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Cystic fibrosis 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/29115094/the-first-successful-lung-transplantation-in-a-korean-child-with-cystic-fibrosis
#2
Soo Ran Noh, Eun Lee, Jisun Yoon, Sungsu Jung, Song I Yang, Jinho Yu, Soo Jong Hong
Cystic fibrosis (CF) is an autosomal recessive inherited multisystem disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Respiratory failure remains the most frequent cause of morbidity and mortality. Lung transplantation is the only option to treat end-stage lung disease. Very few cases of CF occur in Koreans. We report the case of a 12-year-old girl with respiratory failure due to CF who underwent lung transplantation. She had been diagnosed with CF 8 years previously after being treated for recurrent Pseudomonas aeruginosa pneumonia and malnutrition based on sweat chloride concentrations and the CFTR protein gene mutation test...
December 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29115018/relationship-between-exercise-capacity-and-glucose-tolerance-in-cystic-fibrosis
#3
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/29093615/less-common-etiologies-of-exocrine-pancreatic-insufficiency
#4
REVIEW
Vikesh K Singh, Mark E Haupt, David E Geller, Jerry A Hall, Pedro M Quintana Diez
Exocrine pancreatic insufficiency (EPI), an important cause of maldigestion and malabsorption, results from primary pancreatic diseases or secondarily impaired exocrine pancreatic function. Besides cystic fibrosis and chronic pancreatitis, the most common etiologies of EPI, other causes of EPI include unresectable pancreatic cancer, metabolic diseases (diabetes); impaired hormonal stimulation of exocrine pancreatic secretion by cholecystokinin (CCK); celiac or inflammatory bowel disease (IBD) due to loss of intestinal brush border proteins; and gastrointestinal surgery (asynchrony between motor and secretory functions, impaired enteropancreatic feedback, and inadequate mixing of pancreatic secretions with food)...
October 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29090734/physical-exercise-training-for-cystic-fibrosis
#5
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/29068606/salivary-lipids-a-review
#6
REVIEW
Jan Matczuk, Małgorzata Żendzian-Piotrowska, Mateusz Maciejczyk, Krzysztof Kurek
Saliva is produced by both large and small salivary glands and may be considered one of the most important factors influencing the behavior of oral cavity homeostasis. Secretion of saliva plays an important role in numerous significant biological processes. Saliva facilitates chewing and bolus formation as well as performs protective functions and determines the buffering and antibacterial prosperities of the oral environment. Salivary lipids appear to be a very important component of saliva, as their qualitative and quantitative composition can be changed in various pathological states and human diseases...
September 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29056168/cystic-fibrosis-a-risk-condition-for-renal-disease
#7
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/29055982/vitamin-a-status-and-its-determinants-in-patients-with-cystic-fibrosis
#8
Ewa Sapiejka, Patrycja Krzyżanowska, Dariusz Walkowiak, Ewa Wenska-Chyży, Mariusz Szczepanik, Szczepan Cofta, Andrzej Pogorzelski, Wojciech Skorupa, Jarosław Walkowiak
BACKGROUND: Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. METHODS: In 196 CF patients aged from 4 months to 47 years, the following parameters  were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function - spirometry; biochemical markers of liver function - ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function - fecal elastase-1 concentration; blood clotting -  INR  and  vitamin  A supplementation)...
July 2017: Acta Scientiarum Polonorum. Technologia Alimentaria
https://www.readbyqxmd.com/read/29053438/tailored-approach-to-surgical-exposure-reduces-surgical-site-complications-after-bilateral-lung-transplantation
#9
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
#10
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
#11
Leslie S Satin, Vishal S Parekh
No abstract text is available yet for this article.
October 1, 2017: Endocrinology
https://www.readbyqxmd.com/read/28977595/glucose-sensitive-cftr-suppresses-glucagon-secretion-by-potentiating-katp-channels-in-pancreatic-islet-%C3%AE-cells
#12
Wen Qing Huang, Jing Hui Guo, Xiao Hu Zhang, Mei Kuen Yu, Yiu Wa Chung, Ye Chun Ruan, Hsiao Chang Chan
The secretion of glucagon by islet α cells is normally suppressed by high blood glucose, but this suppressibility is impaired in patients with diabetes or cystic fibrosis (CF), a disease caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), a cyclic adenosine monophosphate-activated Cl- channel. However, precisely how glucose regulates glucagon release remains controversial. Here we report that elevated glucagon secretion, together with increased glucose-induced membrane depolarization and Ca2+ response, is found in CFTR mutant (DF508) mice/islets compared with the wild-type...
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
#13
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/28969684/group-b-streptococcus-gbs-is-an-important-pathogen-in-human-disease-but-what-about-in-cystic-fibrosis
#14
Kate Skolnik, Austin Nguyen, Christina S Thornton, Barbara Waddell, Tyler Williamson, Harvey R Rabin, Michael D Parkins
BACKGROUND: Group B Streptococcus (GBS) is a common commensal capable of causing severe invasive infections. Most GBS infections occur in neonates (often as pneumonia). GBS can also cause infection in adults with diabetes and other immunological impairments but rarely leads to pneumonia in adults. GBS has occasionally been found in the sputum of Cystic Fibrosis (CF) patients, an inherited condition known for progressive lung disease. However, the epidemiology and clinical significance of GBS in CF are not understood...
October 2, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28941954/a-parental-report-of-youth-transition-readiness-the-parent-starx-questionnaire-starx-p-and-re-evaluation-of-the-starx-child-report
#15
Meaghan Nazareth, Laura Hart, Maria Ferris, Eniko Rak, Stephen Hooper, Miranda A L van Tilburg
PURPOSE: The STARx Questionnaire is a self-report measure of health care transition (HCT) readiness in youth with chronic diseases. We aimed to improve reliability and generalizability of the STARx and report initial reliability data on the STARx-P Questionnaire, a self-report measure of parent perspective on their child's HCT readiness. METHODS: Participants were recruited in several clinics from a large academic hospital in the southeastern USA and via the therapeutic summer camp for children with chronic disease...
September 20, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28938479/pulmonary-function-and-sleep-breathing-two-new-targets-for-type-2-diabetes-care
#16
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/28937998/selection-of-dna-aptamers-specific-for-live-pseudomonas-aeruginosa
#17
Jennifer Soundy, Darren Day
Pseudomonas aeruginosa is an opportunistic pathogen that causes significant morbidity and mortality in immunocompromised patients, particular cystic fibrosis sufferers, burns victims, diabetics and neonates. It thrives in moist places where it forms biofilms that are exceedingly difficult to eradicate on hospital surfaces, in water supplies and implanted biomaterials. Using a live cell SELEX approach we selected DNA aptamers to P. aeruginosa grown as biofilms in microfluidic cells. From a pool of aptamer candidates showing tight binding a stem-loop structure was identified as being important for binding...
2017: PloS One
https://www.readbyqxmd.com/read/28934665/venous-thromboembolism-in-children-with-cystic-fibrosis-retrospective-incidence-and-intrapopulation-risk-factors
#18
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
#19
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
#20
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
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