A5047261074- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Tracheal and airway disorders
- Cardiovascular Function and Risk Factors
- Cardiac Imaging and Diagnostics
- Respiratory viral infections research
- Immunodeficiency and Autoimmune Disorders
- Child Nutrition and Feeding Issues
- Pediatric health and respiratory diseases
- Liver Disease Diagnosis and Treatment
- Renal and Vascular Pathologies
- Congenital Ear and Nasal Anomalies
- Asthma and respiratory diseases
- Inhalation and Respiratory Drug Delivery
- Pancreatitis Pathology and Treatment
- Atrial Fibrillation Management and Outcomes
- Infant Nutrition and Health
- Cardiac pacing and defibrillation studies
- Pediatric Hepatobiliary Diseases and Treatments
- Acute Kidney Injury Research
- Hepatitis C virus research
- Hepatitis B Virus Studies
- Cardiac electrophysiology and arrhythmias
- Intestinal Malrotation and Obstruction Disorders
- Infections and bacterial resistance
Meyer Children's Hospital
2016-2025
Istituti di Ricovero e Cura a Carattere Scientifico
2024
Ospedali Riuniti di Foggia
2021-2024
University of Foggia
2018-2024
Ospedale Santa Maria
2023
Medizinische Hochschule Hannover
2021
Royal Manchester Children's Hospital
2021
Nicolae Testemițanu State University of Medicine and Pharmacy
2021
University of Florence
2021
Meyer (China)
2021
The aims of this study were to evaluate the frequency Achromobacter xylosoxidans infection in a cohort cystic fibrosis patients, investigate antimicrobial sensitivity, establish possible clonal likeness among strains, and address clinical impact or colonization on general outcome these patients. was undertaken between January 2004 December 2008 300 patients receiving care at Regional Cystic Fibrosis Center Naples University "Federico II". Sputum samples checked for bacterial identification....
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:...
<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:...
Improved therapy in CF has led to an overall improvement nutritional status. The objectives of our study are: cross-sectionally assess status and serum levels fat-soluble vitamins; retrospectively evaluate the efficacy modulators on vitamin levels.In patients younger than 2 years age, we evaluated growth, aged 2-18 years, assessed BMI z-scores, adults, absolute values. Levels 25(OH)D, vitamins A, E were measured.A cross-sectional analysis was conducted 318 patients, 109 (34.3%) with...
Abstract Background Elexacaftor‐tezacaftor‐ivacaftor (ETI) is a highly effective cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulating therapy for people with CF and at least one F508del variant. However, there limited data about the safety efficacy of this in pediatric populations real‐world settings. This study aimed evaluating effectiveness, tolerability, ETI children CF. Methods was prospective observational including all aged 6–11 years who initiated between October...
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators represent targeted therapies directly acting on the CFTR channel. The triple therapy Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) has been demonstrated to improve lung function and quality of life in cystic (CF) patients. However, effects ELX/TEZ/IVA sleep-disordered breathing (SDB) respiratory muscle strength are poorly studied. aim this study was assess patients with CF severe disease cardiorespiratory polygraphy...
Pharmacological rescue of mutant cystic fibrosis transmembrane conductance regulator (CFTR) in (CF) depends on the specific defect caused by different mutation classes. We asked whether a patient with rare p.Gly970Asp (c.2909G>A) could benefit from CFTR pharmacotherapy since similar missense p.Gly970Arg (c.2908G>C) was previously found to be sensitive potentiators vitro but not vivo. By complementary DNA transfection, we that both mutations are associated defective function amenable...
ABSTRACT Background New drugs that target the basic defect in cystic fibrosis (CF) patients may now be used a large number of carrying responsive mutations. Nevertheless, further research is needed to extend benefit these treatments with rare mutations are still uncharacterized vitro and not included clinical trials. For this purpose, ex vivo models necessary preliminary assessing effect CFTR modulators cases. Method We report effectiveness lumacaftor/ivacaftor therapy prescribed CF child...