NOVOSTI V OBRAVNAVI PLJUČNE BOLEZNI PRI OTROCIH IN MLADOSTNIKIH S CISTIČNO FIBROZO

Cystic fibrosis (CF) is a multi-organ disease, affecting mostly lungs and gastrointestinal tract. Data from patient registries show that the survival of patients with CF has progressively improved over the past several decades, as a result of advances in antibiotic treatment, supplementation of pancreatic enzymes, better nutrition and a holistic approach to treatment in CF centres.The purpose of this review is to survey recent developments in the treatment of lung disease in children and adolescents with CF.We describe newborn screening for CF.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue.Lung disease is the most common cause of morbidity and mortality in CF patients. Emerging new therapies are targeted at all points in the pathogenesis of lung disease, from drugs that treat infection and inflammation in the airways to gene transfer studies and to drugs that augment airway surface liquid height. A number of antibacterial agents formulated for inhalation are at various stages of study and there are several anti-inflammatory candidate drugs in clinical trials. The most important development in the recent years is modulation of the abnormal protein that causes CF, the cystic fibrosis transmembrane regulator (CFTR), where drugs are targeted at specific defects in the transcription, processing or functioning.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue. The role of the CF nurse, who has responsibilities in educating and teaching clinical skills to patients and families, is described.

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Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?

CHEST Journal ◽

10.1016/j.chest.2021.08.073 ◽

2021 ◽

Author(s):

Oded Breuer ◽

David Shoseyov ◽

Shifra Koretz ◽

Nadia Alyan ◽

Joel Reiter ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Transplantation ◽

Lung Disease ◽

Ethical Dilemma ◽

End Stage

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The chemokine CCL18 characterises Pseudomonas infections in cystic fibrosis lung disease

European Respiratory Journal ◽

10.1183/09031936.00070014 ◽

2014 ◽

Vol 44 (6) ◽

pp. 1608-1615 ◽

Cited By ~ 9

Author(s):

Andreas Hector ◽

Carolin Kröner ◽

Melanie Carevic ◽

Martina Bakele ◽

Nikolaus Rieber ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Disease ◽

Gene Expression Analysis ◽

Ex Vivo ◽

Healthy Controls ◽

Protein Levels ◽

Cystic Fibrosis Lung Disease ◽

Potential Biomarker ◽

Infection And Inflammation ◽

Airway Cells

Cystic fibrosis (CF) lung disease is characterised by chronic Pseudomonas aeruginosa infection and leukocyte infiltration. Chemokines recruit leukocytes to sites of infection. Gene expression analysis identified the chemokine CCL18 as upregulated in CF leukocytes. We hypothesised that CCL18 characterises infection and inflammation in patients with CF lung disease.Therefore, we quantified CCL18 protein levels in the serum and airway fluids of CF patients and healthy controls, and studied CCL18 protein production by airway cells ex vivo.These studies demonstrated that CCL18 levels were increased in the serum and airway fluids from CF patients compared with healthy controls. Within CF patients, CCL18 levels were increased in P. aeruginosa-infected CF patients. CCL18 levels in the airways, but not in serum, correlated with severity of pulmonary obstruction in CF. Airway cells isolated from P. aeruginosa-infected CF patients produced significantly higher amounts of CCL18 protein compared with airway cells from CF patients without P. aeruginosa infection or healthy controls.Collectively, these studies show that CCL18 levels characterise chronic P. aeruginosa infection and pulmonary obstruction in patients with CF. CCL18 may, thus, serve as a potential biomarker and therapeutic target in CF lung disease.

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The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis

Pediatric Pulmonology ◽

10.1002/ppul.1950200203 ◽

1995 ◽

Vol 20 (2) ◽

pp. 63-70 ◽

Cited By ~ 226

Author(s):

K. Balough ◽

M. McCubbin ◽

M. Weinberger ◽

W. Smits ◽

R. Ahrens ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Disease ◽

Early Stages ◽

Infection And Inflammation ◽

The Relationship

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Neutrophils in cystic fibrosis

Biological Chemistry ◽

10.1515/hsz-2015-0271 ◽

2016 ◽

Vol 397 (6) ◽

pp. 485-496 ◽

Cited By ~ 32

Author(s):

Julie Laval ◽

Anjali Ralhan ◽

Dominik Hartl

Keyword(s):

Cystic Fibrosis ◽

Lung Disease ◽

Structural Damage ◽

Fungal Pathogens ◽

Therapeutic Potential ◽

Inflammatory Cells ◽

Current Evidence ◽

Infection And Inflammation ◽

Shed Light

Abstract Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. Among inflammatory cells, neutrophils represent the major cell population accumulating in the airways of CF patients. While neutrophils provide the first defensive cellular shield against bacterial and fungal pathogens, in chronic disease conditions such as CF these short-lived immune cells release their toxic granule contents that cause tissue remodeling and irreversible structural damage to the host. A variety of human and murine studies have analyzed neutrophils and their products in the context of CF, yet their precise functional role and therapeutic potential remain controversial and incompletely understood. Here, we summarize the current evidence in this field to shed light on the complex and multi-faceted role of neutrophils in CF lung disease.

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ASTHMA IN CYSTIC FIBROSIS

Romanian Journal of Pediatrics ◽

10.37897/rjp.2015.2.2 ◽

2015 ◽

Vol 64 (2) ◽

pp. 118-121

Author(s):

Marcela Daniela Ionescu ◽

◽

Ioana-Alina Anca ◽

Mihaela Balgradean ◽

◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Disease ◽

Airway Obstruction ◽

Autosomal Recessive ◽

Pulmonary Infection ◽

Autosomal Recessive Disease ◽

Personal History ◽

Common Symptom ◽

Infection And Inflammation ◽

History Of

Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians. Lung disease is characterized by impaired mucocilliary clearance with airway obstruction and chronic pulmonary infection and inflammation. Wheeze is a common symptom in CF, but in some cases the wheeze is due to the presence of concomitant asthma. There is no consensus on how to define CF asthma, but the diagnosis is predominantly based on the patient’s strong family and personal history of atopy.

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Coinfection with Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis

European Respiratory Review ◽

10.1183/16000617.0011-2020 ◽

2020 ◽

Vol 29 (158) ◽

pp. 200011

Author(s):

Karen Keown ◽

Alastair Reid ◽

John E. Moore ◽

Clifford C. Taggart ◽

Damian G. Downey

Keyword(s):

Cystic Fibrosis ◽

Pseudomonas Aeruginosa ◽

Aspergillus Fumigatus ◽

Lung Disease ◽

Disease Progression ◽

Expert Knowledge ◽

Fungal Species ◽

Interspecies Interactions ◽

Cross Sectional ◽

Infection And Inflammation

ObjectivesCystic fibrosis (CF) lung disease is characterised by mucus stasis, chronic infection and inflammation, causing progressive structural lung disease and eventual respiratory failure. CF airways are inhabited by an ecologically diverse polymicrobial environment with vast potential for interspecies interactions, which may be a contributing factor to disease progression. Pseudomonas aeruginosa and Aspergillus fumigatus are the most common bacterial and fungal species present in CF airways respectively and coinfection results in a worse disease phenotype.MethodsIn this review we examine existing expert knowledge of chronic co-infection with P. aeruginosa and A. fumigatus in CF patients. We summarise the mechanisms of interaction and evaluate the clinical and inflammatory impacts of this co-infection.ResultsP. aeruginosa inhibits A. fumigatus through multiple mechanisms: phenazine secretion, iron competition, quorum sensing and through diffusible small molecules. A. fumigatus reciprocates inhibition through gliotoxin release and phenotypic adaptations enabling evasion of P. aeruginosa inhibition. Volatile organic compounds secreted by P. aeruginosa stimulate A. fumigatus growth, while A. fumigatus stimulates P. aeruginosa production of cytotoxic elastase.ConclusionA complex bi-directional relationship exists between P. aeruginosa and A. fumigatus, exhibiting both mutually antagonistic and cooperative facets. Cross-sectional data indicate a worsened disease state in coinfected patients; however, robust longitudinal studies are required to derive causality and to determine whether interspecies interaction contributes to disease progression.

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Lung transplantation: overall approach regarding its major aspects

Jornal Brasileiro de Pneumologia ◽

10.1590/s1806-37562015000000100 ◽

2015 ◽

Vol 41 (6) ◽

pp. 547-553 ◽

Cited By ~ 5

Author(s):

Priscila Cilene León Bueno de Camargo ◽

Ricardo Henrique de Oliveira Braga Teixeira ◽

Rafael Medeiros Carraro ◽

Silvia Vidal Campos ◽

José Eduardo Afonso Junior ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Transplantation ◽

Lung Disease ◽

Organ Transplant ◽

Sao Paulo ◽

Waiting List ◽

São Paulo ◽

Lung Transplants ◽

Transplant Candidates ◽

The City

ABSTRACT Lung transplantation is a well-established treatment for patients with advanced lung disease. The evaluation of a candidate for transplantation is a complex task and involves a multidisciplinary team that follows the patient beyond the postoperative period. Currently, the mean time on the waiting list for lung transplantation in the state of São Paulo, Brazil, is approximately 18 months. For Brazil as a whole, data from the Brazilian Organ Transplant Association show that, in 2014, there were 67 lung transplants and 204 patients on the waiting list for lung transplantation. Lung transplantation is most often indicated in cases of COPD, cystic fibrosis, interstitial lung disease, non-cystic fibrosis bronchiectasis, and pulmonary hypertension. This comprehensive review aimed to address the major aspects of lung transplantation: indications, contraindications, evaluation of transplant candidates, evaluation of donor candidates, management of transplant recipients, and major complications. To that end, we based our research on the International Society for Heart and Lung Transplantation guidelines and on the protocols used by our Lung Transplant Group in the city of São Paulo, Brazil.

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