scholarly journals Rapidly progressive lung disease in a patient with cystic fibrosis on long-term azithromycin: possible role of mycoplasma infection

2005 ◽  
Vol 4 (1) ◽  
pp. 71-73 ◽  
Author(s):  
I.A. Janahi ◽  
A. Abdulwahab ◽  
S. Elshafie Sittana ◽  
A. Bush
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Mycoplasma Infection

scholarly journals 2045 The role of TGFβ in driving early cystic fibrosis lung disease

2018 ◽  
Vol 2 (S1) ◽  
pp. 33-33
Author(s):  
Elizabeth L. Kramer ◽  
William Hardie ◽  
Kristin Hudock ◽  
Cynthia Davidson ◽  
Alicia Ostmann ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Bronchoalveolar Lavage ◽  
Lung Disease ◽  
Bronchoalveolar Lavage Fluid ◽  
Transforming Growth Factor ◽  
Genetic Modifier ◽  
Lavage Fluid ◽  
Lung Mechanics ◽  
Patient Specific

OBJECTIVES/SPECIFIC AIMS: Transforming growth factor-beta (TGFβ) is a genetic modifier of cystic fibrosis (CF) lung disease. TGFβ’s pulmonary levels in young CF patients and its mechanism of action in CF are unknown. We examined TGFβ levels in children with CF and investigated responses of human airway epithelial cells (AECs) and mice to TGFβ. METHODS/STUDY POPULATION: TGFβ levels in bronchoalveolar lavage fluid from CF patients (n=15) and non-CF control patients (n=21)<6 years old were determined by ELISA. CF mice and non-CF mice were intratracheally treated with an adenoviral TGFβ1 vector or PBS; lungs were collected for analysis at day 7. Human CF and non-CF AECs were treated with TGFβ or PBS for 24 hours then collected for analysis. RESULTS/ANTICIPATED RESULTS: Young CF patients had higher bronchoalveolar lavage fluid TGFβ than non-CF controls (p=0.03). Mouse lungs exposed to TGFβ demonstrated inflammation, goblet cell hyperplasia, and decreased CFTR expression. CF mice had greater TGFβ-induced lung mechanics abnormalities than controls; both CF human AECs and CF mice showed higher TGFβ induced MAPK and PI3K signaling compared with controls. DISCUSSION/SIGNIFICANCE OF IMPACT: For the first time, we show increased TGFβ levels very early in CF. TGFβ drives CF lung abnormalities in mouse and human models; CF models are more sensitive to TGFβ’s effects. Understanding the role of TGFβ in promoting CF lung disease is critical to developing patient specific treatments.

scholarly journals Posaconazole therapy in children with cystic fibrosis and Aspergillus-related lung disease

2019 ◽  
Vol 58 (1) ◽  
pp. 11-21 ◽  
Author(s):  
Deepa Patel ◽  
Sarah Popple ◽  
Alison Claydon ◽  
Deborah E Modha ◽  
Erol A Gaillard
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Plasma Levels ◽  
Allergic Bronchopulmonary Aspergillosis ◽  
Tablet Formulation ◽  
Full Blood Count ◽  
Systemic Corticosteroids ◽  
Liquid Preparation ◽  
A Prospective Study

Abstract There is emerging evidence for the role of posaconazole in the management of Aspergillus-related cystic fibrosis (CF) lung disease. The tolerability and efficacy of posaconazole in paediatric CF is not well established. We report a prospective study over a fifty-three month period evaluating the safety, tolerability, and efficacy of posaconazole in pediatric CF. Fourteen children (seven males, median age 13 years, range 3–17 years) received a total of twenty-three courses of posaconazole (13 oral suspension and 10 tablet formulation). Of these patient episodes, nine received posaconazole for emerging or active allergic bronchopulmonary aspergillosis (ABPA) and two required a combination of posaconazole and systemic corticosteroids for difficult-to-treat ABPA. A subgroup of patients (n = 12) with persistent isolates of Aspergillus fumigatus, in the absence of serological markers of ABPA, received posaconazole monotherapy for pulmonary exacerbations not responding to conventional broad-spectrum antibiotic treatment. Posaconazole levels, full blood count, electrolytes, and liver function were monitored on day 7 of treatment and then monthly. Posaconazole was well tolerated in all but three patients. Therapeutic plasma levels &gt;1 mg/l were achieved in all receiving the tablet formulation in comparison to 60% on the liquid preparation. There was a modest but significant improvement in FEV1 (% predicted) demonstrated for the cohort as a whole (p = 0.015) following posaconazole therapy. Posaconazole is well tolerated in children as young as six years old, improvements in lung function are observed, and therapeutic plasma levels are readily achieved in patients taking the tablet formulation and in adherent patients taking the liquid formulation.

Neutrophils in cystic fibrosis

2016 ◽  
Vol 397 (6) ◽  
pp. 485-496 ◽  
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.

Acute and Long-term Amiloride Inhalation in Cystic Fibrosis Lung Disease: A Rational Approach to Cystic Fibrosis Therapy

1990 ◽  
Vol 141 (3) ◽  
pp. 605-612 ◽  
Author(s):  
Ernst M. App ◽  
Malcolm King ◽  
Renate Helfesrieder ◽  
Dieter Köhler ◽  
Heinrich Matthys
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
Rational Approach ◽  
Cystic Fibrosis Lung Disease

scholarly journals The CFTR and ENaC debate: how important is ENaC in CF lung disease?

2012 ◽  
Vol 302 (11) ◽  
pp. L1141-L1146 ◽  
Author(s):  
James F. Collawn ◽  
Ahmed Lazrak ◽  
Zsuzsa Bebok ◽  
Sadis Matalon
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Bacterial Infections ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Ongoing Debate ◽  
The Subject ◽  
Cystic Fibrosis Transmembrane ◽  
Epithelial Sodium Channel Enac

Cystic fibrosis (CF) is caused by the loss of the cystic fibrosis transmembrane conductance regulator (CFTR) function and results in a respiratory phenotype that is characterized by dehydrated mucus and bacterial infections that affect CF patients throughout their lives. Much of the morbidity and mortality in CF results from a failure to clear bacteria from the lungs. What causes the defect in the bacterial clearance in the CF lung has been the subject of an ongoing debate. Here we discuss the arguments for and against the role of the epithelial sodium channel, ENaC, in the development of CF lung disease.

scholarly journals The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Matthew S. Twigg ◽  
Simon Brockbank ◽  
Philip Lowry ◽  
S. Peter FitzGerald ◽  
Clifford Taggart ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Immune Responses ◽  
Lung Disease ◽  
Healthy Individual ◽  
Chronic Infection ◽  
Serine Proteases ◽  
High Concentration ◽  
Mucus Clearance ◽  
Potential Therapeutics

Cystic fibrosis (CF) lung disease is an inherited condition with an incidence rate of approximately 1 in 2500 new born babies. CF is characterized as chronic infection of the lung which leads to inflammation of the airway. Sputum from CF patients contains elevated levels of neutrophils and subsequently elevated levels of neutrophil serine proteases. In a healthy individual these proteases aid in the phagocytic process by degrading microbial peptides and are kept in homeostatic balance by cognate antiproteases. Due to the heavy neutrophil burden associated with CF the high concentration of neutrophil derived proteases overwhelms cognate antiproteases. The general effects of this protease/antiprotease imbalance are impaired mucus clearance, increased and self-perpetuating inflammation, and impaired immune responses and tissue. To restore this balance antiproteases have been suggested as potential therapeutics or therapeutic targets. As such a number of both endogenous and synthetic antiproteases have been trialed with mixed success as therapeutics for CF lung disease.

scholarly journals CYSTIC FIBROSIS: A BREAKTHROUGH IN 21ST-CENTURY THERAPY

2020 ◽  
Vol 23 (1) ◽  
pp. 35-41
Author(s):  
Olga I. Simonova ◽  
Yulia V. Gorinova ◽  
Vera P. Chernevich
Keyword(s):  
Cystic Fibrosis ◽  
Web Of Science ◽  
New Drugs ◽  
Cystic Fibrosis Gene ◽  
Multicenter Studies ◽  
Antibacterial Compounds ◽  
The Russian Federation ◽  
Mucolytic Agents

The review presents new data on the latest advances in the treatment of cystic fibrosis, a rare genetic disease. The methods used were literature search in the Scopus, Web of Science, and EMBASE databases. The importance of a number of drugs prescribed for anti-inflammatory purposes (ibuprofen, azithromycin) is discussed, data from multicenter studies of new drugs are presented. The role of mucolytic agents and the need to develop new antibacterial compounds are shown. Particular attention in the review is given to the development of new targeted therapies for cystic fibrosis. The data of studies of ivacaftor, lumacactor, tezacactor in this category of patients are presented, as well as a spectrum of mutations of the cystic fibrosis gene in which these molecules can be prescribed. The experience of the use of correctors in adult patients with cystic fibrosis in the Russian Federation is described, the effectiveness and safety of the long-term use of these drugs are described.

scholarly journals Effect of Early Initiation of Combination Therapy of Anti-Inflammatory and Anti-Fibrotic Drugs on Post COVID-19 Interstitial Lung Disease: A Case Report

2021 ◽  
Vol 7 (3) ◽  
Keyword(s):  
Case Report ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Oral Prednisone ◽  
Evidence Based ◽  
Limited Evidence ◽  
Anti Inflammatory ◽  
Short Time

The persistence of lung parenchymal changes Post COVID-19 is increasingly recognized as a vital outcome observed on some patients recovering from SARS-CoV2 infection with limited evidence-based management so far. Here we present a 64-year-old male developed extensive interstitial lung changes post SARS-CoV2 infection and was successfully managed with oral prednisone, pirfenidone, and azithromycin maintenance regimen with significant improvement in his clinical and radiographic parameters noted within a relatively short time. The role of a combined therapeutic approach with anti-inflammatory and anti-fibrotic drugs might be provocative action in patients with COVID-19 related interstitial lung disease, especially those at risk for persistent long-term abnormalities and pulmonary fibrosis development.

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