A5090434699- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Tracheal and airway disorders
- Inhalation and Respiratory Drug Delivery
- Pediatric health and respiratory diseases
- Immunodeficiency and Autoimmune Disorders
- Respiratory viral infections research
- Asthma and respiratory diseases
- Cardiac Ischemia and Reperfusion
- Central Venous Catheters and Hemodialysis
- Blood Pressure and Hypertension Studies
- Cerebrospinal fluid and hydrocephalus
- Cardiac Health and Mental Health
- Cardiac Arrhythmias and Treatments
- Olfactory and Sensory Function Studies
- Pharmaceutical studies and practices
- Child Nutrition and Feeding Issues
- Esophageal and GI Pathology
- Bacterial biofilms and quorum sensing
- Congenital Heart Disease Studies
- Cholesterol and Lipid Metabolism
- Vascular anomalies and interventions
- COVID-19 Impact on Reproduction
- Infant Nutrition and Health
- Genetic and Kidney Cyst Diseases
University of Naples Federico II
2016-2025
Regione Campania
2006-2024
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico
2021-2022
Medizinische Hochschule Hannover
2021
Cystic Fibrosis Research Foundation
2020-2021
Federico II University Hospital
2014-2021
Ospedale Monaldi
2012
University of Foggia
2012
Elexacaftor/tezacaftor/ivacaftor (ETI) is the newest cystic fibrosis transmembrane conductance regulator (
Introduction: Modulators of cystic fibrosis transmembrane conductance regulator mutated protein significantly improved the outcome patients with (CF). We describe 63 who were independently followed up in two CF regional centers (i.e., Campania and Tuscany regions). Methods: All homozygous for F508del mutation treated lumacaftor/ivacaftor (LI) 3 years, by 1 year treatment elexacaftor/tezacaftor/ivacaftor (ETI). studied biochemical parameters liver damage cholesterol metabolism. Results:...
Recent reports document an increasing incidence of new Gram-negative pathogens such as Stenotrophomonas maltophilia and Alcaligenes xylosoxidans isolated from patients with Cystic Fibrosis, along increase in common Pseudomonas aeruginosa Burkholderia cepacia complex. Furthermore, the multidrug-resistance organisms makes therapeutic management these more problematic. Therefore, careful isolation identification, accurate studies susceptibility to antibiotics are critical for predicting spread...
<h3>Background</h3> The effect of complex alleles in cystic fibrosis (CF) is poorly defined for the lack functional studies. <h3>Objectives</h3> To describe genotype–phenotype correlation and results either vitro ex vivo studies performed on nasal epithelial cells (NEC) a cohort patients with CF carrying <i>cystic transmembrane conductance regulator</i> (<i>CFTR</i>) alleles. <h3>Methods</h3> We studied 70 homozygous, compound heterozygous or <i>CFTR</i> mutations:...
In cystic fibrosis (CF) patients airways mucus shows an increased viscoelasticity due to the concentration of high molecular weight components. Such thickening eventually leads bacterial overgrowth and prevents clearance. The altered rheological behavior results in chronic lung infection inflammation, which causes most cases morbidity mortality, although complications affect other organs as well. Here, we present a quantitative study on correlation between clinical status. particular, new...
Abstract The clinical manifestation of cystic fibrosis (CF) is heterogeneous also in patients with the same transmembrane regulator (CFTR) genotype and affected sibling pairs. Other genes, inherited independently CFTR , may modulate complications CF, including severity chronic sinonasal disease occurrence Pseudomonas aeruginosa colonization. T2R38 gene encodes a taste receptor recently its functionality was related to diseases upper respiratory infections. We assessed 210 CF 95 controls,...
The last ten years have been characterized by an enormous step forward in the therapy and management of patients with Cystic Fibrosis (CF), thanks to development combination Transmembrane Receptor (CFTR) correctors potentiators. Specifically, approved triple elexacaftor/tezacaftor/ivacaftor has demonstrated improve lung function CF both homozygous Phe508del Phe508del/minimal genotypes. Here we assessed effect carrying genotype (n = 20) after one year treatments on liver nutrient absorption a...
Italian cystic fibrosis registry (ICFR) collects data from (CF) patients through the collaboration with CF referral and support Centres (Italian law 548/93). ICFR contributes: • to analysis of medium long term clinical epidemiological trends disease; identification main health care needs at regional national level contribute Health Care programmes distribution resources; comparison international ones. This latter is based on European and, due COVID-19 pandemic emergency, important global...
The development of targeted therapies that correct the effect mutations in patients with cystic fibrosis (CF) and relevant heterogeneity clinical expression disease require biomarkers correlated to severity useful for monitoring therapeutic effects. We applied a metabolomic approach by LC-MS/MS on saliva samples from 70 adult CF 63 age/sex-matched controls investigate alterations metabolic pathways related pancreatic insufficiency (PI), Pseudomonas aeruginosa (PA) colonization, liver (CFLD),...
Background Drugs with anticholinergic properties are frequently prescribed to patients cognitive impairment. The cholinergic system plays an important role in learning processes, memory, and emotions regulation. aim of this research is report use drugs a clinical population investigate the correlation between risk presenting more severe behavioral psychological symptoms (BPSD). Method Patients diagnosis subjective impairment, mild impairment (MCI) or dementia were recruited. Screening tests...
Background/Objective: Cystic Fibrosis (CF) is a common, life-threatening genetic disorder that leads to progressive lung function decline, respiratory failure, and premature death. Musculoskeletal complications, affecting both peripheral muscles, are major concerns in CF patients. Inflammatory cytokines seem be responsible for the activation of molecular pathways involved imbalance between protein synthesis catabolism, with consequent loss muscle mass function. This study aims assess effects...
A clinical heterogeneity was reported in patients with Cystic Fibrosis (CF) the same CFTR genotype and between siblings CF.We investigated all aspects a cohort of 101 pairs CF (including 6 triplets) followed since diagnosis.Severe lung disease had 22.2% concordance sib-pairs, occurred early FEV1% at 12 years predictive severity adulthood. Similarly, liver (median: 15 years) showed 27.8% sib-pairs suggesting scarce contribution genetic factors; fact, only 2/15 discordant deficiency...
Cystic fibrosis is a hereditary metabolic disorder characterized by impaired traffic of chloride ions and water through membranes the respiratory gastrointestinal, that causes inadequate hydration airway surfaces, dehydrated mucous secretions high-sodium sweat. Although classical presentation condition well known, better characterization alterations related need. In particular, composition biological fluids may be influence disease state could captured as putative signature to set targeted...
Modulators of cystic fibrosis transmembrane conductance regulator (CFTR) improved (CF) patients' outcome. The elexacaftor/tezacaftor/ivacaftor (ETI) combination was safe and effective improving lung function in patients with different CFTR genotypes, including at least one F508del mutation. However, cases liver damage were reported. We describe 105 CF heterozygous for trans another mutation, treated 1 year ETI. analyzed biochemical parameters cholesterol metabolism, lathosterol phytosterols,...
Respiratory infections represent a major threat for people affected by cystic fibrosis, leading to pulmonary deterioration and lung transplantation as therapeutic option end-stage patients. A fast correct identification of pathogens in airway fluid these patients is crucial establish appropriate therapies, prevent cross-infections and, ultimately, preserve function. In this study, we used Raman spectroscopy reveal bacteria the sputa such Pseudomonas aeruginosa Staphylococcus aureus, which...
Background Cystic fibrosis (CF) is a multisystem disease that involves the upper airways with chronic rhinosinusitis (CRS) causing nasal congestion, rhinorrhea, mouth breathing, facial pain, and olfactory dysfunction. Twelve percent to 71% of CF patients report smelling alterations an impact on nutrition quality life. Objectives The goal was study olfaction performance in CRS worsens Methods A total 121 subjects were enrolled this study. Seventy-one had underwent ear, nose, throat evaluation...
Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There no chest magnetic resonance imaging (MRI) comparative studies of PCD CF. We assessed clinical, functional, microbiological MRI findings in mild CF patients order to evaluate different expression lung disease. Twenty (15.1 years) 20 subjects with respiratory impairment (16 years, 70% pancreatic insufficiency) underwent MRI, spirometry, sputum cultures when clinically stable. was scored using the...