Cytokines Measured in Nasal Lavage Compared to Induced Sputum in Patients with Mild Cystic Fibrosis.

The measurement of cytokines in induced sputum and nasal lavage (NL) samples has been performed for years in people with cystic fibrosis (CF). The aim of this study was to directly compare sputum and NL samples and interpret results based on disease severity in patients who were categorized as having mild or severe lung disease. The categorization was based primarily on structural abnormalities detected on lung computed tomography and secondarily on lung function.

Detection of cytokines in nasal lavage samples of patients with cystic fibrosis: comparison of two different cytokine detection assays.

Background: Cystic fibrosis (CF) is a genetic multisystem disorder. Inflammatory processes, which presumably begin early in infancy, play a crucial role in the progression of the disease. The detection of inflammatory biomarkers, especially in the airways, has therefore gained increasing attention.

Long-Term Assessment of Antibody Response to COVID-19 Vaccination in People with Cystic Fibrosis and Solid Organ Transplantation.

With the worldwide spread of SARS-CoV-2 disease, people with cystic fibrosis (CF), especially solid organ transplant recipients, have quickly been identified as a risk group for severe disease. Studies have shown low antibody response to SARS-CoV-2 vaccines in recipients of solid organ transplant compared to the healthy population. Information on immune response in CF patients with solid organ transplantation is limited, especially regarding long-term efficacy.

Is There a Difference in Immune Response to SARS-CoV-2 Vaccination between Liver and Lung Transplant Patients with Cystic Fibrosis?

People with Cystic Fibrosis (CF), especially solid organ transplant recipients, have been prioritized in the SARS-CoV-2 vaccination program. This study assesses antibody response of patients with CF who have undergone liver (CF-LI) or lung (CF-LU) transplantation, and compares results to published data of patients with solid organ transplantation without CF as underlying disease. Antibodies against the spike receptor-binding domain were measured within the routine visits at the CF Centre in Innsbruck, Austria, after the second and third doses of SARS-CoV-2 mRNA vaccines.

Early and sustained improvements of lung clearance index from two to sixteen weeks of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis-a real world study.

Since the introduction of CFTR modulator therapies, longitudinal real-life data of lung clearance index (LCI) during treatment is scarce. In this single-centre, post-approval setting, we report data of 51 patients with different stages of lung disease, age 2-52 years with repeated measurements of forced expiratory volume as a percentage of the predicted value (ppFEV₁) and LCI after 2, 4, and 16 weeks of CFTR modulator treatment and at baseline. In 25 patients during elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) treatment, significant improvements of LCI (median -1.

Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary?

Cystic fibrosis (CF) is caused by two mutations in the Cystic Fibrosis Transmembrane Conductance Regulator () gene. In the last years, drugs targeting the underlying protein defect like lumacaftor/ivacaftor (LUM/IVA) or tezacaftor/ivacaftor (TEZ/IVA) and more recently elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) were admitted. Outcome parameters evaluating therapy response like forced expiratory pressure in 1 s (FEV), body mass index (BMI) or the efficacy of function in sweat glands showed improvement in several cases.

REAL-world clinical effectiveness of ivacaftor therapy in the first 24 months in two infants with cystic fibrosis and different gating mutations-A case report.

This study summarizes efficacy of ivacaftor treatment in 2 infants in a real-world setting. A distinct decline of sweat chloride and lung clearance index plus increase in fecal elastase was seen. The results underline the early and sustainable effect and give cause for discussing whether a reduction in standard cystic fibrosis therapy is possible.

Malignancies in patients with cystic fibrosis: a case series.

Background: Previous reports have shown an increased number of colorectal cancers in patients with cystic fibrosis. We assessed the database of our cystic fibrosis center to identify patients with all kinds of cancer retrospectively. All patients visiting the Cystic Fibrosis Centre Innsbruck between 1995 and 2019 were included.