Matthias Griese
A5065547785- Neonatal Respiratory Health Research
- Cystic Fibrosis Research Advances
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Congenital Diaphragmatic Hernia Studies
- Medical Imaging and Pathology Studies
- Inhalation and Respiratory Drug Delivery
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Asthma and respiratory diseases
- Tracheal and airway disorders
- Neuroscience of respiration and sleep
- Respiratory Support and Mechanisms
- Pulmonary Hypertension Research and Treatments
- Respiratory viral infections research
- Pediatric health and respiratory diseases
- Neonatal Health and Biochemistry
- Respiratory and Cough-Related Research
- Pneumocystis jirovecii pneumonia detection and treatment
- Pharmaceutical studies and practices
- Transplantation: Methods and Outcomes
- RNA modifications and cancer
- Pleural and Pulmonary Diseases
- Pneumonia and Respiratory Infections
- Drug Transport and Resistance Mechanisms
- Cholesterol and Lipid Metabolism
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
Ludwig-Maximilians-Universität München
2016-2025
German Center for Lung Research
2016-2025
LMU Klinikum
1992-2025
München Klinik
2013-2023
University of Amsterdam
2023
Panagiotis & Aglaia Kyriakou Children's Hospital
2023
National and Kapodistrian University of Athens
2023
Sotiria General Hospital
2023
Baylor College of Medicine
2023
American College of Medical Genetics
2023
Increasing the activity of defective cystic fibrosis transmembrane conductance regulator (CFTR) protein is a potential treatment for fibrosis.
Abstract The Human Phenotype Ontology (HPO, https://hpo.jax.org) was launched in 2008 to provide a comprehensive logical standard describe and computationally analyze phenotypic abnormalities found human disease. HPO is now worldwide for phenotype exchange. has grown steadily since its inception due considerable contributions from clinical experts researchers diverse range of disciplines. Here, we present recent major extensions the neurology, nephrology, immunology, pulmonology, newborn...
Primary ciliary dyskinesia (PCD) is characterized by recurrent airway infections and randomization of left-right body asymmetry. To date, autosomal recessive mutations have only been identified in a small number patients involving DNAI1 DNAH5, which encode outer dynein arm components.We screened 109 white PCD families originating from Europe North America for presence DNAH5 haplotype analyses and/or sequencing.Haplotype excluded linkage 26 families. In 30 families, we 33 novel (12 nonsense,...
Abstract The Human Phenotype Ontology (HPO) is a widely used resource that comprehensively organizes and defines the phenotypic features of human disease, enabling computational inference supporting genomic analyses through semantic similarity machine learning algorithms. HPO has widespread applications in clinical diagnostics translational research, including diagnostics, gene-disease discovery, cohort analytics. In recent years, groups around world have developed translations from English...
Background In two pivotal phase 3 trials, up to 24 weeks of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in patients cystic fibrosis (CF) ≥12 years age who have at least one F508del allele. The aim this study is assess long-term safety efficacy ELX/TEZ/IVA these patients. Methods 3, open-label, single-arm extension study, participants –minimal function (from a 24-week parent study; n=399) or – 4-week n=107) genotypes receive the same dose (ELX 200 mg...
Various inflammatory diseases are characterized by tissue infiltration of neutrophils. Chemokines recruit and activate leukocytes, but neutrophils traditionally known to be restricted in their chemokine receptor (CR) expression repertoire. Neutrophils undergo phenotypic functional changes under conditions, the mechanisms regulating CR infiltrated at sites chronic inflammation poorly defined. Here we show that from patients with lung rheumatoid arthritis highly express on surface absent or...
The airways of cystic fibrosis (CF) patients are characterised by neutrophils that release high amounts elastase overwhelming the local antiprotease shield. Inhalation α 1 -antitrypsin (AAT) may restore protease–antiprotease balance and attenuate airway inflammation in CF airways. aims present study were: 1) to assess best deposition region for inhaled AAT two different inhalation strategies; 2) examine effect 4 weeks on lung function, patients. In a prospective, randomised study, 52...
Rationale: Tezacaftor (formerly VX-661) is an investigational small molecule that improves processing and trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) in vitro, CFTR function alone combination with ivacaftor.Objectives: To evaluate safety efficacy tezacaftor monotherapy tezacaftor/ivacaftor therapy subjects homozygous for F508del or compound heterozygous G551D.Methods: This was a randomized, placebo-controlled, double-blind, multicenter, phase 2 study...