The Power of Two

After nearly 30 years, a long, hard fight against cystic fibrosis and 3 miraculous double lung transplants between them, Ana and Isa Stenzel are still alive at 41. Watch their incredible story in the acclaimed film THE POWER OF TWO premiering May 22 at 8pm ET &PT on Link TV and KCET. Watch free online starting May 22 at
Source: Cystic Fibrosis Transplants Forum - Category: Respiratory Medicine Authors: Tags: Transplants Source Type: forums

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ConclusionsTherapeutic drug monitoring-guided meropenem may be a sound new therapeutic option in eradicating multidrug resistant Acinetobacter and offer a novel therapeutic option in the field of personalized medicine.
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research
Authors: Ruszel N, Kubisa B, Lisowski P, Piotrowska M, Kubisa MJ, Brykczyński M, Wojtyś M, Pieróg J, Czarnecka M, Wójcik J, Wójcik N, Sielicki P, Bielewicz M, Grodzki T PMID: 31708987 [PubMed]
Source: Polish Journal of Cardio-Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Tags: Kardiochir Torakochirurgia Pol Source Type: research
Conclusion. Further investigation to confirm the hypothesis that C9-PQS may be associated with increased antibiotic usage for pulmonary exacerbations is warranted as AQ-dependent signalling is a potential future target for anti-virulence therapies. PMID: 31671047 [PubMed - as supplied by publisher]
Source: Journal of Medical Microbiology - Category: Microbiology Authors: Tags: J Med Microbiol Source Type: research
Cystic fibrosis (CF) is the most common genetically inherited disease in the Caucasian population [1]. Respiratory manifestations of CF include reduction of mucus clearance, chronic pulmonary infections, and bronchiectasis, causing a progressive respiratory failure that is the primary cause of death in CF patients. Advanced CF might be complicated by pulmonary hypertension, right ventricular hypertrophy, and right heart failure [2]. Bilateral lung transplantation (LUTX) is a viable option for CF, providing a significant survival benefit [3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
Pseudomonas aeruginosa (P. aeruginosa) is a common pathogen in the setting of lung transplantation.1 Most recipients become chronically colonized with P. aeruginosa. Previously we have demonstrated in a subgroup of patients with non-cystic fibrosis (CF) bronchiectasis who are chronically colonized with P. aeruginosa, the presence of high titres of IgG2 antibodies specific for the O-antigen of lipopolysaccharide (LPS) which impair serum-mediated killing of the cognate strain.2 In contrast to the serum bactericidal effect normally associated with antibody, this ‘inhibitory antibody’ prevented immune killing of the infecting strain.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
In individuals with cystic fibrosis (CF), severe pulmonary or liver disease is frequently addressed with lung or liver transplant, respectively. Specific endocrine co-morbidities including diabetes mellitus, osteoporosis, and adrenal insufficiency accompany solid organ transplant and may be particularly problematic in individuals with CF, who are already at increased risk of diabetes and compromised bone health. Diabetes and osteoporosis screening and initiation of appropriate preventive measures are recommended prior to transplant.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
In this study, the contractile and proliferative properties of ASM cells isolated from healthy donor and CF transplant lungs were compared. Proliferation assays showed CF ASM proliferate at a higher rate than healthy cells. Through calcium analysis, no differences in contractile activation was found, however, CF ASM lagged in their reuptake of calcium. The combination CFTR corrector and potentiator, VX-809/770, was used to restore proper CFTR function in CF ASM which resulted in a reduction in proliferation and in a normalization of calcium reuptake kinetics. These results show that improper CFTR function in ASM cause intr...
Source: Am J Physiol Lung Ce... - Category: Respiratory Medicine Authors: Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research
Despite improvements in the control of respiratory infections and life expectancy in cystic fibrosis (CF), highly resistant pathogens such as nontuberculous mycobacteria have emerged as an increasing challenge [1]. Disease due to M. abscessus is of particular concern as it is associated with increased healthcare utilisation, accelerated lung function decline, impaired quality of life (QoL) [2], adverse impacts on post-lung transplant outcomes [3], and increased mortality [2]. Good quality evidence to support current treatment regimens is lacking, and access to effective antimicrobial treatment options is limited.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Editorial Source Type: research
Abstract RATIONALE: Cystic fibrosis (CF) pulmonary disease is characterized by chronic infection with Pseudomonas aeruginosa and sustained neutrophil-dominant inflammation. The lack of effective anti-inflammatory therapies for people with CF (PWCF) represents a significant challenge. OBJECTIVES: To identify altered immunometabolism in the CF neutrophil, and investigate the feasibility of specific inhibition of the NLR family, pyrin domain-containing protein 3 (NLRP3) inflammasome as a CF anti-inflammatory strategy in vivo. METHODS: Key markers of increased aerobic glycolysis, known as a Warburg effect, i...
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
Lung transplantation is an established therapeutic option for patients with irreversible end-stage pulmonary disease limiting life expectancy and quality of life. Common indications for lung transplantation include chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis, pulmonary arterial hypertension, and alpha-1 antitrypsin deficiency. Complications of lung transplantation can be broadly divided etiologically into surgical, infectious, immunologic, or neoplastic. Moreover, specific complications often occur within a certain time interval following surgery, which can be broadly classified as...
Source: Journal of Thoracic Imaging - Category: Radiology Tags: Review Articles Source Type: research
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