A5102916037- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Sphingolipid Metabolism and Signaling
- Lysosomal Storage Disorders Research
- Lipid Membrane Structure and Behavior
- Glycosylation and Glycoproteins Research
- Pancreatic function and diabetes
- Diabetes and associated disorders
- Moyamoya disease diagnosis and treatment
- Respiratory viral infections research
- Pharmacological Effects and Toxicity Studies
- Probiotics and Fermented Foods
- Mitochondrial Function and Pathology
- Inhalation and Respiratory Drug Delivery
- Microbial Metabolites in Food Biotechnology
- Celiac Disease Research and Management
- Metabolism and Genetic Disorders
- Neurological Complications and Syndromes
- Digestive system and related health
- Diabetes Management and Research
- Cellular transport and secretion
- Inflammasome and immune disorders
- Endoplasmic Reticulum Stress and Disease
- Asthma and respiratory diseases
- Immunodeficiency and Autoimmune Disorders
Vita-Salute San Raffaele University
2022-2023
Istituti di Ricovero e Cura a Carattere Scientifico
2022-2023
Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele
2022-2023
University of Milan
2015-2020
University of Perugia
2016-2019
CFTR modulators have been developed to correct and/or enhance activity in patients with specific cystic fibrosis (CF) genotypes. However, it is of great importance identify potential off-targets these novel therapies understand how they affect lung physiology CF.
This study was planned to evaluate whether a 3-month treatment with Lactobacillus rhamnosus GG (LGG) can modify immune system functions in children and adolescents type 1 diabetes (T1D), leading an increased response injectable quadrivalent inactivated influenza vaccine (QIV). A total of 87 pediatric patients T1D were screened, although 34 the Probiotic group 30 Control accepted be vaccinated QIV completed study. Vaccine immunogenicity safety inflammatory cytokine studied. Results showed...
Objectives Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized by focal neurological signs, headache, confusion, seizure, associated with transitory lesions in the posterior areas of brain detectable neuroimaging. Among children, one most common causes PRES cancer. Materials Methods In this review, we present cases 5 children developing after stem cell transplantation for hematological disease review all reported English literature to...
Cystic fibrosis (CF) is the most common autosomal genetic recessive disease caused by mutations of gene encoding for cystic transmembrane conductance regulator. Patients with CF display a wide spectrum symptoms, severe being chronic lung infection and inflammation, which lead to onset disease. Several studies indicate that sphingolipids play regulatory role in airway inflammation. The inhibition downregulation GBA2, enzyme catabolizing glucosylceramide ceramide, are associated significant...
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein is expressed at the apical plasma membrane (PM) of different epithelial cells. The most common mutation responsible for onset cystic fibrosis (CF), F508del, inhibits biosynthesis and transport PM, also presents gating stability defects anion channel upon its rescue by use correctors potentiators. This prompted a multiple drug strategy F508delCFTR aimed simultaneously rescue, functional potentiation PM stabilization. Since...
Human primary bronchial epithelial cells differentiated in vitro represent a valuable tool to study lung diseases such as cystic fibrosis (CF), an inherited disorder caused by mutations the gene coding for Cystic Fibrosis Transmembrane Conductance Regulator. In CF, sphingolipids, ubiquitous class of bioactive lipids mainly associated with outer layer plasma membrane, seem play crucial role establishment severe complications. Nevertheless, no information on involvement sphingolipids and their...
To compare growth and glycemic control in children with type 1 diabetes silent celiac disease treated a gluten-free diet for year those of similar age gender but without disease, 16 patients were enrolled each disease-positive case was matched age, sex, duration two controls negative serologic markers disease. All positive serology had histologic features consistent despite the absence symptoms. The mean metabolic values between This study seems to suggest that early diagnosis initiation may...
Abstract Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve clinical outcomes with variable efficacy in patients cystic (CF). However, changes produced by bacterial persistence and adaptation addition to antibiotic regimens could influence CFTR modulator vice versa hence outcomes. We first evaluated the effects of ivacaftor (IVA), lumacaftor (LUM), tezacaftor, elexacaftor elexacaftor/tezacaftor/ivacaftor (ETI), alone or combined antibiotics, on sequential...