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Cystic fibrosis diabetes

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https://www.readbyqxmd.com/read/28727474/a-primer-on-exocrine-pancreatic-insufficiency-fat-malabsorption-and-fatty-acid-abnormalities
#1
Samer Alkaade, Ashley A Vareedayah
Exocrine pancreatic insufficiency (EPI) is characterized by a deficiency of exocrine pancreatic enzymes, resulting in deficits in digestion of all macronutrients, with deficiencies in digestion of fats being the most clinically relevant. The leading cause of EPI is chronic pancreatitis. However, many other causes and conditions may be implicated, including cystic fibrosis, pancreatic duct obstruction, gastric and pancreatic surgery, diabetes mellitus and other conditions. Physical and biochemical causes of EPI include decreased production and secretion of lipase, increased lipase destruction, pancreatic duct obstruction, decreased lipase stimulation and degradation, as well as gastrointestinal motility disorders...
July 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28714601/cf-related-diabetes-containing-the-metabolic-miscreant-of-cystic-fibrosis
#2
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
#3
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
#4
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 aged 30 years or younger, currently younger than 50 years, in two U...
July 12, 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
#5
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/28676554/diabetic-rats-present-higher-urinary-loss-of-proteins-and-lower-renal-expression-of-megalin-cubilin-clc-5-and-cftr
#6
Miriam F Figueira, Raquel C Castiglione, Carolina M de Lemos Barbosa, Felipe M Ornellas, Geórgia da Silva Feltran, Marcelo M Morales, Rodrigo N da Fonseca, Jackson de Souza-Menezes
Diabetic nephropathy (DN) occurs in around 40% of those with diabetes. Proteinuria is the main characteristic of DN and develops as a result of increased permeability of the glomerulus capillary wall and/or decreased proximal tubule endocytosis. The goal of this work was to evaluate renal function and the expression of megalin, cubilin, CFTR (cystic fibrosis transmembrane conductance regulator), and ClC-5 in the proximal tubule and renal cortex of rats with type 1 diabetes. Male Wistar rats were randomly assigned to control (CTRL) and diabetic (DM) groups for 4 weeks...
July 2017: Physiological Reports
https://www.readbyqxmd.com/read/28648506/the-role-of-glis3-in-thyroid-disease-as-part-of-a-multisystem-disorder
#7
REVIEW
P Dimitri
Congenital hypothyroidism is the most common hereditary endocrine disorder. In a small number of cases, mutations have been identified that are associated with maldevelopment and maldescent of the thyroid. Some of these mutations present as syndromes with a multisystem phenotype such as NKX2-1, PAX8, and FOXE. The association of permanent neonatal diabetes and congenital hypothyroidism was first reported in 2003 and subsequently led to the identification GLIS3 as the mutation responsible for this presentation...
March 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28648493/the-use-of-fructosamine-in-cystic-fibrosis-related-diabetes-cfrd-screening
#8
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
#9
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/28632196/drug-hypersensitivity-and-desensitizations-mechanisms-and-new-approaches
#10
REVIEW
Leticia de Las Vecillas Sánchez, Leila A Alenazy, Marlene Garcia-Neuer, Mariana C Castells
Drug hypersensitivity reactions (HSRs) are increasing in the 21st Century with the ever expanding availability of new therapeutic agents. Patients with cancer, chronic inflammatory diseases, cystic fibrosis, or diabetes can become allergic to their first line therapy after repeated exposures or through cross reactivity with environmental allergens. Avoidance of the offending allergenic drug may impact disease management, quality of life, and life expectancy. Precision medicine provides new tools for the understanding and management of hypersensitivity reactions (HSRs), as well as a personalized treatment approach for IgE (Immunoglobuline E) and non-IgE mediated HSRs with drug desensitization (DS)...
June 20, 2017: International Journal of Molecular Sciences
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...
June 15, 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/28605194/discovery-of-two-bacterial-nitric-oxide-responsive-proteins-and-their-roles-in-bacterial-biofilm-regulation
#13
Sajjad Hossain, Lisa-Marie Nisbett, Elizabeth M Boon
Bacterial biofilms form when bacteria adhere to a surface and produce an exopolysaccharide matrix ( Costerton Science 1999 , 284 , 1318 ; Davies Science 1998 , 280 , 295 ; Flemming Nat. Rev. Microbiol. 2010 , 8 , 623 ). Because biofilms are resistant to antibiotics, they are problematic in many aspects of human health and welfare, causing, for instance, persistent fouling of medical implants such as catheters and artificial joints ( Brunetto Chimia 2008 , 62 , 249 ). They are responsible for chronic infections in the lungs of cystic fibrosis patients and in open wounds, such as those associated with burns and diabetes...
June 12, 2017: Accounts of Chemical Research
https://www.readbyqxmd.com/read/28539697/physical-activity-levels-in-individuals-with-cystic-fibrosis-related-diabetes
#14
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
#15
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/28507430/investigating-the-feasibility-of-text-message-reminders-to-improve-adherence-to-nebulized-medication-in-children-and-adolescents-with-cystic-fibrosis
#16
Robert W Morton, Heather E Elphick, Elaine Edwards, William J Daw, Noreen S West
BACKGROUND: Children with cystic fibrosis (CF) often have suboptimal adherence rates to nebulized medication. Adherence barriers cited include forgetting to take the nebulizers, due to busy home and social lives. Text message reminders have been shown to be effective at improving adherence rates in other chronic diseases such as asthma and diabetes. OBJECTIVE: The objective of this study was to assess the feasibility and efficacy of sending text reminders for a prolonged period of time to children with CF...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/28502446/pancreatic-disorders
#17
REVIEW
Aliye Uc, Douglas S Fishman
Once considered uncommon, pancreatic diseases are increasingly recognized in the pediatric age group. Acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis occur in children with an incidence approaching that of adults. Risk factors are broad, prompting the need for a completely different diagnostic and therapeutic approach in children. Although cystic fibrosis remains the most common cause of exocrine pancreatic insufficiency, other causes such as chronic pancreatitis may be as common as Shwachman Diamond syndrome...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28499359/cystic-fibrosis-carriership-and-tuberculosis-hints-toward-an-evolutionary-selective-advantage-based-on-data-from-the-brazilian-territory
#18
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
#19
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
#20
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
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