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Cystic fibrosis

Paola Catastini, Serena Di Marco, Maria Furriolo, Carmela Genovese, Alessia Grande, Eugenia Iacinti, Rosa Danila Iusco, Rita Maria Vittoria Nobili, Rita Pescini, Roberto Ragni, Roberto Randazzo, Cristiana Risso, Paola Tabarini, Cesare Braggion, Salvatore De Masi, Kathleen S McGreevy
BACKGROUND: Cystic fibrosis, like other chronic diseases, is a risk factor for the development of elevated symptoms of depression and anxiety. The objective of this study was to investigate the prevalence of anxiety and depression in Italian patients with CF and their parents. METHODS: The Hospital Anxiety and Depression Scale (HADS) and Center for Epidemiologic Studies Depression Scale (CES-D) questionnaires were administered to a sample of patients and their parents recruited at the cystic fibrosis centers in Italy...
October 19, 2016: Pediatric Pulmonology
Ping Wei, Jia-Wei Yang, Hai-Wen Lu, Bei Mao, Wen-Lan Yang, Jin-Fu Xu
BACKGROUND AND OBJECTIVE: There is presently no clear evidence on the effect of combined treatment for non-cystic fibrosis (non-CF) bronchiectasis with inhaled corticosteroid (ICS) and long-acting β2-adrenergic agonist (LABA). The objective of this study is to assess the efficacy and safety of salmeterol-fluticasone combined inhaled therapy for non-CF bronchiectasis with airflow limitation. METHODS: An observational study was performed in 120 non-CF bronchiectasis patients diagnosed by high-resolution computed tomography (HRCT) scanning of the chest...
October 2016: Medicine (Baltimore)
Suzanne Drury, Sarah Mason, Fiona McKay, Kitty Lo, Christopher Boustred, Lucy Jenkins, Lyn S Chitty
Our UK National Health Service regional genetics laboratory offers NIPD for autosomal dominant and de novo conditions (achondroplasia, thanataphoric dysplasia, Apert syndrome), paternal mutation exclusion for cystic fibrosis and a range of bespoke tests. NIPD avoids the risks associated with invasive testing, making prenatal diagnosis more accessible to families at high genetic risk. However, the challenge remains in offering definitive diagnosis for autosomal recessive diseases, which is complicated by the predominance of the maternal mutant allele in the cell-free DNA sample and thus requires a variety of different approaches...
2016: Advances in Experimental Medicine and Biology
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
Vladislav Raclavsky, Nikola Stromerova, Dana Safarova, Jan Bardon, Jaromir Zatloukal, Martin Zapalka, Petr Jakubec, Lucie Navratilova, Radko Novotny
BACKGROUND AND AIMS: Haemophilus influenzae new strain acquisition has been demonstrated to increase the relative risk of acute exacerbation fourfold in contrast to colonisation or chronic infection by the same strain in chronic obstructive pulmonary disease (COPD). Unfortunately, molecular typing techniques are not suitable for routine use due to cost, labour-intensity and need for special expertise. We tested two techniques potentially useful for routine typing, namely the newly available MALDI-TOF MS and the modified McRAPD compared to MLST as the gold standard...
October 6, 2016: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
Dean G Assimos
No abstract text is available yet for this article.
November 2016: Journal of Urology
Mauricio A Salinas-Santander, Elizabeth Bazan-Mendoza, Marisol Espinoza-Ruiz, Rocio Ortiz-Lopez, Adriana Bustamante, Celia N Sanchez-Dominguez
Cystic fibrosis (CF) is the most common autosomal recessive disease in the Caucasian population, but it has also been widely diagnosed in the Mexican population. Production of viscous secretions affects the secretory epithelia and the respiratory condition usually leads to death. The relationship between the CFTR genotype and the disease phenotype is not well understood. Other risk factors such as genetic and autoimmune influence the development of this disease. We analyzed the PTPN22 R620W polymorphism (+1858 C/T, rs2476601) in 78 DNA samples from CF patients and 232 healthy controls from northeast Mexico using the polymerase chain reaction-restriction fragment length (PCR-RFLP) method...
July 2016: Archives of Medical Research
Traci M Kazmerski, Daniel J Weiner, Janice Matisko, Diane Schachner, Whitney Lerch, Carol May, Scott H Maurer
INTRODUCTION: Advance care planning (ACP), though recommended, has not been studied in adolescents with cystic fibrosis (CF). This quality improvement project engaged adolescents with advanced CF disease in ACP and assessed patient and CF provider attitudes and preferences regarding ACP discussions and tools. MATERIALS AND METHODS: Patients ≤22 years with advanced CF (FEV1 ≤40% predicted, >2 pulmonary exacerbations requiring IV antibiotics in 1 year, and/or use of home oxygen or non-invasive ventilation) were referred to the pediatric palliative care team (PC)...
October 17, 2016: Pediatric Pulmonology
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
Kenneth N Olivier, David E Griffith, Gina Eagle, John P McGinnis Ii, Liza Micioni, Keith Liu, Charles L Daley, Kevin L Winthrop, Stephen Ruoss, Doreen J Addrizzo-Harris, Patrick A Flume, Daniel Dorgan, Matthias Salathe, Barbara A Brown-Elliott, Renu Gupta, Richard J Wallace
Rationale Lengthy multi-drug, toxic, and low efficacy regimens limit management of pulmonary nontuberculous mycobacterial (PNTM) disease. Objective This phase 2 study investigated efficacy and safety of liposomal amikacin for inhalation (LAI) in treatment-refractory PNTM (Mycobacterium avium complex [MAC] or Mycobacterium abscessus) disease. Methods During the double-blind phase, patients were randomly assigned to LAI (590 mg) or placebo once daily added to their multi-drug regimen for 84 days. Both groups could receive open-label LAI for 84 additional days...
October 17, 2016: American Journal of Respiratory and Critical Care Medicine
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
Constantina Boikos, Lawrence Joseph, Christine Martineau, Jesse Papenburg, David Scheifele, Larry C Lands, Gaston De Serres, Mark Chilvers, Caroline Quach
Background.  We aimed to explore the detection profile of influenza viruses following live-attenuated intranasal influenza vaccination (LAIV) in children aged 2-19 years with and without cystic fibrosis (CF). Methods.  Before the 2013-2014 influenza season, flocked nasal swabs were obtained before vaccination and 4 times in the week of follow-up from 76 participants (nCF: 57; nhealthy: 19). Influenza was detected by reverse transcription polymerase chain reaction (RT-PCR) assays. A Bayesian hierarchical logistic regression model was used to estimate the effect of CF status and age on influenza detection...
October 2016: Open Forum Infectious Diseases
Arianna Pompilio, Valentina Crocetta, Dipankar Ghosh, Malabika Chakrabarti, Giovanni Gherardi, Luca Agostino Vitali, Ersilia Fiscarelli, Giovanni Di Bonaventura
The present study was carried out to understand the adaptive strategies developed by Stenotrophomonas maltophilia for chronic colonization of the cystic fibrosis (CF) lung. For this purpose, 13 temporally isolated strains from a single CF patient chronically infected over a 10-year period were systematically characterized for growth rate, biofilm formation, motility, mutation frequencies, antibiotic resistance, and pathogenicity. Pulsed-field gel electrophoresis (PFGE) showed over time the presence of two distinct groups, each consisting of two different pulsotypes...
2016: Frontiers in Microbiology
Richard L Blackmon, Silvia M Kreda, Patrick R Sears, Lawrence E Ostrowski, David B Hill, Brian S Chapman, Joseph B Tracy, Amy L Oldenburg
Mucus hydration (wt%) has become an increasingly useful metric in real-time assessment of respiratory health in diseases like cystic fibrosis and COPD, with higher wt% indicative of diseased states. However, available in vivo rheological techniques are lacking. Gold nanorods (GNRs) are attractive biological probes whose diffusion through tissue is sensitive to the correlation length of comprising biopolymers. Through employment of dynamic light scattering theory on OCT signals from GNRs, we find that weakly-constrained GNR diffusion predictably decreases with increasing wt% (more disease-like) mucus...
2016: Proceedings of SPIE
Shinya Kawamoto, Ryo Koda, Atsunori Yoshino, Tetsuro Takeda, Yoshihiko Ueda
Medullary cystic kidney disease (MCKD) is a hereditary disease associated with bilateral medullary polycysts and interstitial fibrosis. MCKD is typically associated with slowly progressive renal dysfunction. We herein report two rare elderly cases with enlarged kidneys and rapidly progressive renal dysfunction without myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), PR3-ANCA, or anti-glomerular basement membrane (GBM) antibodies. Renal biopsies revealed extensive tubular dilatation and atrophy with interstitial fibrosis consistent with MCKD...
2016: Internal Medicine
Isabelle Sermet-Gaudelus, Martial Delion, Isabelle Durieu, Jacky Jacquot, Dominique Hubert
Low bone mineral density (BMD) is a common problem in adults with cystic fibrosis (CF), the etiology of which is multifactorial. In this study, we provide the first evidence that ivacaftor improves BMD in CF patients carrying the p.Gly551Asp mutation. Consistently, in vitro experiments with TNF-α-stimulated primary F508del-CFTR osteoblasts demonstrated that correction of p.Phe508del-CFTR markedly decreased RANKL protein production, a major factor of bone resorption. These clinical and fundamental observations suggest that rescue of mutated CFTR protein improves bone remodeling and support the link between CFTR and bone cell physiology...
October 10, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
K De Boeck, A Zolin
In patients with cystic fibrosis, most treatments addressing the underlying basic defect are mutation or mutation class specific. These treatments are disease modifying if they lower the year to year change in lung function. We therefore calculated the current loss of lung function, measured by year to year change in forced expired volume in 1s in 11,417 patients included in the European Cystic Fibrosis Society Patient Registry. Whereas patients with at least one mutation of class IV or V have on average a lower year to year change, we did not find a difference between patients with a stop codon mutation, homozygous for F508del or at least one class III mutation...
October 11, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Grace Hubert, Theresa Tam Chung, Connie Prosser, Dale Lien, Justin Weinkauf, Neil Brown, Marianne Goodvin, Kathy Jackson, Joan Tabak, Josette Salgado, Abeer Salman Alzaben, Diana R Mager
PURPOSE: Patients with cystic fibrosis (CF) often experience low bone mineral density (BMD) pre- and post-lung transplantation (LTX). The study purpose was to describe BMD and micronutrient status in adults with CF pre- and post-LTX. METHODS: Twelve patients with CF (29 ± 8 years) were recruited from the CF clinic at the University of Alberta Lung Transplant Program. BMD and vitamins A, D, E, K status, and parathyroid hormone were measured pre- and post-LTX. RESULTS: No significant differences pre- and post-LTX were observed at the different bone sites measured (lumber-spine, femoral-neck (FN), hip, and femoral-trochlea) (P > 0...
July 19, 2016: Canadian Journal of Dietetic Practice and Research
Ritesh Agarwal, Inderpaul S Sehgal, Sahajal Dhooria, Ashutosh N Aggarwal
Allergic bronchopulmonary aspergillosis (ABPA) is a complex pulmonary disorder characterized by recurrent episodes of wheezing, fleeting pulmonary opacities and bronchiectasis. It is the most prevalent of the Aspergillus disorders with an estimated five million cases worldwide. Despite six decades of research, the pathogenesis, diagnosis and treatment of this condition remains controversial. The International Society for Human and Animal Mycology has formed a working group to resolve the controversies around this entity...
October 17, 2016: Expert Review of Respiratory Medicine
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