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Cystic fibrosis diabetes

Iris K Minichmayr, André Schaeftlein, Joseph L Kuti, Markus Zeitlinger, Charlotte Kloft
OBJECTIVES: We aimed to assess linezolid pharmacokinetics in the plasma and interstitial space fluid (ISF) of patients with sepsis, diabetic foot infections or cystic fibrosis and healthy volunteers. The impacts of joint characteristics and disease on plasma and target-site exposure were to be identified together with the benefit of dose intensification in critically ill patients. METHODS: Rich plasma (n = 1598) and ISF concentrations in subcutaneous adipose (n = 1430) and muscle tissue (n = 1089) measured by microdialysis were pooled from three clinical trials with 51 individuals receiving 600 mg of intravenous and oral linezolid...
October 17, 2016: Clinical Pharmacokinetics
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
Aliye Uc, Dana K Andersen, Melena D Bellin, Jason I Bruce, Asbjørn M Drewes, John F Engelhardt, Christopher E Forsmark, Markus M Lerch, Mark E Lowe, Brent A Neuschwander-Tetri, Stephen J OʼKeefe, Tonya M Palermo, Pankaj Pasricha, Ashok K Saluja, Vikesh K Singh, Eva M Szigethy, David C Whitcomb, Dhiraj Yadav, Darwin L Conwell
A workshop was sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases to focus on research gaps and opportunities in chronic pancreatitis (CP) and its sequelae. This conference marked the 20th year anniversary of the discovery of the cationic trypsinogen (PRSS1) gene mutation for hereditary pancreatitis. The event was held on July 27, 2016, and structured into 4 sessions: (1) pathophysiology, (2) exocrine complications, (3) endocrine complications, and (4) pain. The current state of knowledge was reviewed; many knowledge gaps and research needs were identified that require further investigation...
November 2016: Pancreas
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
Nadia Beaudoin, Guillaume F Bouvet, Adèle Coriati, Rémi Rabasa-Lhoret, Yves Berthiaume
PURPOSE: Glucose abnormality and diabetes are the most common comorbidities in cystic fibrosis (CF). Combined (aerobic & resistance) exercise program in type 2 diabetes patients demonstrated an improvement of glycemic control. The aim of the study was to determine if a combined exercise program is beneficial to improve plasma glucose at 2-h of the oral glucose tolerance test (OGTT) in CF. METHOD: 18 adult with CF with glucose abnormality were recruited (Clinicaltrial...
September 23, 2016: Medicine and Science in Sports and Exercise
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
Jesica Pagano-Therrien, Susan Sullivan-Bolyai
The aims of this qualitative descriptive study were to describe how past experiences with research (including communication, information, values, and support) may contribute to research fatigue among youth and parents of youth with HIV, cystic fibrosis, and Type 1 diabetes. Eighteen parents and youth were purposively recruited from outpatient subspecialty clinics at a major academic medical center. They took part in qualitative interviews and completed a demographics form and the Decisional Conflict Scale. Youth participants also completed the Erikson Psychosocial Stage Inventory...
August 20, 2016: Journal of Pediatric Health Care
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
Saba Sheikh, Lalitha Gudipaty, Diva D De Leon, Denis Hadjiliadis, Christina Kubrak, Nora K Rosenfeld, Sarah Nyirjesy, Amy Pelekis, Saloni Malik, Darko Stefanovski, Marina Cuchel, Ronald C Rubenstein, Andrea Kelly, Michael R Rickels
Patients with pancreatic insufficient cystic fibrosis (PI-CF) are at increased risk for developing diabetes. We determined β-cell secretory capacity and insulin secretory rates from glucose-potentiated arginine and mixed meal tolerance tests (MMTT), respectively, in pancreatic sufficient cystic fibrosis (PS-CF), PI-CF, and normal control subjects, all with normal glucose tolerance, in order to identify early pathophysiologic defects. Acute islet cell secretory responses were determined under fasting, 230 mg/dL, and 340 mg/dL hyperglycemia clamp conditions...
August 5, 2016: Diabetes
Sasiharan Sithamparanathan, Logan Thirugnanasothy, Stephen Clark, John H Dark, Andrew J Fisher, Kate F Gould, Asif Hasan, James L Lordan, Gerard Meachery, Gareth Parry, Paul A Corris
BACKGROUND: Lung transplant recipients have reduced long-term survival compared with other solid organ recipients. There is a lack of published data on the characteristics of very long term survivors. METHODS: We describe the demographics, clinical history and post-procedure function of all lung transplant recipients who have survived greater than 20 years at our centre. RESULTS: At the time of analysis there were 21 (16.4%) of 128 patients who survived over 20 years...
August 2016: Respiratory Medicine
A D Lalayiannis, C Thompson, R Malcomson, D V Milford
BACKGROUND: Nodular glomerulosclerosis is seen in insulin dependent diabetic patients with nephropathy. Kimmelstiel-Wilson nodules on biopsy are considered pathognomonic. Diabetic nephropathy is a spectrum of glomerular and tubular disease which correlates with the duration of the diabetes and the extent of glycaemic control. CASE REPORT: An eleven year old girl with cystic fibrosis was referred with persistent heavy proteinuria. She underwent a renal biopsy which revealed nodular glomerulosclerosis with Kimmelstiel-Wilson-like nodules...
2016: Respiratory Medicine Case Reports
Kris English, Emily Pajevic
In 2007, the World Health Organization published a set of International Classification of Functioning, Disability and Health (ICF) codes designed for children and youth (ICF-CY version). The ICF-CY considers typical developmental changes associated with childhood while describing health status and the effects of intervention. In this article we will describe how a specific intervention (transition planning for adolescents) can be documented with the ICF-CY. Transition planning in health care prepares adolescents and their families for the transfer from pediatric to adult health services and has been demonstrated to be an effective practice for adolescents with many types of chronic health conditions (e...
August 2016: Seminars in Hearing
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
Esther Nasuuna, Giuseppe Santoro, Peter Kremer, Andrea M de Silva
AIM: Chronic health conditions are associated with poor academic outcomes. This study examines the relationship between health conditions, specialist health service utilisation and academic performance in Australian children. METHODS: This was a quasi-longitudinal study where School Entrant Health Questionnaire (a survey tool with parent report on children's health) data for 24 678 children entering school in 2008 was matched with the 2011 National Assessment Program - Literacy and Numeracy (NAPLAN)...
July 2016: Journal of Paediatrics and Child Health
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