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Cystic fibrosis

Xiao Wei, Zongshi Lu, Tao Yang, Peng Gao, Sijiao Chen, Daoyan Liu, Zhiming Zhu
BACKGROUND/AIMS: High salt consumption is a major risk factor for hypertension, and sodium homeostasis is regulated by both intestinal sodium absorption and urinary sodium excretion. Chronic caffeine intake has been reported to attenuate salt-sensitive hypertension by promoting urinary sodium excretion; however, its exact role in intestinal sodium absorption remains unknown. Here, we investigated whether and how chronic caffeine consumption antagonizes salt-sensitive hypertension by inhibiting intestinal sodium absorption...
March 16, 2018: Kidney & Blood Pressure Research
Tamer Sekmenli, Metin Gündüz, Hikmet Akbulut, H Haldun Emiroglu, Mustafa Koplay, Ilhan Ciftci
Percutaneous endoscopic gastrostomy (PEG) is used as an alternative to enteral/nasoenteral feeding in situations where long-term oral feeding is ineffective or not tolerated. It is mostly preferred in patients with neurological conditions and also to support nutrition in patients with congenital heart diseases, cystic fibrosis, inflammatory bowel disease, and various oropharyngeal diseases. Although it is easily applicable compared to many invasive procedures, it has complications ranging from wound infection to death...
April 1, 2018: Archivos Argentinos de Pediatría
Candice Bjornson, Parco Chan, Abby Li, Bosco Paes, Krista L Lanctôt, Ian Mitchell
Respiratory syncytial virus (RSV) infection in cystic fibrosis (CF) infants is associated with significant morbidities. This study's objective is to evaluate the effectiveness and adverse events related to palivizumab (PVZ) in CF infants. Data on respiratory-related illness (RIH) and RSV hospitalizations (RSVH) were collected retrospectively in CF infants aged < 2 years in Alberta, Canada, from 2000 to 2017. Logistic regression models were used to compare the odds of RSVH or RIH in PVZ infants from the Canadian registry of palivizumab (CARESS) versus untreated (UPVZ) infants from Alberta, after adjusting for potential confounders...
March 19, 2018: European Journal of Clinical Microbiology & Infectious Diseases
K Schneider
The radiographic technique of pediatric chest X‑rays is substantially different from that in adults. In nearly all cases ap/pa X‑rays are sufficient and lateral radiographs are rarely needed. In the first years of life the thymus may overshadow the heart, the great vessels and the lung hila. The most important anatomical structures essential for diagnosing pathological findings of the lungs and mediastinum are the trachea with the bifurcation and the main bronchi with the adjacent great vessels. For the assessment of distended lungs and intrathoracic consolidations, fundamental knowledge of the anatomy in childhood and malformations which can involve the airways, the lungs, the heart, as well as systemic and pulmonary vessels are indispensable...
March 19, 2018: Der Radiologe
Emily K Cope
Despite growing efforts to understand the role of the microbiota in airway disease, mechanisms that link microbial community dysbiosis to chronic inflammation remain elusive. Our laboratory is interested in how altered microbiota composition or function influences airway inflammatory diseases, including chronic rhinosinusitis, asthma, and cystic fibrosis. Given the tight interplay between host-associated microbes and host immunity, the potential for translational microbiome research to guide clinical decisions and novel therapeutics is becoming better appreciated...
March 2018: MSystems
Xia Hou, Hongguang Wei, Carthic Rajagopalan, Hong Jiang, Qingtian Wu, Khalequz Zaman, Youming Xie, Fei Sun
Endoplasmic reticulum (ER)-associated protein degradation (ERAD) is an important quality control mechanism that eliminates misfolded proteins from the ER. The Derlin-1/VCP/VIMP protein complex plays an essential role in ERAD. Although the roles of Derlin-1 and VCP are relatively clear, the functional activity of VIMP in ERAD remains to be understood. Here we investigate the role of VIMP in the degradation of CFTRΔF508, a cystic fibrosis transmembrane conductance regulator (CFTR) mutant known to be a substrate of ERAD...
March 19, 2018: Scientific Reports
Thomas Gaisl, Lukas Bregy, Nina Stebler, Martin Thomas Gaugg, Tobias Bruderer, Diego García-Gómez, Alexander Moeller, Florian Singer, Esther Irene Schwarz, Christian Benden, Pablo M-L Sinues, Renato Zenobi, Malcolm Kohler
We aimed at defining profiles of volatile organic compounds in exhaled breath from patients with cystic fibrosis (CF) using a novel real-time mass spectrometry technique.&#13; In this prospective matched case-control study, 30 patients with CF, and 30 healthy control subjects were matched one-to-one according to age, gender, and smoking state. We performed exhaled breath analysis by untargeted secondary electrospray ionization-high resolution mass spectrometry (SESI-HRMS). &#13; Patients with CF (mean age 26...
March 20, 2018: Journal of Breath Research
Mohamed Salla, Rodrigo Aguayo-Ortiz, Danmaliki Gaddafi Ibrahim, Alaa Zare, Ahmed Said, Jack Moore, Vrajeshkumar Pandya, Robin Manaloor, Sunny Fong, Anna R Blankstein, Spencer Gibson, Laura Ramos Garcia, Pascal Meier, Khushwant S Bhullar, Basil P Hubbard, Yahya Fiteh, Harissios Vliagoftis, Ing Swie Goping, Dion Brocks, Peter Hwang, Jose Carlos A Martinez Velazquez, Shairaz Baksh
Receptor interacting protein kinase 2 (RIP2 or RICK herein referred to as RIPK2) is linked to the pathogen pathway that activates NFkB and autophagic activation. Using molecular modeling (docking) and chemoinformatics analyses we utilized the RIPK2/ponatinib crystal structure and searched in chemical databases for small molecules exerting binding interactions similar to those exerted by ponatinib. The identified RIPK2 inhibitors potently inhibited the proliferation of cancer cells by > 70% as well as inhibition of NFkB activity...
March 19, 2018: Journal of Pharmacology and Experimental Therapeutics
Francesca Forti, Dwayne R Roach, Marco Cafora, Maria E Pasini, David S Horner, Ersilia V Fiscarelli, Martina Rossitto, Lisa Cariani, Federica Briani, Laurent Debarbieux, Daniela Ghisotti
The alarming diffusion of multidrug resistant (MDR) bacterial strains requires investigations on non-antibiotic therapies. Amongst them, the use of bacteriophages (phages) as antimicrobial agents, namely phage therapy, is a promising treatment strategy with support by recent successful compassionate treatments in Europe and the U.S.A. In this work, we combined host range and genomic information to design a 6-phage cocktail killing several clinical strains of P. aeruginosa , including those collected from Italian cystic fibrosis (CF) patients, and analyzed the cocktail performance...
March 19, 2018: Antimicrobial Agents and Chemotherapy
Elliott C Dasenbrook, Gregory S Sawicki
Cystic Fibrosis (CF) patient registries are valuable data sources for researchers studying the natural history, treatment paradigms, and long-term health outcomes of individuals with CF. In this review, we discuss the role of CF patient registries in facilitating comparative effectiveness research, particularly evaluating therapies and variation in health care delivery. We also discuss the limitations of registry-based research, particularly indication bias, as well as statistical methods that can be used to address these issues...
March 16, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Vander Borght Mélodie, Wyns Christine
Infertility is a disease characterized by the failure to establish a clinical pregnancy after 12 months of regular and unprotected sexual intercourse. It is estimated to affect between 8 and 12% of reproductive-aged couples worldwide. Males are found to be solely responsible for 20-30% of infertility cases but contribute to 50% of cases overall. Secondary infertility is the most common form of female infertility around the globe, often due to reproductive tract infections. The three major factors influencing the spontaneous probability of conception are the time of unwanted non-conception, the age of the female partner and the disease-related infertility...
March 16, 2018: Clinical Biochemistry
Graciela Rodríguez-Sevilla, Marta García-Coca, David Romera-García, John Jairo Aguilera-Correa, Ignacio Mahíllo-Fernández, Jaime Esteban, Concepción Pérez-Jorge
Lung disease in cystic fibrosis (CF) is characterized by the progressive colonization of the respiratory tract by different bacteria, which develop polymicrobial biofilms. In the past decades, there has been an increase in the number of CF patients infected with Non-Tuberculous Mycobacteria (NTM). Although Mycobacterium abscessus is the main NTM isolated globally, little is known about M. abscessus multispecies biofilm formation. In the present study we developed an in vitro model to study the phenotypic characteristics of biofilms formed by M...
March 6, 2018: International Journal of Medical Microbiology: IJMM
Aarne Fleischer, Iván M Lorenzo, Esther Palomino, Trond Aasen, Fernando Gómez, Miguel Servera, Víctor J Asensio, Víctor Gálvez, Juan Carlos Izpisúa-Belmonte, Daniel Bachiller
Cystic Fibrosis (CF) is a monogenic, lethal disease caused by mutations in the cystic fibrosis transmembrane conductance (CFTR) gene. Here we report the production of CF-iPS cell lines from two different p.F508del homozygous female patients (Table 1). Two different primary cell types, skin fibroblasts and keratinocytes, were transfected with retroviral cocktails containing four: c-MYC, KLF4, OCT4 and SOX2 (MKOS) or three: KLF4, OCT4 and SOX2 (KOS) reprogramming factors. Two fibroblast-derived MKOS lines are described in the main text...
March 11, 2018: Stem Cell Research
M Boyle, J E Moore, J L Whitehouse, D Bilton, D G Downey
Aspergillus fumigatus is commonly found in the airways of patients with cystic fibrosis (CF), and allergic bronchopulmonary aspergillosis (ABPA) is the most recognized associated clinical condition. However, accurate diagnosis remains challenging, and there is a paucity of clinical trials to guide clinical management of fungal disease. The aim of this survey was to assess the variability in current practice across the UK in diagnosis and management of fungal lung disease in CF patients. A 21 question anonymous online survey was sent to 94 paediatric and adult CF consultants in the UK...
March 15, 2018: Medical Mycology: Official Publication of the International Society for Human and Animal Mycology
David M Orenstein, Robert N Abood
PURPOSE OF REVIEW: Cystic fibrosis (CF) has received a lot of attention in the past few years because of increased longevity and the emergence of ground-breaking new drugs targeting the molecular and cellular defects, making a huge clinical difference, and - not incidentally - carrying massive price tags. The prices of these new drugs make the question of overall costs of CF care highly relevant. RECENT FINDINGS: This article reviews recent developments in CF science and treatment, and highlights areas that contribute to costs of CF care, emphasizing how these costs have increased...
March 16, 2018: Current Opinion in Pediatrics
Hinnerk Schulz-Hildebrandt, Mario Pieper, Charlotte Stehmar, Martin Ahrens, Christian Idel, Barbara Wollenberg, Peter König, Gereon Hüttmann
Intravital microscopy (IVM) offers the opportunity to visualize static and dynamic changes of tissue on a cellular level. It is a valuable tool in research and may considerably improve clinical diagnosis. In contrast to confocal and non-linear microscopy, optical coherence tomography (OCT) with microscopic resolution (mOCT) provides intrinsically cross-sectional imaging. Changing focus position is not needed, which simplifies especially endoscopic imaging. For in-vivo imaging, here we are presenting endo-microscopic OCT (emOCT)...
February 1, 2018: Biomedical Optics Express
David Z Silberstein, Kalimuthu Karuppanan, Hnin Hnin Aung, Ching-Hsien Chen, Carroll E Cross, Karen A McDonald
Proteases and reactive oxygen species (ROS) have long been implicated in playing key roles in host tissue injury at sites of inflammatory processes dominated by macrophage activations and/or neutrophil infiltrations. Imbalances between proteases/antiproteases and ROS/antioxidants are recognized to contribute to amplification of inflammatory-based host tissue injury. This has been especially well-documented in such respiratory tract diseases as chronic obstructive pulmonary disease, cystic fibrosis, and acute respiratory distress syndrome...
March 15, 2018: Free Radical Biology & Medicine
Roberto Buzzetti, Valeria Galici, Natalia Cirilli, Fabio Majo, Luigi Graziano, Stefano Costa, Simona Bonacina, Marila Carrubba, Giuseppe Davì, Sabrina Gagliano, Clizia Cazzarolli, Francesca Ficili, Federico Alghisi, Michele Samaja, Giuseppe Magazzù
The aim of this report is to assess whether the research issues priorities are perceived differently according to the Stakeholders (SH)'s individual knowledge of research topics and degree of training in biomedical research. Four groups of SH were enrolled in this study: 1. Skilled SH, specifically trained in biomedicine; 2. Unskilled untrained SH who responded to a written questionnaire in 2015; 3. SH who were trained for one year in a course delivered by professionals; 4. Untrained SH who responded to an online questionnaire in 2017...
March 15, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Kana R Jat, Dinesh K Walia, Anju Khairwa
BACKGROUND: Cystic fibrosis is an autosomal recessive multisystem disorder with an approximate prevalence of 1 in 3500 live births. Allergic bronchopulmonary aspergillosis is a lung disease caused by aspergillus-induced hypersensitivity with a prevalence of 2% to 15% in people with cystic fibrosis. The mainstay of treatment includes corticosteroids and itraconazole. The treatment with corticosteroids for prolonged periods of time, or repeatedly for exacerbations of allergic bronchopulmonary aspergillosis, may lead to many adverse effects...
March 18, 2018: Cochrane Database of Systematic Reviews
Giuseppe Brescia, Claudia Zanotti, Daniela Parrino, Umberto Barion, Gino Marioni
PURPOSE: Endotyping chronic rhinosinusitis with nasal polyps (CRSwNP) poses a challenge for rhinologists nowadays. Phenotyping CRSwNP proved inappropriate as an approach to their classification because of their common clinical features. Endotyping, being based on the pathogenic mechanism, provides a precise picture more appropriate for use in clinical practice. Patients' treatment and follow-up can thus be tailored to cope with the degree of aggressiveness of a specific CRSwNP endotype...
March 7, 2018: American Journal of Otolaryngology
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