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Cystic fibrosis related diabetes

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https://www.readbyqxmd.com/read/29137964/oral-glucose-tolerance-test-and-continuous-glucose-monitoring-to-assess-diabetes-development-in-cystic-fibrosis-patients
#1
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...
November 11, 2017: Endocrinología, diabetes y nutrición
https://www.readbyqxmd.com/read/29115018/relationship-between-exercise-capacity-and-glucose-tolerance-in-cystic-fibrosis
#2
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)...
November 8, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29090734/physical-exercise-training-for-cystic-fibrosis
#3
REVIEW
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
https://www.readbyqxmd.com/read/29056168/cystic-fibrosis-a-risk-condition-for-renal-disease
#4
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
https://www.readbyqxmd.com/read/29053438/tailored-approach-to-surgical-exposure-reduces-surgical-site-complications-after-bilateral-lung-transplantation
#5
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
https://www.readbyqxmd.com/read/29037538/glucose-trajectories-in-cystic-fibrosis-and-their-association-with-pulmonary-function
#6
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
https://www.readbyqxmd.com/read/28977616/cftr-ferreting-out-its-role-in-cystic-fibrosis-related-diabetes
#7
Leslie S Satin, Vishal S Parekh
No abstract text is available yet for this article.
October 1, 2017: Endocrinology
https://www.readbyqxmd.com/read/28977592/cftr-influences-beta-cell-function-and-insulin-secretion-through-non-cell-autonomous-exocrine-derived-factors
#8
Xingshen Sun, Yaling Yi, Weiliang Xie, Bo Liang, Michael C Winter, Nan He, Xiaoming Liu, Meihui Luo, Yu Yang, Katie Larson Ode, Aliye Uc, Andrew W Norris, John F Engelhardt
Although β-cell dysfunction in cystic fibrosis (CF) leads to diabetes, the mechanism by which the cystic fibrosis transmembrane conductance regulator (CFTR) channel influences islet insulin secretion remains debated. We investigated the CFTR-dependent islet-autonomous mechanisms affecting insulin secretion by using islets isolated from CFTR knockout ferrets. Total insulin content was lower in CF as compared with wild-type (WT) islets. Furthermore, glucose-stimulated insulin secretion (GSIS) was impaired in perifused neonatal CF islets, with reduced first, second, and amplifying phase secretion...
October 1, 2017: Endocrinology
https://www.readbyqxmd.com/read/28938479/pulmonary-function-and-sleep-breathing-two-new-targets-for-type-2-diabetes-care
#9
Albert Lecube, Rafael Simó, Maria Pallayova, Naresh M Punjabi, Carolina López-Cano, Cecilia Turino, Cristina Hernández, Ferran Barbé
Population based studies showing the negative impact of type 2 diabetes (T2D) on lung function are overviewed. Among the well-recognized pathophysiological mechanisms, the metabolic pathways related to insulin resistance, low-grade chronic inflammation, leptin resistance, microvascular damage, and autonomic neuropathy are emphasized. Histopathological changes are exposed, and findings reported from experimental models are clearly differentiated from those described in humans. The accelerated decline in pulmonary function that appears in patients with cystic fibrosis with related abnormalities of glucose tolerance and diabetes is considered as an example to further investigate the relationship between T2D and the lung...
September 4, 2017: Endocrine Reviews
https://www.readbyqxmd.com/read/28934665/venous-thromboembolism-in-children-with-cystic-fibrosis-retrospective-incidence-and-intrapopulation-risk-factors
#10
Jessica Knight-Perry, Brian R Branchford, Dianne Thornhill, Stacey L Martiniano, Scott D Sagel, Michael Wang
INTRODUCTION: Pediatric venous thromboembolism (VTE) is a rare but serious medical condition. Cystic fibrosis (CF) is a risk for recurrent pediatric VTE and has potential thrombophilic tendency. However, much remains unknown, including incidence and intrapopulation risk factors. METHODS: A retrospective cohort of pediatric CF patients followed at Children's Hospital Colorado from January 1st 2003 through May 20th 2016 was examined. Cases were identified by informatics and validated manually...
September 5, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28919081/body-weight-and-body-mass-index-in-patients-with-end-stage-cystic-fibrosis-stabilize-after-the-start-of-enteral-tube-feeding
#11
Francis M Hollander, Nicole M de Roos, Gerdien Belle van Meerkerk, Ferdinand Teding van Berkhout, Harry G M Heijerman, Ed A van de Graaf
BACKGROUND: Enteral tube feeding (ETF) is widely used in patients with cystic fibrosis (CF) and end-stage lung disease, but previous studies have been limited to investigating whether ETF improves outcomes in patients with moderately or mildly impaired pulmonary function. OBJECTIVE: This study investigated body weight, body mass index (BMI; calculated as kg/m(2)), pulmonary function, and the presence of CF-related diabetes before and after the start of ETF. DESIGN: This was a retrospective observational study...
November 2017: Journal of the Academy of Nutrition and Dietetics
https://www.readbyqxmd.com/read/28906321/variceal-hemorrhage-and-adverse-liver-outcomes-in-patients-with-cystic-fibrosis-cirrhosis
#12
Wen Ye, Michael R Narkewicz, Daniel H Leung, Wikrom Karnsakul, Karen F Murray, Estella M Alonso, John C Magee, Sarah Jane Schwarzenberg, Alexander Weymann, Jean P Molleston
OBJECTIVES: Cirrhosis occurs in 5-10% of CF (cystic fibrosis) patients, often accompanied by portal hypertension. We analyzed three adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver death (LD), and risk factors for these in CF Foundation Patient Registry (CFFPR) subjects with reported cirrhosis. METHODS: We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression...
September 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28830798/implementation-of-depression-screening-and-global-health-assessment-in-pediatric-subspecialty-clinics
#13
Esti Iturralde, Rebecca N Adams, Regan C Barley, Rachel Bensen, Megan Christofferson, Sarah J Hanes, David M Maahs, Carlos Milla, Diana Naranjo, Avni C Shah, Molly L Tanenbaum, Sruthi Veeravalli, K T Park, Korey K Hood
PURPOSE: Adolescents with chronic illness face greater risk of psychosocial difficulties, complicating disease management. Despite increased calls to screen for patient-reported outcomes, clinical implementation has lagged. Using quality improvement methods, this study aimed to investigate the feasibility of standardized screening for depression and assessment of global health and to determine recommended behavioral health follow-up, across three pediatric subspecialty clinics. METHODS: A total of 109 patients aged 12-22 years (median = 16...
August 19, 2017: Journal of Adolescent Health: Official Publication of the Society for Adolescent Medicine
https://www.readbyqxmd.com/read/28770464/long-term-follow-up-in-a-girl-with-cystic-fibrosis-and-diabetes-since-the-first-year-of-life
#14
Valentina Fattorusso, Alida Casale, Valeria Raia, Enza Mozzillo, Adriana Franzese
Diabetes mellitus is the most common comorbidity in cystic fibrosis (CF). Recently, more attention has been paid to early glucose metabolism derangements (GMDs). The subject of this report is a female patient, affected by CF since 3 months of age. She presented with intermittent diabetes during early childhood. At the age of 10 years, oral glucose tolerance test (OGTT) was performed and showed glucose intolerance (IGT) status; glargine insulin therapy was started. At the age of 13 years, CF-related diabetes with fasting hyperglycemia occurred, so rapid insulin at meals was added...
October 2017: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
https://www.readbyqxmd.com/read/28747217/persistence-of-candida-dubliniensis-and-lung-function-in-patients-with-cystic-fibrosis
#15
Atqah AbdulWahab, Husam Salah, Prem Chandra, Saad J Taj-Aldeen
OBJECTIVES: Candida dubliniensis is an emerging yeast and demonstrated a high adherence property to cystic fibrosis respiratory tract. Therefore, it is important to determine the persistence of C. dubliniensis and to assess the possible relationship to the body mass index (BMI) and forced expiratory volume in 1st second (FEV1). RESULTS: Candida isolates were identified by MALDI-TOF MS to species level from 40/52 (76.9%) cystic fibrosis patients. C. dubliniensis was the most common organism isolated from 50/77 (65%) lower respiratory specimens of 29 patients...
July 26, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28714601/cf-related-diabetes-containing-the-metabolic-miscreant-of-cystic-fibrosis
#16
Amir Moheet, Antoinette Moran
Cystic fibrosis-related diabetes (CFRD) is associated with both an increase in morbidity and mortality in people with cystic fibrosis (CF). With increased screening and improved life expectancy of people with CF, the prevalence of CFRD is expected to rise further. The underlying pathophysiological mechanisms causing glucose intolerance and diabetes in patients with CF are not well understood but both functional and structural abnormalities in islet cells are likely to have key roles. Insulin therapy improves health outcomes in patients with CF...
July 17, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28705217/monitoring-clinical-and-microbiological-evolution-of-a-cystic-fibrosis-patient-over-26%C3%A2-years-experience-of-a-brazilian-cf-centre
#17
Cassiana da Costa Ferreira Leite, Tania Wrobel Folescu, Mônica de Cássia Firmida, Renata Wrobel Folescu Cohen, Robson Souza Leão, Flávia Alvim Dutra de Freitas, Rodolpho Mattos Albano, Claudia Henrique da Costa, Elizabeth Andrade Marques
BACKGROUND: Burkholderia cepacia complex is a group of opportunistic pathogens in cystic fibrosis (CF) patients believed to be associated with poor prognosis and patient-to-patient transmissibility. Little is known about clinical outcomes after B. vietnamiensis chronic colonization/infection. CASE PRESENTATION: A 33 yo male patient had diagnosis of CF by 7 yo, after recurrent pneumonia during infancy and lobectomy (left upper lobe) at 6 yo. Burkholderia cepacia complex (Bcc) was first isolated by 13 yo, and the patient fulfilled the criteria for chronic colonization by 15 yo...
July 14, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28701371/population-based-assessment-of-a-biomarker-based-screening-pathway-to-aid-diagnosis-of-monogenic-diabetes-in-young-onset-patients
#18
Beverley M Shields, Maggie Shepherd, Michelle Hudson, Timothy J McDonald, Kevin Colclough, Jaime Peters, Bridget Knight, Chris Hyde, Sian Ellard, Ewan R Pearson, Andrew T Hattersley
OBJECTIVE: Monogenic diabetes, a young-onset form of diabetes, is often misdiagnosed as type 1 diabetes, resulting in unnecessary treatment with insulin. A screening approach for monogenic diabetes is needed to accurately select suitable patients for expensive diagnostic genetic testing. We used C-peptide and islet autoantibodies, highly sensitive and specific biomarkers for discriminating type 1 from non-type 1 diabetes, in a biomarker screening pathway for monogenic diabetes. RESEARCH DESIGN AND METHODS: We studied patients diagnosed at age 30 years or younger, currently younger than 50 years, in two U...
August 2017: Diabetes Care
https://www.readbyqxmd.com/read/28699086/estimating-direct-cost-of-cystic-fibrosis-care-using-irish-registry-healthcare-resource-utilisation-data-2008-2012
#19
Abaigeal D Jackson, Andrew L Jackson, Godfrey Fletcher, Gerardine Doyle, Mary Harrington, Shijun Zhou, Fiona Cullinane, Charles Gallagher, Edward McKone
BACKGROUND: Understanding the determinants of cost of cystic fibrosis (CF) care and health outcomes may be useful for financial planning for the delivery of CF services. Registries contain information otherwise unavailable to healthcare activity/cost monitoring systems. We estimated the direct medical cost of CF care using registry data and examined how cost was affected by patient characteristics and CF gene (CF Transmembrane Conductance Regulator [CFTR]) mutation. METHODS: Healthcare resource utilisation data (2008-2012) were obtained for CF patients enrolled with the Irish CF Registry by 2013 from linked registry and national hospitalisation database records...
October 2017: PharmacoEconomics
https://www.readbyqxmd.com/read/28648493/the-use-of-fructosamine-in-cystic-fibrosis-related-diabetes-cfrd-screening
#20
Grace Y Lam, Michelle Doll-Shankaruk, Jan Dayton, Karina Rodriguez-Capote, Trefor N Higgins, Dylan Thomas, Kimberley Mulchey, Maeve P Smith, Neil E Brown, Winnie M Leung, Mathew P Estey
OBJECTIVE: To determine whether serum fructosamine correlates with glycemic control and clinical outcomes in patients being screened for cystic fibrosis-related diabetes (CFRD). METHODS: Fructosamine and percent predicted forced expiratory volume in 1s (FEV1) were measured in patients undergoing a 2h oral glucose tolerance test (OGTT) for CFRD screening. Fractional serum fructosamine (FSF) was calculated as fructosamine/total protein. RESULTS: FSF exhibited a positive correlation with 2h OGTT results (r(2)=0...
June 22, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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