Cystic fibrosis: a clinical view

C Castellani, BM Assael - Cellular and molecular life sciences, 2017 - Springer
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 …

Background and epidemiology

DB Sanders, AK Fink - Pediatric Clinics, 2016 - pediatric.theclinics.com
Cystic fibrosis (CF) is the most common autosomal-recessive cause of early mortality in
caucasians worldwide. 1 However, significant advances in therapies and outcomes for …

Neutrophil dysfunction in the pathogenesis of cystic fibrosis

G Wang, WM Nauseef - Blood, The Journal of the American …, 2022 - ashpublications.org
Polymorphonuclear neutrophils (PMNs) figure prominently in host defense against infection
and in noninfectious inflammation. Mobilized early in an inflammatory response, PMNs …

[HTML][HTML] Molecular basis of cystic fibrosis: from bench to bedside

MC Dechecchi, A Tamanini… - Annals of translational …, 2018 - ncbi.nlm.nih.gov
Cystic fibrosis (CF), is an autosomal recessive disease affecting different organs. The lung
disease, characterized by recurrent and chronic bacterial infection and inflammation since …

[HTML][HTML] Cystic fibrosis-related diabetes: Prevalence, screening, and diagnosis

S Khare, M Desimone, N Kasim, CL Chan - Journal of Clinical & …, 2022 - Elsevier
Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in patients with
cystic fibrosis (CF). Prevalence of CFRD increases with age and is greater with severe …

Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications

C Edmondson, JC Davies - Therapeutic advances in chronic …, 2016 - journals.sagepub.com
Treatment for cystic fibrosis (CF) has conventionally targeted downstream consequences of
the defect such as mucus plugging and infection. More recently, significant advances have …

Azole Resistance in Aspergillus fumigatus in Patients with Cystic Fibrosis: A Matter of Concern?

A Hamprecht, F Morio, O Bader, P Le Pape… - Mycopathologia, 2018 - Springer
Aspergillus fumigatus is the most frequent filamentous fungus isolated from respiratory
specimens from patients with cystic fibrosis (CF). Triazoles are the most widely used …

Emergence of antimicrobial resistance among Pseudomonas aeruginosa: implications for therapy

JP Lynch III, GG Zhanel… - Seminars in respiratory and …, 2017 - thieme-connect.com
Pseudomonas aeruginosa (PA), a nonlactose fermenting gram-negative bacillus, is a
common cause of nosocomial infections in critically ill or debilitated patients, particularly …

[HTML][HTML] Outcomes following lung re-transplantation in patients with cystic fibrosis

EG Chan, EJ Hyzny, JP Ryan, MR Morrell… - Journal of Cystic …, 2022 - Elsevier
Purpose We examined cystic fibrosis (CF) patients and compared their clinical status at the
time of primary versus double lung re-transplantation (re-DLTx) in order to better understand …

Screening strategies for glucose tolerance abnormalities and diabetes in people with cystic fibrosis

L Weiss, P Reix, H Mosnier-Pudar, O Ronsin… - Diabetes & …, 2023 - Elsevier
The increase in life expectancy of patients with cystic fibrosis has come with new
comorbidities, particularly diabetes. The gradual development of glucose tolerance …