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Cystic fibrosis related diabetes

Gregory C Jones, Christopher A R Sainsbury
Cystic fibrosis is a common genetic condition and abnormal glucose handling leading to cystic fibrosis-related diabetes (CFRD) is a frequent comorbidity. CFRD is mainly thought to be the result of progressive pancreatic damage resulting in beta cell dysfunction and loss of insulin secretion. Whilst Oral Glucose Tolerance Testing is still recommended for diagnosing CFRD, the relationship between glucose abnormalities and adverse outcomes in CF is complex and occurs at stages of dysglycaemia occurring prior to diagnosis of diabetes by World Health Organisation criteria...
October 17, 2016: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
Danièle Pacaud, Anke Schwandt, Carine de Beaufort, Kristina Casteels, Jacques Beltrand, Niels H Birkebaek, Myrna Campagnoli, Natasa Bratina, Catarina Limbert, Stephen Mp O'Riordan, Rogério Ribeiro, Andriani Gerasimidi-Vazeou, Lenka Petruzelkova, Rasa Verkauskiene, Iveta Dzivite Krisane
BACKGROUND: Although type 1 diabetes (T1D) remains the most frequent form of diabetes in individuals aged less than 20 years at onset, other forms of diabetes are being increasingly recognized. OBJECTIVES: To describe the population of children with other forms of diabetes (non-type 1) included in the multinational SWEET (Better control in Pediatric and Adolescent diabeteS: Working to crEate CEnTers of Reference) database for children with diabetes. METHODS: Cases entered in the SWEET database are identified by their physician as T1D, type 2 diabetes (T2D) and other types of diabetes according to the ISPAD classification...
October 2016: Pediatric Diabetes
Valérie Boudreau, Quitterie Reynaud, Catherine Lehoux Dubois, Adèle Coriati, Katherine Desjardins, Isabelle Durieu, Rémi Rabasa-Lhoret
Nearly 50% of adult patients with cystic fibrosis (CF) have diabetes. The occurrence of CF-related diabetes (CFRD) is preceded and is associated with deterioration of lung function and nutritional status. Microvascular complications can occur, but the main cause of death is respiratory failure rather than cardiovascular causes as in type 1 or type 2 diabetes. Because other methods such as glycated hemoglobin (A1C) levels are less sensitive in patients with CF, the recommended screening test is the oral glucose tolerance test (OGTT) with a 75 g glucose dose...
October 2016: Canadian Journal of Diabetes
Carlo Castellani, Baroukh M Assael
Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude...
October 5, 2016: Cellular and Molecular Life Sciences: CMLS
Luisa Montanini, Arianna Smerieri, Mariolina Gullì, Francesca Cirillo, Giovanna Pisi, Chiara Sartori, Sergio Amarri, Sergio Bernasconi, Nelson Marmiroli, Maria E Street
CONTEXT: Cystic Fibrosis Related Diabetes (CFRD) is the most frequent and severe co-morbidity in CF. Presentation and severity are quite variable. OBJECTIVE: To investigate changes in miRNAs due to CFTR malfunctioning in vitro and to study the circulating levels of selected miRNAs in serum samples from patients, and assess their relationships in different age-groups with genotype, glucose tolerance state and at onset of CFRD. Design/Setting/Patients/Interventions: Transcriptional profiling of all known miRNAs in CFBE41o- cells, in their normal counterparts (16HBE14o- cells), and in IB3 cells was performed...
September 30, 2016: Journal of Clinical Endocrinology and Metabolism
A E While, E Heery, A M Sheehan, I Coyne
BACKGROUND: The numbers of children with long-term illnesses surviving into adulthood and transferring from child to adult services has increased dramatically in the last 30 years. This study aimed to examine health-related quality of life pre- and post-transfer from child to adult healthcare for young people with three long-term illnesses. METHODS: A total of 217 young people with cystic fibrosis, congenital heart defects or diabetes attending child and adult hospital services in Dublin, Ireland completed a questionnaire survey...
September 27, 2016: Child: Care, Health and Development
Roy J Kim
Cystic fibrosis-related diabetes mellitus (CFRD) is the most common endocrine complication of cystic fibrosis (CF), affecting more than 50% of patients by the 4th decade of life. CFRD is often preceded by worsening pulmonary status and nutritional decline. Treatment of CFRD is associated with improvements in body weight and pulmonary function and a reduction in pulmonary exacerbations. Because of the clinical significance of CFRD, diabetes screening with an oral glucose tolerance test (OGTT) is recommended annually for all patients with CF starting at age 10 years...
September 1, 2016: Pediatric Annals
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
Gregory C Jones, Zhou M Chong, Jennifer Gilmour, Christine Matheson, Gordon MacGregor, Christopher A R Sainsbury
INTRODUCTION: Mortality in patients with cystic fibrosis-related diabetes (CFRD) is higher than that in patients with cystic fibrosis without diabetes. Hypoglycemia, hyperglycemia, and glucose variability confer excess mortality and morbidity in the general inpatient population with diabetes. METHODS: We investigated patterns of hypoglycemia and the association of hypoglycemia, hyperglycemia, and glucose variability with mortality and readmission rate in inpatients with CFRD...
September 2016: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
Scott M Blackman, Vin Tangpricha
Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF...
August 2016: Pediatric Clinics of North America
Yaling Yi, Andrew W Norris, Kai Wang, Xingshen Sun, Aliye Uc, Antoinette Moran, John F Engelhardt, Katie Larson Ode
RATIONALE: In cystic fibrosis, abnormal glucose tolerance is associated with decreased lung function and worsened outcomes. Translational evidence indicates that abnormal glucose tolerance may begin in early life. OBJECTIVES: To determine whether very young children with cystic fibrosis have increased abnormal glucose tolerance prevalence compared with control subjects. The secondary objective was to compare area under the curve for glucose and insulin in children with cystic fibrosis with control subjects...
October 15, 2016: American Journal of Respiratory and Critical Care Medicine
Sophie Ziai, Adèle Coriati, David St-Pierre, Katherine Chabot, Katherine Desjardins, Catherine Leroux, Martin V Richter, Rémi Rabasa-Lhoret
OBJECTIVES: To determine the modifiable factors affecting glucose variability in people with cystic fibrosis (CF). CF-related diabetes (CFRD) is the most common complication of CF, and its presence increases morbidity and mortality in patients. Patients with CF (with and without CFRD) have potentially harmful glucose fluctuations and glucose excursions when compared to healthy adults. Carbohydrate intake and exercise have been shown to affect glycemia. Therefore, our hypothesis was that the proportion of energy from carbohydrates and total energy expenditure (TEE) would influence glucose fluctuations in adults with CF...
July 7, 2016: Canadian Journal of Diabetes
G Belle-van Meerkerk, H W de Valk, M C Stam-Slob, F Teding van Berkhout, P Zanen, E A van de Graaf
AIMS: Pulmonary infections are more frequent in and associated with higher mortality in Cystic Fibrosis-Related Diabetes (CFRD) patients compared to CF patients without CFRD. Hyperglycaemia can lead to a higher vulnerability for infections. Aim of the study was to test whether the infection rate in well-controlled CFRD patients was similar to that in CF patients without CFRD. METHODS: This is a retrospective six-year cohort analysis on a consecutive series of 138 CF patients...
June 2016: Diabetes Research and Clinical Practice
Katja Konrad, Thomas Kapellen, Eggert Lilienthal, Nicole Prinz, Maria Bauer, Angelika Thon, Ernst Rietschel, Dagobert Wiemann, Reinhard W Holl
OBJECTIVE: Research on β-cell autoimmunity in cystic fibrosis (CF)-related diabetes (CFRD) is still rare. We aimed to analyze the frequency of β-cell autoimmunity and the influence on age at diabetes onset, insulin requirement, type of insulin therapy, and hypoglycemic or ketoacidotic events in patients with CFRD compared with antibody-negative patients with CFRD in the Diabetes Patienten Verlaufsdokumentation (DPV) registry. RESEARCH DESIGN AND METHODS: We analyzed data of 837 patients with CFRD in the German/Austrian DPV database by multivariable mixed-regression modeling...
August 2016: Diabetes Care
Bernadette Prentice, Shihab Hameed, Charles F Verge, Chee Y Ooi, Adam Jaffe, John Widger
INTRODUCTION: Cystic fibrosis-related diabetes (CFRD) is the end-point of a spectrum of glucose abnormalities in cystic fibrosis that begins with early insulin deficiency and ultimately results in accelerated nutritional decline and loss of lung function. Current diagnostic and management regimens are unable to entirely reverse this clinical decline. AREAS COVERED: This review summarises the current understanding of the pathophysiology of CFRD, the issues associated with using oral glucose tolerance tests in CF and the challenges faced in making the diagnosis of CFRD...
July 2016: Expert Review of Respiratory Medicine
Sharon Levy, Fatma Dedeoglu, Jonathan M Gaffin, Katharine C Garvey, Elizabeth Harstad, Andrew MacGinnitie, Paul A Rufo, Qian Huang, Rosemary E Ziemnik, Lauren E Wisk, Elissa R Weitzman
BACKGROUND: In an effort to reduce barriers to screening for alcohol use in pediatric primary care, the National Institute on Alcoholism and Alcohol Abuse (NIAAA) developed a two-question Youth Alcohol Screening Tool derived from population-based survey data. It is unknown whether this screening tool, designed for use with general populations, accurately identifies risk among youth with chronic medical conditions (YCMC). This growing population, which comprises nearly one in four youth in the US, faces a unique constellation of drinking-related risks...
2016: PloS One
Małgorzata Olszowiec-Chlebna, Agnieszka Koniarek-Maniecka, Włodzimierz Stelmach, Katarzyna Smejda, Joanna Jerzyńska, Paweł Majak, Monika Białas, Iwona Stelmach
INTRODUCTION: Severity of lung disease varies in patients with the same CFTR genotype. It suggests that other factors affect the severity of cystic fibrosis (CF). The aim of the study was to identify risk factors that determine lung function decline in Polish cystic fibrosis children. MATERIAL AND METHODS: The follow-up time was no less than 5 years of respiratory status observation based on the forced expiratory volume in 1 s value (FEV1). The socio-economic data, perinatal interview, presence of meconium ileus (MI), time of CF diagnosis, initiation of tobramycin inhalation solution (TIS), pancreatic function, sensitization to Aspergillus fumigatus, presence of impaired glucose tolerance (IGT) or diabetes mellitus, chronic bacterial colonization and number of exacerbations and hospitalizations were assessed...
April 1, 2016: Archives of Medical Science: AMS
Theodore G Liou, Judith L Jensen, Sarah E Allen, Sara J Brayshaw, Mark A Brown, Barbara Chatfield, Joni Koenig, Catherine McDonald, Kristyn A Packer, Kimberly Peet, Peggy Radford, Linda M Reineke, Kim Otsuka, Jeffrey S Wagener, David Young, Bruce C Marshall
OBJECTIVE: Cystic fibrosis (CF)-related diabetes (CFRD) is associated with increased morbidity and mortality. Improved detection and management may improve outcomes; however, actual practice falls short of published guidelines. We studied efforts to improve CFRD screening and management in the Mountain West CF Consortium (MWCFC). RESEARCH DESIGN AND METHODS: This is a prospective observational cohort study evaluating quality improvement by accredited CF centers in Arizona, Colorado, New Mexico, and Utah performed between 2002 and 2008...
2016: BMJ Open Diabetes Research & Care
Huaqiong Zhou, Pamela Roberts, Satvinder Dhaliwal, Phillip Della
AIMS AND OBJECTIVES: This paper aims to provide an updated comprehensive review of the research-based evidence related to the transitions of care process for adolescents and young adults with chronic illness/disabilities since 2010. BACKGROUND: Transitioning adolescent and young adults with chronic disease and/or disabilities to adult care services is a complex process, which requires coordination and continuity of health care. The quality of the transition process not only impacts on special health care needs of the patients, but also their psychosocial development...
May 5, 2016: Journal of Clinical Nursing
Valérie Boudreau, Adèle Coriati, Imane Hammana, Sophie Ziai, Katherine Desjardins, Yves Berthiaume, Rémi Rabasa-Lhoret
BACKGROUND: Reduced insulin secretion is a key factor to explain high prevalence of glucose intolerance in patients with cystic fibrosis (CF). However, the role of insulin sensitivity remains unclear. The aim of this study is to investigate the association of insulin secretion and sensitivity with the evolution of glucose tolerance. METHODS: A total of 152 patients without known diabetes from the Montreal CF cohort underwent two 2-h oral glucose tolerance tests (OGTT) at baseline and again after 21...
April 29, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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