Association between Polyunsaturated Fatty Acid Profile and Bronchial Inflammation in Bronchiolitis Obliterans by Silvija P. Jerkic, Laura Bächle, Ruth Pia Duecker, Lucia Gronau, Andreas G. Chiocchetti, Stefan Zielen, Ralf Schubert

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Pyoverdine, the Major Siderophore in Pseudomonas aeruginosa, Evades NGAL Recognition by Mary E. Peek, Abhinav Bhatnagar, Nael A. McCarty, Susu M. Zughaier

“…Pseudomonas aeruginosa is the most common pathogen that persists in the cystic fibrosis lungs. Bacteria such as P. aeruginosa secrete siderophores (iron-chelating molecules) and the host limits bacterial growth by producing neutrophil-gelatinase-associated lipocalin (NGAL) that specifically scavenges bacterial siderophores, therefore preventing bacteria from establishing infection. …”
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The Contribution of the Airway Epithelial Cell to Host Defense by Frauke Stanke

“…In the context of cystic fibrosis, the epithelial cell has been characterized in terms of its ion transport capabilities. …”
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Inflammation and ER Stress Downregulate BDH2 Expression and Dysregulate Intracellular Iron in Macrophages by Susu M. Zughaier, Brandon B. Stauffer, Nael A. McCarty

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Impact of elexacaftor/tezacaftor/ivacaftor (ETI) on the prescription refill rate (PRR) for inhaled medications in people with CF: an Italian multicenter analysis by Andrea Gramegna, Giuseppe Cimino, Marco Cipolli, Giovanna Gandini, Maria Adelaide Calderazzo, Angelica Profiti, Federica Piedepalumbo, Francesco Blasi

“…Abstract This retrospective population-based study investigated the impact of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on inhaled medication adherence in people with cystic fibrosis (pwCF). Prescription refill rate (PRR) for several inhaled medications were compared before and after ETI introduction in three major Italian CF centers. …”
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Motion-Genetic Testing is Useful in the Diagnosis of Nonhereditary Pancreatic Conditions: Arguments for the Motion by David C Whitcomb

“…Mutations of the CFTR gene can lead either to cystic fibrosis or to idiopathic chronic pancreatitis, and to a variety of cystic fibrosis-associated disorders, including congenital bilateral absence of the vas deferens and sinusitis. …”
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Case Study: Analyzing CFTR Mutations and SNPs in Pulmonary Fibrosis Patients with Unclear Symptoms by Sahar Yousaf, null Sumaira, Iqbal Bano, Atia Rehman, Samra Kousar, Muhammad Usman Ghani, Mariam Shahid

“…Cystic fibrosis (CF) is a genetic monogenic disorder inherited in an autosomal recessive manner, marked by persistent airway infections in the endobronchial region. …”
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Motion-Genetic Testing is Useful in the Diagnosis of Nonhereditary Pancreatic Conditions: Arguments Against the Motion by Jonathan A Cohn

“…Mutations of two genes, the cystic fibrosis transmembrane conductance regulator gene (CFTR) and the pancreatic secretory trypsin inhibitor gene (PSTI), are associated with an increase in the risk of idiopathic chronic pancreatitis. …”
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Energy Expenditure in Infants in Health and Disease by Ross Shepherd

“…In cystic fibrosis, both TEE and REE are 25% higher than values in healthy infants. …”
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Clinical Evaluation of Specific Oral Manifestations in Pediatric Patients with Ascertained versus Potential Coeliac Disease: A Cross-Sectional Study by Ennio Bramanti, Marco Cicciù, Giada Matacena, Stefano Costa, Giuseppe Magazzù

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Heme alters biofilm formation in Mycobacterium abscessus by Hadia Aftab, Jessica Samudio, Grace Wang, Lily Le, Rajesh K. Soni, Rebecca K. Donegan

“…ABSTRACT Mycobacterium abscessus (Mabs) is commonly found in the cystic fibrosis (CF) lung. During infection, Mabs can form biofilms in the lung which reduce both the ability of the immune response to clear infection and the effectiveness of antibiotic therapy. …”
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Prevalence and features of allergic bronchopulmonary aspergillosis, United States, 2016-2022. by Kaitlin Benedict, Jeremy A W Gold, Mitsuru Toda, Joy Hsu

“…To estimate national ABPA prevalence among patients with asthma or cystic fibrosis, characterize ABPA testing practices, and describe ABPA clinical features, treatment, and 6-month outcomes. …”
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Immunopathology of Airway Surface Liquid Dehydration Disease by Brandon W. Lewis, Sonika Patial, Yogesh Saini

“…Defective MCC is often associated with airway mucoobstruction, increased occurrence of respiratory infections, and progressive decrease in lung function in mucoobstructive lung diseases including cystic fibrosis (CF). In this disease, a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene results in dehydration of the airway surface liquid (ASL) layer. …”
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Heterogeneity of Clostridioides difficile asymptomatic colonization prevalence: a systematic review and meta-analysis by Daniel De-la-Rosa-Martínez, Rodrigo Villaseñor-Echavarri, Diana Vilar-Compte, Virna Mosqueda-Larrauri, Paola Zinser-Peniche, Seth Blumberg

“…Our meta-regression analysis revealed lower rates in healthy individuals and higher rates in cystic fibrosis patients and studies from North America. …”
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Chronic Disease Management in Children Based on the Five Domains of Health by Wing Lung Alvin So

“…Through a case study of a child with cystic fibrosis, the interactions among various domains of health have been discussed—namely, biomedical, physical, psychological/behavioural, and social. …”
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Inherited Causes of Exocrine Pancreatic Dysfunction by Peter R Durie

“…Most inherited causes of pancreatic dysfunction are due to a generalized disorder. Cystic fibrosis is, by far, the most common inherited cause of disturbed pancreatic function among Caucasian children. …”
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Influence of a macrolide antibiotic, roxithromycin, on mast cell growth and activation in vitro by Toshikazu Shimane, Kazuhito Asano, Mayumi Suzuki, Tadashi Hisamitsu, Harumi Suzaki

“…Background: Long-term administration of macrolide antibiotics is recognized to be able to favorably modify the clinical condition of inflammatory diseases, such as diffuse panbronchiolitis and cystic fibrosis. However, the precise mechanisms by which macrolide antibiotics could improve clinical conditions of the patients are not well understood.…”
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Newborn Screening Saves Lives but Cannot Replace the Need for Clinical Vigilance by F. Neemuchwala, M. Taki, E. Secord, S. Z. Nasr

“…Newborn screening for cystic fibrosis (CF) enables early diagnosis and treatment leading to improved health outcomes for patients with CF. …”
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Glutamine Supplementation in Sick Children: Is It Beneficial? by Elise Mok, Régis Hankard

“…This includes premature infants, infants with gastrointestinal disease, children with Crohn's disease, short bowel syndrome, malnutrition/diarrhea, cancer, severe burns/trauma, Duchenne muscular dystrophy, sickle cell anemia, cystic fibrosis, and type 1 diabetes. Moreover, methodological issues have been noted in some studies. …”
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Autophagy in Inflammatory Diseases by Alexander J. S. Choi, Stefan W. Ryter

“…Furthermore, autophagy-dependent mechanisms have been proposed in the pathogenesis of several pulmonary diseases that involve inflammation, including cystic fibrosis and pulmonary hypertension. Strategies aimed at modulating autophagy may lead to therapeutic interventions for diseases associated with inflammation.…”
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