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.
View Article and Find Full Text PDFBackground: 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.
View Article and Find Full Text PDFWith 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.
View Article and Find Full Text PDFPeople 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.
View Article and Find Full Text PDFCystic 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.
View Article and Find Full Text PDFThis 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.
View Article and Find Full Text PDFBackground: 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.
View Article and Find Full Text PDFAim: This study examined the relationship between head growth and cognitive outcome at the age of five years in preterm infants born at less than 32 weeks of gestation from 2003 to 2009, as previous research has mostly focused on outcomes in toddlers.
Methods: The head circumference of 273 very preterm infants born in Tyrol, Austria, was measured at birth, discharge, the corrected ages of three, 12 and 24 months and the chronological age of five years. Suboptimal head size was defined as a head circumference of more than one standard deviation below the mean.
Aim: This study compared postnatal growth patterns calculated using different reference data in a large cohort of very preterm infants.
Methods: The weight, length and head circumference of 551 very preterm infants born in the Tyrol, Austria, between 2003 and 2011, were obtained at birth, discharge and the corrected ages of three, 12 and 24 months. Growth data are presented as Z-scores in relation to four reference populations: LMS growth by Pan et al.
Aim: In children with bilateral spastic cerebral palsy (CP), periventricular leukomalacia (PVL) is commonly identified on magnetic resonance imaging. We characterized this white matter condition by examining callosal microstructure, interhemispheric inhibitory competence (IIC), and mirror movements.
Method: We examined seven children (age range 11y 9mo-17y 9mo, median age 15y 10mo, four females, three males) with bilateral spastic CP/PVL (Gross Motor Function Classification System level I or II, Manual Ability Classification System level I) and 12 age-matched controls (age range 11y 7mo-17y 1mo, median age 15y 6mo, seven females, five males).
Objectives: The corpus callosum (CC) represents a key structure for hand motor development and is accessible to investigation by diffusion tensor magnetic resonance imaging (DTI) and transcranial magnetic stimulation (TMS). To identify quantifiable markers for motor development, we combined DTI with TMS.
Materials And Methods: We examined groups of 11 healthy preschool-aged children, 10 healthy adolescents, and 10 healthy adults with both, DTI and TMS/ipsilateral silent period (iSP).