients with hepatic steatosis as compared to normal CF controls. METHODS: We performed a retrospective review of adult CF patients in an academic outpatient setting during 2016. Baseline characteristics, genetic mutation analysis as well as laboratory values were collected. Abdominal imaging (ultrasound, computed tomography, magnetic resonance) was used to determine presence of hepatic steatosis...
January 27, 2018: World Journal of Hepatology
Pavana G Rotti, Weiliang Xie, Ananta Poudel, Yaling Yi, Xingshen Sun, Scott R Tyler, Aliye Uc, Andrew W Norris, Manami Hara, John F Engelhardt, Katherine N Gibson-Corley
In cystic fibrosis (CF), there is early destruction of the exocrine pancreas, and this results in a unique form of diabetes that affects approximately half of adult CF individuals. An animal model of cystic fibrosis-related diabetes has been developed in the ferret, which progresses through phases of glycemic abnormalities because of islet remodeling during and after exocrine destruction. Herein, we quantified the pancreatic histopathological changes that occur during these phases. There was an increase in percentage ductal, fat, and islet area in CF ferrets over time compared with age-matched wild-type controls...
January 31, 2018: American Journal of Pathology
Rossa Brugha, Marie Wright, Suzie Nolan, Nicola Bridges, Siobhán B Carr
BACKGROUND: Cystic fibrosis related diabetes (CFRD) is associated with increased morbidity in CF. Variability in physiological systems is associated with dysfunctional homeostasis. We examined whether fluctuation in glucose is a marker of CFRD or "pre-diabetes". METHODS: Using a machine learning approach, we compared glucose IQR to current diagnostic criteria in a review of continuous glucose monitoring data. RESULTS: Analysis was performed on 248 studies from 142 children...
January 9, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
E Molina-Montes, P Gomez-Rubio, M Márquez, M Rava, M Löhr, C W Michalski, X Molero, A Farré, J Perea, W Greenhalf, L Ilzarbe, M O'Rorke, A Tardón, T Gress, V M Barberà, T Crnogorac-Jurcevic, E Domínguez-Muñoz, L Muñoz-Bellvís, J Balsells, E Costello, J Huang, M Iglesias, J Kleeff, Bo Kong, J Mora, L Murray, D O'Driscoll, I Poves, A Scarpa, W Ye, M Hidalgo, L Sharp, A Carrato, F X Real, N Malats
Background: Family history (FH) of pancreatic cancer (PC) has been associated with an increased risk of PC, but little is known regarding the role of inherited/environmental factors or that of FH of other comorbidities in PC risk. We aimed to address these issues using multiple methodological approaches. Methods: Case-control study including 1431 PC cases and 1090 controls and a reconstructed-cohort study (N = 16 747) made up of their first-degree relatives (FDR)...
January 10, 2018: International Journal of Epidemiology
Jan C Thomassen, Matthias I Mueller, Miguel A Alejandre Alcazar, Ernst Rietschel, Silke van Koningsbruggen-Rietschel
OBJECTIVE: To investigate the effect of Lumacaftor/Ivacaftor on glucose metabolism and insulin secretion in patients with cystic fibrosis (CF) (Phe508del/Phe508del). METHODS: A standard oral glucose tolerance test (OGTT) and an intravenous glucose tolerance test (IVGTT) were performed to investigate glucose metabolism and insulin secretion before and after 6-8weeks of treatment with Lumacaftor/Ivacaftor in 5 Phe508del-homozygous CF patients. The area under the curve (AUC) for glucose and insulin levels was calculated using the trapezoidal approximation...
December 15, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Juergen Grulich-Henn, Daniela Klose
Diabetes mellitus (DM) is not a single disease, but several pathophysiological conditions where synthesis, release, and/or action of insulin are disturbed. A progressive autoimmune/autoinflammatory destruction of islet cells is still considered the main pathophysiological event in the development of T1DM, but there is evidence that T1DM itself is a heterogeneous disease. More than 50 gene regions are closely associated with T1DM and a variety of epigenetic factors and metabolic patterns have been characterized, which may play a role in the development of T1DM...
January 2018: Journal of Inherited Metabolic Disease
Marika Bogdani, Scott M Blackman, Cecilia Ridaura, Jean-Pierre Bellocq, Alvin C Powers, Lydia Aguilar-Bryan
Cystic fibrosis (CF)-related diabetes (CFRD) is thought to result from beta-cell injury due in part to pancreas exocrine damage and lipofibrosis. CFRD pancreata exhibit reduced islet density and altered cellular composition. To investigate a possible etiology, we tested the hypothesis that such changes are present in CF pancreata before the development of lipofibrosis. We evaluated pancreas and islet morphology in tissues from very young CF children (<4 years of age), and adult patients with CF and CFRD...
December 8, 2017: Scientific Reports
Manfred Ballmann, Dominique Hubert, Baroukh M Assael, Doris Staab, Alexandra Hebestreit, Lutz Naehrlich, Tanja Nickolay, Nicole Prinz, Reinhard W Holl
BACKGROUND: As survival among patients with cystic fibrosis has improved in recent decades, complications have become increasingly relevant. The most frequent complication is cystic-fibrosis-related diabetes. The recommended treatment is injected insulin, but some patients are treated with oral antidiabetic drugs to ease the treatment burden. We assessed the efficacy and safety of oral antidiabetic drugs. METHODS: We did a multicentre, open-label, comparative, randomised trial in 49 centres in Austria, France, Germany, and Italy...
February 2018: Lancet Diabetes & Endocrinology
Antoinette Moran
No abstract text is available yet for this article.
February 2018: Lancet Diabetes & Endocrinology
Kristina H Berg, Lene Ryom, Daniel Faurholt-Jepsen, Tania Pressler, Terese L Katzenstein
BACKGROUND: With improved prognosis of CF, comorbidities including chronic kidney disease (CKD) are becoming increasingly important. Identification of those at highest CKD risk is hence a priority. METHODS: In this cross-sectional study, adults with CF attending the Copenhagen CF Centre at Rigshospitalet with ≥2 measurements of serum creatinine from 2013 to 2015 were included. Data was obtained from an electronic CF database, which contains anonymised clinical and laboratory data on all individuals attending the clinic...
November 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
María Clemente León, Laura Bilbao Gassó, Antonio Moreno-Galdó, Ariadna Campos Martorrell, Silvia Gartner Tizzano, Diego Yeste Fernández, Antonio Carrascosa Lezcano
INTRODUCTION: Patients with cystic fibrosis (CF) undergo a slow and progressive process toward diabetes. Oral glucose tolerance test (OGTT) is recommended to diagnose impaired glucose levels in these patients. Continuous glucose monitoring (CGM) measures glucose profiles under real-life conditions. OBJECTIVE: To compare OGTT and CGM results in CF patients. METHODS: Paired OGTT and 6-day CGM profiles (146.2±9.1h/patient) were performed in 30 CF patients aged 10-18 years...
January 2018: Endocrinología, Diabetes y Nutrición
Karla Foster, Guixia Huang, Nanhua Zhang, Joseph Crisalli, Barbara Chini, Raouf Amin, Deborah Elder
BACKGROUND: Improved exercise capacity (EC) and normal glucose tolerance (NGT) are independently associated with favorable outcomes in CF, however, little information on this relationship exists in patients with CF. METHODS: Cardiopulmonary exercise tests, oral glucose tolerance tests (OGTT), and HbA1c values measured within a 12-month period were evaluated on 83 pediatric patients diagnosed with CF. Patients were categorized as having NGT, abnormal glucose tolerance (AGT), or CF-related diabetes (CFRD)...
February 2018: Pediatric Pulmonology
Thomas Radtke, Sarah J Nevitt, Helge Hebestreit, Susi Kriemler
BACKGROUND: Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. OBJECTIVES: To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings...
November 1, 2017: Cochrane Database of Systematic Reviews
Domenico Santoro, Adele Postorino, Cristina Lucanto, Stefano Costa, Simona Cristadoro, Salvatore Pellegrino, Giovanni Conti, Michele Buemi, Giuseppe Magazzù, Guido Bellinghieri
OBJECTIVE: Cystic fibrosis (CF) is the most common autosomal recessive disease affecting the Caucasian population, with a birth incidence ranging between 1:2,500 and 1:1,800. It is caused by mutations in the CF transmembrane regulator gene which is localized on 7 chromosomes. Renal disease is reported as a relatively rare complication in adult patient with CF. We evaluated proteinuria and chronic renal failure (CRF) in a population of patients with CF. METHODS: A retrospective study was carried out in a referral center for CF at University of Messina in Italy...
November 2017: Journal of Renal Nutrition
Stefan Elde, Stephen Huddleston, Scott Jackson, Rosemary Kelly, Sara Shumway, Gabriel Loor
BACKGROUND: We evaluated the effects of tailoring the operative approach on major surgical site complications and outcomes in lung transplant recipients. PATIENTS AND METHODS: Beginning in July 2013, bilateral lung transplants at a single institution were performed either through sternotomy or clamshell depending on proximity of hilar structures by computed tomography (CT), anticipated complexity, past surgical history, and surgeon experience. Patient demographics and outcomes were collected in the institution's Transplant Information Services (TIS)...
October 20, 2017: Surgical Infections
Quitterie Reynaud, Muriel Rabilloud, Sylvain Roche, Stéphanie Poupon-Bourdy, Jean Iwaz, Raphaële Nove-Josserand, Emilie Blond, Martine Laville, Cathy Llerena, Sébastien Quetant, Philippe Reix, Sandrine Touzet, Isabelle Durieu
BACKGROUND: The prevalence of cystic fibrosis-related diabetes is increasing. This condition is potentially responsible for respiratory decline. METHODS: At inclusion, then yearly (over three years), 111 children and 117 adults with cystic fibrosis had oral glucose tolerance and insulin tests at one (G1) and 2h (G2). KmL analysis identified homogeneous G1 and G2 glucose trajectories. A linear mixed model quantified the relationships between trajectories and FEV1 changes...
October 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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