Related Experiment Video
Updated: May 29, 2026

08:00
Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Cystic fibrosis lung transplantation
Andrew T Braun1, Christian A Merlo
1Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, School of Medicine, Johns Hopkins Hospital, Baltimore, Maryland 21205, USA. abraun1@jhmi.edu
Current Opinion in Pulmonary Medicine
|September 8, 2011
Summary
Lung transplantation offers a life-saving option for cystic fibrosis (CF) patients. Careful candidate selection and managing CF-specific complications are key to improving long-term outcomes after lung transplant.
Area of Science:
- Pulmonology
- Transplant Surgery
- Genetics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
- Lung transplantation is a critical intervention for end-stage CF lung disease.
- Recent advancements necessitate updated reviews on CF lung transplantation protocols.
Purpose of the Study:
- To review recent literature on indications and candidate selection for CF lung transplantation.
- To discuss infectious and noninfectious complications specific to CF lung transplant recipients.
- To highlight strategies for optimizing outcomes in this patient population.
Main Methods:
- Comprehensive literature search of recent studies on cystic fibrosis lung transplantation.
- Focus on high-risk patient populations, including those on mechanical ventilation and with pulmonary hypertension.
- Analysis of peri-operative infectious challenges and management of common comorbidities.
Main Results:
- Recent research emphasizes evaluating high-risk CF patients for lung transplantation.
- Management of multidrug-resistant infections (bacterial, mycobacterial, fungal) is a significant peri-operative concern.
- Addressing comorbidities like CF-related diabetes, GERD, liver disease, and bone metabolism is crucial.
Conclusions:
- Lung transplantation is a viable, potentially life-saving therapy for select CF patients.
- Optimizing pre-transplant candidate selection is vital for successful outcomes.
- Awareness and management of CF-specific issues pre- and post-transplant improve long-term results.
Related Concept Videos
Cystic Fibrosis: Management
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Sinus disease and chronic sinusitis...
Cystic Fibrosis: Pathogenesis
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
