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Cystic fibrosis diabetes

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https://www.readbyqxmd.com/read/29780417/osteoporosis-is-associated-with-deteriorating-clinical-status-in-adults-with-cystic-fibrosis
#1
Inger Hee Mathiesen, Tacjana Pressler, Peter Oturai, Terese Lea Katzenstein, Marianne Skov, Ruth Frikke-Schmidt, Mette Friberg Hitz
Background: Cystic fibrosis (CF) patients are in increased risk of osteoporosis. We aimed to determine the osteoporosis prevalence in an adult CF cohort and investigate calcium metabolic parameters and clinical status' association with bone mineral density evaluated by dual X-ray absorptiometry scan. Methods: We performed a cross section database study of adults at a tertiary CF Center. Z scores were applied for patients < 50 years of age and T scores for patients > 50 years of age...
2018: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29773689/risk-factors-for-lung-disease-progression-in-children-with-cystic-fibrosis
#2
Marieke van Horck, Kim van de Kant, Bjorn Winkens, Geertjan Wesseling, Vincent Gulmans, Han Hendriks, Chris van der Grinten, Quirijn Jӧbsis, Edward Dompeling
To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009-2014). Lung disease progression was expressed as a decline in lung function (FEV1 %pred) and the pulmonary exacerbation rate (PEx). Potential risk factors at baseline included: sex, age, best FEV1%pred, best forced vital capacity (FVC)%pred, genotype, BMI z-score, pancreatic insufficiency, medication use (proton pump inhibitors [PPI], prophylactic antibiotics, and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis, and colonisation with Pseudomonas aeruginosa The data of 545 children were analysed...
May 17, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29769033/case-report-on-pathogenetic-link-between-gluten-and-iga-nephropathy
#3
Stefano Costa, Giovanni Currò, Salvatore Pellegrino, Maria Cristina Lucanto, Giovanni Tuccari, Antonio Ieni, Giuseppina Visalli, Giuseppe Magazzù, Domenico Santoro
BACKGROUND: A relationship between IgA nephropathy (IgAN) and celiac disease (CD) has been reported. We show the pathogenetic link for the first time. CASE PRESENTATION: A 39-year-old man with cystic fibrosis (CF) and CF-related diabetes started to present gross hematuria, back pain and headache. At admission, laboratory analysis showed increase in serum creatinine of 1.5 mg/dl, together with hematuria and mild proteinuria (1 g/24 h). He underwent a renal biopsy to investigate the cause of hematuria and renal failure...
May 16, 2018: BMC Gastroenterology
https://www.readbyqxmd.com/read/29761141/unexpected-height-loss-in-an-adolescent-with-cystic-fibrosis
#4
Anne Schendel, Jillian Mai, Rachel Espinosa, Carlye T Tomcyzk, Theresa A Laguna
This case report describes an adolescent with pancreatic insufficient cystic fibrosis, malnutrition, and cystic fibrosis-related diabetes found to have a loss in height at a routine outpatient clinic visit. He was subsequently admitted to the hospital for treatment of a pulmonary exacerbation and was found to have multiple, nontraumatic vertebral compression fractures. This report emphasizes the importance of routine review of growth charts and aggressive treatment of vitamin deficiencies and malnutrition in people with cystic fibrosis...
2018: Global Pediatric Health
https://www.readbyqxmd.com/read/29742360/disparities-in-mortality-of-hispanic-cystic-fibrosis-patients-in-the-united-states-a-national-and-regional-cohort-study
#5
Jason Rho, Chul Ahn, Ang Gao, Gregory S Sawicki, Ashley Keller, Raksha Jain
RATIONALE: Cystic Fibrosis (CF) patients of Hispanic origin are the largest growing minority, representing 8.5% of CF patients in the United States. No national survival analysis of this group has ever been undertaken. OBJECTIVE: We aimed to determine whether Hispanic ethnicity within the CF population is associated with worse outcomes and whether any geographic differences exist. METHODS: Using U.S. CF Foundation Patient Registry data from 2010-2014, we performed a retrospective cohort analysis comparing survival rates between Hispanics and non-Hispanics using Kaplan-Meier and Cox regression analysis...
May 9, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29740976/high-prevalence-of-diabetes-before-and-after-lung-transplantation-target-for-improving-outcome
#6
Monika Fazekas-Lavu, Michael Reyes, Monique Malouf, Marshall Plit, Adrian Havryk, Lesley V Campbell, Jacqueline R Center, Allan R Glanville, Jerry R Greenfield
BACKGROUND: Diabetes increases morbidity and mortality of lung transplantation. However, the reported prevalence of diabetes varies post-transplantation partly due to lack of detection protocols. AIMS: We determined the prevalence of diabetes in patients (i) waitlisted for lung transplant and (ii) early post-transplantation. METHODS: We analyzed patients on the St Vincent's Heart Lung database from 1/4/14 to 30/9/15 on the waitlist (Study 1) and those transplanted (Study 2)...
May 9, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29700855/long-term-extra-pulmonary-comorbidities-after-lung-transplantation-in-cystic-fibrosis-update-of-specificities
#7
REVIEW
Sabine Jardel, Quitterie Reynaud, Isabelle Durieu
Lung transplantation (LT) is the standard therapeutic option for cystic fibrosis (CF) patients with end-stage lung disease. Both conditions lead to extra-respiratory complications, such as diabetes, renal insufficiency, bone disease, and cancer. The purpose of the present paper is to provide an update of the non-respiratory comorbidities following LT in adult patients with CF and their specificities regarding their multi-systemic underlying condition despite their younger age compared to other patients undergoing LT...
April 26, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29691278/decreasing-the-time-to-insulin-administration-for-hospitalized-patients-with-cystic-fibrosis-related-diabetes
#8
Allison Smego, Sarah Lawson, Joshua D Courter, Deborah Warden, Sarah Corathers
OBJECTIVES: Children with cystic fibrosis-related diabetes (CFRD) represent a commonly hospitalized pediatric population whose members require insulin for blood glucose (BG) control. The aim of this quality improvement initiative was to increase the proportion of hospitalized patients with CFRD receiving insulin within 30 minutes of a BG check while decreasing severe hypo- and hyperglycemia episodes. METHODS: Quality improvement methodology (gathering a team of stakeholders, identifying metrics, implementing iterative plan-do-study-act cycles and analysis of data over time) was applied in the setting of a cystic fibrosis unit in a tertiary care children's hospital...
May 2018: Hospital Pediatrics
https://www.readbyqxmd.com/read/29687501/unique-challenges-of-cystic-fibrosis-related-diabetes
#9
REVIEW
N Bridges, R Rowe, R I G Holt
Individuals with cystic fibrosis and pancreatic insufficiency have a gradual decline in insulin secretion over time, which results in an increase in the prevalence of diabetes with age; up to 50% of adults with cystic fibrosis aged over 35-40 years have diabetes. Cystic fibrosis-related diabetes differs from Type 1 and Type 2 diabetes in several ways; there is a pattern of insulin deficiency with reduced and delayed insulin response to carbohydrates but a sparing of basal insulin that results in glucose abnormalities, which are frequently characterized by normal fasting glucose and postprandial hyperglycaemia...
April 23, 2018: Diabetic Medicine: a Journal of the British Diabetic Association
https://www.readbyqxmd.com/read/29686977/a-prototype-exercise-empowerment-mobile-video-game-for-children-with-cancer-and-its-usability-assessment-developing-digital-empowerment-interventions-for-pediatric-diseases
#10
Carol S Bruggers, Sabrina Baranowski, Mathew Beseris, Rachel Leonard, Derek Long, Elizabeth Schulte, Ashton Shorter, Rowan Stigner, Clinton C Mason, Alisa Bedrov, Ian Pascual, Grzegorz Bulaj
Background: Medical advances continue to improve morbidity and mortality of serious pediatric diseases, including cancer, driving research addressing diminished physical and psychological quality of life in children with these chronic conditions. Empowerment enhances resilience and positively influences health, disease, and therapy understanding. We describe the development and usability assessment of a prototype Empower Stars! mobile video game grounded in behavioral and exercise theories with the purpose of coupling physical exercise with empowerment over disease in children with cancer...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29684175/aminoglycoside-exposure-and-renal-function-before-lung-transplantation-in-adult-cystic-fibrosis-patients
#11
Etienne Novel-Catin, Solenne Pelletier, Quitterie Reynaud, Raphaele Nove-Josserand, Stephane Durupt, Laurence Dubourg, Isabelle Durieu, Denis Fouque
Background: Patients with cystic fibrosis (CF) are at risk of kidney injury even before undergoing lung transplantation, because of prolonged exposure to aminoglycosides (AGs), chronic dehydration and complications of diabetes mellitus. The usual equations estimating the glomerular filtration rate (GFR), such as Cockcroft-Gault and Modification of Diet in Renal Disease, are not adapted to the CF population due to patients' low body weight and reduced muscle mass. The aim of this study was to precisely measure GFR in adult CF patients and to see whether repeated AG treatment would impair renal function before lung transplantation...
April 18, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29680365/prevalence-of-hypoglycemia-during-oral-glucose-tolerance-testing-in-adults-with-cystic-fibrosis-and-risk-of-developing-cystic-fibrosis-related-diabetes
#12
Lisa A Mannik, Kristy A Chang, Pascalyn Q K Annoh, Jenna Sykes, Julie Gilmour, Ronalee Robert, Anne L Stephenson
BACKGROUND: Hypoglycemia in cystic fibrosis (CF) patients during the oral glucose tolerance test (OGTT) has been reported; however, these patients have not been well-characterized. Few studies have examined whether hypoglycemia during the OGTT increases the risk of developing CF-related diabetes (CFRD). Objectives of this study were to describe the characteristics of CF patients with hypoglycemia during the OGTT and to determine the incidence and time to development of CFRD in those with hypoglycemia...
April 18, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29674323/hemoglobin-a-1c-accurately-predicts-continuous-glucose-monitoring-derived-average-glucose-in-youth-and-young-adults-with-cystic-fibrosis
#13
Christine L Chan, Emma Hope, Jessica Thurston, Timothy Vigers, Laura Pyle, Philip S Zeitler, Kristen J Nadeau
OBJECTIVE: In cystic fibrosis (CF), HbA1c is thought to underestimate glycemia. However, few studies have directly assessed the relationship between HbA1c and average glucose in CF. We determined the relationships among glycemic markers-HbA1c , fructosamine (FA), glycated albumin (%GA), and 1,5-anhydroglucitol (1,5-AG)-and continuous glucose monitoring (CGM) in CF, hypothesizing that alternate markers would better predict average sensor glucose (ASG) than HbA1c . RESEARCH DESIGN AND METHODS: CF participants and a group of healthy control subjects (HC), ages 6-25 years, wore CGM for up to 7 days...
April 19, 2018: Diabetes Care
https://www.readbyqxmd.com/read/29669939/cystic-fibrosis-related-diabetes-is-caused-by-islet-loss-and-inflammation
#14
Nathaniel J Hart, Radhika Aramandla, Gregory Poffenberger, Cody Fayolle, Ariel H Thames, Austin Bautista, Aliya F Spigelman, Jenny Aurielle B Babon, Megan E DeNicola, Prasanna K Dadi, William S Bush, Appakalai N Balamurugan, Marcela Brissova, Chunhua Dai, Nripesh Prasad, Rita Bottino, David A Jacobson, Mitchell L Drumm, Sally C Kent, Patrick E MacDonald, Alvin C Powers
Cystic fibrosis-related (CF-related) diabetes (CFRD) is an increasingly common and devastating comorbidity of CF, affecting approximately 35% of adults with CF. However, the underlying causes of CFRD are unclear. Here, we examined cystic fibrosis transmembrane conductance regulator (CFTR) islet expression and whether the CFTR participates in islet endocrine cell function using murine models of β cell CFTR deletion and normal and CF human pancreas and islets. Specific deletion of CFTR from murine β cells did not affect β cell function...
April 19, 2018: JCI Insight
https://www.readbyqxmd.com/read/29669347/combined-pancreatic-islet-lung-liver-transplantation-in-a-pediatric-patient-with-cystic-fibrosis-related-diabetes
#15
Philippe Klee, Mirjam Dirlewanger, Vanessa Lavallard, Valerie A McLin, Anne Mornand, Nadine Pernin, Laetitia-Marie Petit, Paola M Soccal, Barbara E Wildhaber, Urs Zumsteg, Jean-Louis Blouin, Thierry Berney, Valerie M Schwitzgebel
BACKGROUND: Cystic fibrosis-related diabetes (CFRD) is the most frequent extrapulmonary complication of cystic fibrosis (CF). METHODS: We report the first combined pancreatic islet-lung-liver transplantation in a 14-year-old adolescent. CFTR was analyzed by Sanger sequencing. Further genes were analyzed by high-throughput sequencing. RESULTS: The patient was diagnosed with CF at the age of 14 months. Nine years later, after diagnosis of CFRD, the patient's BMI and lung function began to decline...
April 18, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29515516/cystic-fibrosis-related-diabetes
#16
REVIEW
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
https://www.readbyqxmd.com/read/29512777/cftr-ameliorates-high-glucose-induced-oxidative-stress-and-inflammation-by-mediating-the-nf-%C3%AE%C2%BAb-and-mapk-signaling-pathways-in-endothelial-cells
#17
Yang Fei, Liqin Sun, Chungang Yuan, Min Jiang, Qinhua Lou, Yan Xu
Diabetic cardiovascular diseases are characterized by progressive hyperglycemia, which results in excessive production of oxidative stress and pro-inflammatory cytokines. Cystic fibrosis (CF) is characterized by chronic inflammation due to mutations in CF transmembrane conductance regulator (CFTR). However, little information is available about the role of CFTR in hyperglycemia‑induced endothelial cell oxidative stress and inflammation. In the present study, a high glucose‑treatment was applied in human umbilical vein endothelial cells with CFTR overexpression or inhibition, and the oxidative and inflammatory characteristics were measured...
June 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29506377/-italian-cystic-fibrosis-registry-report-2011-2014
#18
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
https://www.readbyqxmd.com/read/29504914/targeted-sequencing-reveals-complex-phenotype-correlated-genotypes-in-cystic-fibrosis
#19
Maxim Ivanov, Alina Matsvay, Olga Glazova, Stanislav Krasovskiy, Mariya Usacheva, Elena Amelina, Aleksandr Chernyak, Mikhail Ivanov, Sergey Musienko, Timofey Prodanov, Sergey Kovalenko, Ancha Baranova, Kamil Khafizov
BACKGROUND: Cystic fibrosis (CF) is one of the most common life-threatening genetic disorders. Around 2000 variants in the CFTR gene have been identified, with some proportion known to be pathogenic and 300 disease-causing mutations have been characterized in detail by CFTR2 database, which complicates its analysis with conventional methods. METHODS: We conducted next-generation sequencing (NGS) in a cohort of 89 adult patients negative for p.Phe508del homozygosity...
February 13, 2018: BMC Medical Genomics
https://www.readbyqxmd.com/read/29490415/concentration-of-8-isoprostanes-in-the-exhaled-breath-condensate-as-a-marker-of-oxidative-stress-in-patients-with-type-1-diabetes
#20
Anna Pękala-Wojciechowska, Michał Poznański, Kamil Szyszow, Adam Antczak
INTRODUCTION: Type 1 diabetes is an insulin deficiency-based chronic disease. It leads to the development of hyperglycaemia, which plays a key role in the initiation and progression of tissue damage in patients with diabetes. This mostly results from oxidative stress, whose increased severity is observed in this group of patients. Increased levels of 8-isoprostanes are seen in many inflammatory diseases, including asthma, COPD and cystic fibrosis. These diseases demonstrated the usefulness of the exhaled breath condensate (EBC) for extracting material for markers of oxidative stress, including 8-isoprostanes...
2018: Advances in Respiratory Medicine
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