Outcomes of combined liver-lung transplant in pediatric patients with cystic fibrosis: An ISHLT transplant registry study by Renita Wilson, BS, J. Asher Jenkins, MD, Juan Maria Farina, MD, Blake Langlais, MS, Bashar Aqel, MD, Ashraf Omar, MD, Jonathan D’Cunha, MD PhD, Pedro Reck dos Santos, MD PhD

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Adrenal Crisis Caused by Inhaled Fluticasone in an Adolescent with Cystic Fibrosis and Advanced Hepatopathy: A Case Report by C. Denne, A. E. Vogl-Voswinckel, A. Gruebl, S. Burdach

“…Inhaled corticosteroids are widely accepted in the treatment of cystic fibrosis. Long-term use may cause systemic complications, especially high-dose fluticasone. …”
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Effects of High Frequency Chest Compression on Respiratory System Mechanics in Normal Subjects and Cystic Fibrosis Patients by Richard L Jones, Richard T Lester, Neil E Brown

“…OBJECTIVE: To investigate the short term effects of high frequency chest compression (HFCC) on several indices of respiratory system mechanics in normal subjects and patients with cystic fibrosis (CF).…”
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Infections, autoimmunity and immunodeficiencies are the leading etiologies of non-cystic fibrosis bronchiectasis in adults from the southwest of Colombia. by Andrés F. Zea-Vera, Carlos Andrés Rodríguez, Sebastián Giraldo, Mario Alejandro Chacón, Luis Fernando Guerrero, Ricardo Mosquera, Raúl Andrés Vallejo, Fabio Samir Vargas, María Andrea García, María A. Rengifo, Anilza Bonelo, Maximiliano Parra

“…There are no data about the prevalence of non-cystic fibrosis bronchiectasis etiologies in the Colombian population. …”
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Pulmonary Bacterial Communities in Surgically Resected Noncystic Fibrosis Bronchiectasis Lungs Are Similar to Those in Cystic Fibrosis by Heather Maughan, Kristopher S. Cunningham, Pauline W. Wang, Yu Zhang, Marcelo Cypel, Cecilia Chaparro, D. Elizabeth Tullis, Thomas K. Waddell, Shaf Keshavjee, Mingyao Liu, David S. Guttman, David M. Hwang

“…We characterized and compared the pulmonary bacterial communities of cystic fibrosis (CF) and non-CF bronchiectasis patients using a culture-independent molecular approach. …”
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Diagnostic yield of cystic fibrosis from a South Australian monocentric cohort: a retrospective study by Maria Fuller, Jasmina Markulić

“…Objectives To determine the diagnostic yield of cystic fibrosis (CF) using a two-tiered genetic testing approach. …”
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Lived experiences for individuals with cystic fibrosis who have undergone lung transplantation: a qualitative study by Ulrika Skogeland, Isabelle de Monestrol, Terezia Pincikova, Tove Godskesen

Subjects: “…Cystic fibrosis…”
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The Novel CFTR Mutation A457P in a Male with a Delayed Diagnosis of Cystic Fibrosis by Kate H. Cole, Patrick R. Sosnay, Lonny B. Yarmus, Jonathan B. Zuckerman

“…Cystic fibrosis (CF) is an autosomal recessive disease that may be caused by more than 1000 different mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. …”
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Myeloperoxidase Promoter Polymorphism −463G Is Associated With More Severe Clinical Expression of Cystic Fibrosis Pulmonary Disease by Wanda F. Reynolds, Isabelle Sermet-Gaudelus, Valérie Gausson, Marie-Noëlle Feuillet, Jean-Paul Bonnefont, Gérard Lenoir, Béatrice Descamps-Latscha, Véronique Witko-Sarsat

“…The severity of cystic fibrosis (CF) pulmonary disease is not directly related to CFTR genotype but depends upon several parameters, including neutrophil-dominated inflammation. …”
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Non-classical pulmonary exacerbation in cystic fibrosis revealing ALK-Translocated lung cancer: A case report by Mohamad Hadhud, Johnathan Arnon, Anat Hershko-Moshe, Adi Hollander, Noa Hurvitz-Lehmann, Assaf Potruch, Henny Azmanov, Rottem Kuint, Nurith Hiller, Elie Picard, Naama Sebbag-Sznajder, Shira Leebhoff, Michael Wilschanski, Myriam Grunewald, Liron Birimberg-Schwartz, Malena Cohen-Cymberknoh

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Evaluation of Elexacafor/Tezacaftor/Ivacaftor therapy after lung transplantation in Cystic Fibrosis: The Dutch National KOALA study by Johanna P. van Gemert, Bart Luijk, Merel E. Hellemons, Klara A. Visser, Carina.M.E. Hansen, Renske van der Meer, C. Tji Gan, Hester van der Vaart, Onno W. Akkerman, Willie N. Steenhuis, Marieke Verkleij, Harry G.M. Heijerman, Erik A.M. Verschuuren

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Elexacaftor/tezacaftor/ivacaftor and inflammation in children and adolescents with cystic fibrosis: a retrospective dual-center cohort study by Angela Pepe, Cristina Fevola, Daniela Dolce, Silvia Campana, Novella Ravenni, Giovanni Taccetti, Donatello Salvatore, Vito Terlizzi

“…Background: Cystic fibrosis (CF) is characterized by chronic neutrophilic inflammation in the airways. …”
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Pulmonary Delivery of Antibiotics to the Lungs: Current State and Future Prospects by Yahya H. Dallal Bashi, Rachel Mairs, Rand Murtadha, Vicky Kett

Subjects: “…cystic fibrosis…”
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The complexities of elexacaftor/tezacaftor/ivacaftor therapeutic drug monitoring in a person with cystic fibrosis and Mycobacterium abscessus pulmonary disease by Claire Y Mou, Daniel J Henderson, Angela G Matson, Karen M Herd, David W Reid, Timothy Riddles, Ellie Johnson, Brett McWhinney, Rebecca Swenson, Andrew Burke, Ieuan E S Evans

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Evidence for the Involvement of Lipid Rafts and Plasma Membrane Sphingolipid Hydrolases in Pseudomonas aeruginosa Infection of Cystic Fibrosis Bronchial Epithelial Cells by Domitilla Schiumarini, Nicoletta Loberto, Giulia Mancini, Rosaria Bassi, Paola Giussani, Elena Chiricozzi, Maura Samarani, Silvia Munari, Anna Tamanini, Giulio Cabrini, Giuseppe Lippi, Maria Cristina Dechecchi, Sandro Sonnino, Massimo Aureli

“…Cystic fibrosis (CF) is the most common autosomal genetic recessive disease caused by mutations of gene encoding for the cystic fibrosis transmembrane conductance regulator. …”
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Targeted Antibiotic Prophylaxis for Lung Transplantation in Cystic Fibrosis Patients Colonised with Pseudomonas aeruginosa Using Multiple Combination Bactericidal Testing by Helmy Haja Mydin, Paul A. Corris, Audrey Nicholson, John D. Perry, Gerard Meachery, Emma C. L. Marrs, Steven Peart, Christine Fagan, James L. Lordan, Andrew J. Fisher, Frances K. Gould

“…Early infection is a recognised complication after lung transplantation in patients with cystic fibrosis (CF). Our centre uses multiple combination bactericidal testing (MCBT) when determining appropriate peritransplant prophylactic regimens. …”
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A Pilot Study to Compare Tobramycin 80 mg Injectable Preparation wth 300 mg Solution for Inhalation in Cystic Fibrosis Patients by Wilfried H Nikolaizik, Delia Vietzke, Felix Ratjen

“…BACKGROUND: Inhaled tobramycin has been shown to improve lung function in cystic fibrosis (CF) patients chronically infected with Pseudomonas aeruginosa. …”
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Using Community Engagement to Create a Telecoaching Intervention to Improve Self-Management in Adolescents and Young Adults With Cystic Fibrosis: Qualitative Study by Christina L Duncan, Emily F Muther, Jennifer J Lindwall, Kristine Durkin, Elizabeth Ruvalcaba, Eliza Williamson, Corrine Ahrabi-Nejad, Evelyn Bord, Angela Green, Megan L Harrison, Deepika Polineni

“… BackgroundAdolescents and young adults (AYA) with cystic fibrosis (CF) are at risk for deviating from their daily treatment regimen due to significant time burden, complicated daily therapies, and life stressors. …”
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Enfrentamento e Maternidade no contexto da Fibrose Cística: estudos de caso // Coping and Maternity in the context of Cystic Fibrosis: case studies by Juliana Laux Soares Schenkel, Tagma Marina Schneider Donelli, Tonantzin Gonçalves

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Body composition changes and clinical outcomes in pediatric cystic fibrosis during 24 months of lumacaftor ivacaftor therapy based on real-world data by Marcell Imrei, Adrienn F. Kéri, Éva Gács, Ildikó Gönczi, Melinda Meláth, Éva Kosaras, Botond Demeter, Csaba Péterfia, Klára Vass, Gyöngyi Székely, Klementina Ocskay, Andrea Párniczky

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