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Cystic fibrosis related diabetes

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https://www.readbyqxmd.com/read/28906321/variceal-hemorrhage-and-adverse-liver-outcomes-in-patients-with-cystic-fibrosis-cirrhosis
#1
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
#2
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
#3
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...
August 2, 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
#4
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
#5
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
#6
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
#7
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
#8
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...
July 11, 2017: PharmacoEconomics
https://www.readbyqxmd.com/read/28648493/the-use-of-fructosamine-in-cystic-fibrosis-related-diabetes-cfrd-screening
#9
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
https://www.readbyqxmd.com/read/28646623/pregnancy-outcome-in-women-with-cystic-fibrosis-related-diabetes
#10
Quitterie Reynaud, Stéphanie Poupon-Bourdy, Muriel Rabilloud, Lina Al Mufti, Christine Rousset Jablonski, Lydie Lemonnier, Raphaële Nove-Josserand, Sandrine Touzet, Isabelle Durieu
With increasing life expectancy more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis related diabetes) influenced pregnancy outcome and the clinical status of these women MATERIAL AND METHODS: We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery RESULTS: A total 249 women delivered 314 infants...
June 24, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28625799/continuous-glucose-monitoring-in-a-cystic-fibrosis-patient-to-predict-pulmonary-exacerbation
#11
T B Inman, J A Proudfoot, M Lim, C Demeterco-Berggren
Patients with cystic fibrosis (CF) experience a significant decline in pulmonary status before the diagnosis of cystic fibrosis related diabetes (CFRD). We hypothesized that hyperglycemia may be a factor in the decline of pulmonary function and increased frequency of pulmonary exacerbations. Long term continuous glucose monitoring (CGM) has not been reported in patients with CF and impaired glucose tolerance. We performed CGM for three months in a 17year old male with F508del and F553X CF mutations, baseline forced expiratory volume in 1s (FEV1) of 92% predicted, and impaired glucose tolerance to evaluate changes in glucose levels prior to the diagnosis of a pulmonary exacerbation...
September 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28622948/high-level-of-%C3%AE-1-3-d-glucan-antigenaemia-in-cystic-fibrosis-in-the-absence-of-invasive-fungal-disease
#12
Vilma Rautemaa, Heather D Green, Andrew M Jones, Riina Rautemaa-Richardson
β-(1,3)-d-glucan (BDG) is used to rule out invasive fungal disease (IFD) but its usefulness in cystic fibrosis (CF) has not been evaluated. We measured serum BDG in CF patients with no clinical suspicion of IFD. Samples from 46 adult CF patients during a stable period and during pulmonary exacerbation were tested. The association of BDG with clinical variables was analyzed. Three hundred and three non-CF patients with suspected IFD were used as comparators. Both samples were negative in 52% of CF patients, whereas 67% of comparators had only negative results (P=0...
May 18, 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/28539697/physical-activity-levels-in-individuals-with-cystic-fibrosis-related-diabetes
#13
Sarah Currie, Krista Greenwood, Lara Weber, Hussein Khakee, Michelle Legasto, Elizabeth Tullis, Kenneth Wu, Anna Tsang, Erika Szego, Sunita Mathur
Purpose: The literature on physical activity (PA) in adults with cystic fibrosis, particularly in those with cystic fibrosis-related diabetes (CFRD), is limited. PA may be an important part of blood glucose management in CFRD. The purpose of this study was to describe PA levels in adults with CFRD and determine their adherence to the Canadian Diabetes Association (CDA) aerobic exercise training guidelines. Methods: Adults with CFRD were recruited from a hospital-based CF clinic. PA was measured using the Seven-Day Physical Activity Recall (telephone interview), adherence to CFRD management with the Self-Care Inventory-Revised (questionnaire), and blood glucose control from glycated hemoglobin levels documented in participants' medical chart within 3 months...
2017: Physiotherapy Canada. Physiothérapie Canada
https://www.readbyqxmd.com/read/28534947/research-and-progress-on-clc%C3%A2-2-review
#14
Hongwei Wang, Minghui Xu, Qingjie Kong, Peng Sun, Fengyun Yan, Wenying Tian, Xin Wang
Chloride channel 2 (ClC-2) is one of the nine mammalian members of the ClC family. The present review discusses the molecular properties of ClC‑2, including CLCN2, ClC‑2 promoter and the structural properties of ClC‑2 protein; physiological properties; functional properties, including the regulation of cell volume. The effects of ClC‑2 on the digestive, respiratory, circulatory, nervous and optical systems are also discussed, in addition to the mechanisms involved in the regulation of ClC‑2. The review then discusses the diseases associated with ClC‑2, including degeneration of the retina, Sjögren's syndrome, age‑related cataracts, degeneration of the testes, azoospermia, lung cancer, constipation, repair of impaired intestinal mucosa barrier, leukemia, cystic fibrosis, leukoencephalopathy, epilepsy and diabetes mellitus...
July 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28499359/cystic-fibrosis-carriership-and-tuberculosis-hints-toward-an-evolutionary-selective-advantage-based-on-data-from-the-brazilian-territory
#15
Lander Bosch, Barbara Bosch, Kris De Boeck, Tim Nawrot, Isabelle Meyts, Dominique Vanneste, Cleonice Alexandre Le Bourlegat, Julio Croda, Luiz Vicente Ribeiro Ferreira da Silva Filho
BACKGROUND: The reason why Cystic Fibrosis (CF) is the most common fatal genetic disease among Caucasians has been incompletely studied. We aimed at deepening the hypothesis that CF carriers have a relative protection against Mycobacterium tuberculosis (Mtb) infection. METHODS: Applying spatial epidemiology, we studied the link between CF carriership rate and tuberculosis (TB) incidence in Brazil. We corrected for 5 potential environmental and 2 immunological confounders in this relation: monthly income, sanitary provisions, literacy rates, racial composition and population density along with AIDS incidence rates and diabetes mellitus type 2...
May 12, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28479021/what-keeps-children-with-cystic-fibrosis-awake-at-night
#16
Moya Vandeleur, Lisa M Walter, David S Armstrong, Philip Robinson, Gillian M Nixon, Rosemary S C Horne
BACKGROUND: Sleep disturbance is common in children with cystic fibrosis (CF) however there are limited studies investigating the causes for poor sleep quality. In a cross sectional observational study we aimed to evaluate the clinical correlates of sleep disturbance in this population. METHODS: Children with CF (7-18years) free from pulmonary exacerbation completed medical review, overnight oximetry, the OSA-18 and 14days of actigraphy recordings with a sleep diary...
May 4, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28472055/abdominal-symptoms-in-cystic-fibrosis-and-their-relation-to-genotype-history-clinical-and-laboratory-findings
#17
Harold Tabori, Christin Arnold, Anke Jaudszus, Hans-Joachim Mentzel, Diane M Renz, Steffen Reinsch, Michael Lorenz, Ruth Michl, Andrea Gerber, Thomas Lehmann, Jochen G Mainz
BACKGROUND & AIMS: Abdominal symptoms (AS) are a hallmark of the multiorgan-disease cystic fibrosis (CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history. METHODS: A total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire (JenAbdomen-CF score 1...
2017: PloS One
https://www.readbyqxmd.com/read/28446387/risk-factors-for-persistent-methicillin-resistant-staphylococcus-aureus-infection-in-cystic-fibrosis
#18
Mark T Jennings, Elliot C Dasenbrook, Noah Lechtzin, Michael P Boyle, Christian A Merlo
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) has emerged as an important pathogen in cystic fibrosis (CF). Over 25% of individuals in the United States with CF are found to have MRSA in respiratory culture specimens, and persistent MRSA infection has been associated with more rapid decline in lung function and increased mortality. The objective of this study was to investigate clinical and demographic characteristics that are associated with the development of persistent MRSA infection in a CF population...
April 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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
#19
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
#20
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...
March 7, 2017: Scientific Reports
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