A5084110830- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Pediatric Hepatobiliary Diseases and Treatments
- Tracheal and airway disorders
- Child Nutrition and Feeding Issues
- Drug Transport and Resistance Mechanisms
- Genetic and Kidney Cyst Diseases
- Respiratory viral infections research
- Thyroid Cancer Diagnosis and Treatment
- Infant Nutrition and Health
- Asthma and respiratory diseases
- Clinical Nutrition and Gastroenterology
- Pediatric health and respiratory diseases
- Esophageal and GI Pathology
- Pneumonia and Respiratory Infections
- Inhalation and Respiratory Drug Delivery
- Liver Disease Diagnosis and Treatment
- Metabolism and Genetic Disorders
- Congenital Ear and Nasal Anomalies
- Nematode management and characterization studies
- Mycobacterium research and diagnosis
- Neonatal Health and Biochemistry
- Child Nutrition and Water Access
- Respiratory Support and Mechanisms
- Antifungal resistance and susceptibility
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico
2015-2024
University of Milan
2015-2024
IRCCS Istituto Auxologico Italiano
2018-2024
Istituti di Ricovero e Cura a Carattere Scientifico
2007-2024
Ospedale Maggiore
2005-2022
Federico II University Hospital
2021-2022
Cystic Fibrosis Trust
2021
Cystic Fibrosis Research Foundation
2003-2019
AT Sciences (United States)
2016
Children's Hospital Agia Sophia
2015
Cystic fibrosis is a life-limiting disease that caused by defective or deficient cystic transmembrane conductance regulator (CFTR) protein activity. Phe508del the most common CFTR mutation.We conducted two phase 3, randomized, double-blind, placebo-controlled studies were designed to assess effects of lumacaftor (VX-809), corrector, in combination with ivacaftor (VX-770), potentiator, patients 12 years age older who had and homozygous for mutation. In both studies, randomly assigned receive...
The taxonomic position of members the Mycobacterium abscessus complex has been subject intensive investigation and, in some aspects confusion, recent years as a result varying approaches to genetic data interpretation. Currently, former species massiliense and bolletii are grouped together subsp. bolletii. They differ greatly, however, M. functional erm(41) gene that confers inducible resistance macrolides, primary therapeutic antimicrobials for abscessus, while is non-functional....
Elexacaftor–tezacaftor–ivacaftor is a small-molecule cystic fibrosis transmembrane conductance regulator (CFTR) modulator regimen shown to be efficacious in patients with at least one Phe508del allele, which indicates that this combination can modulate single allele. In whose other CFTR allele contains gating or residual function mutation already effectively treated previous modulators (ivacaftor tezacaftor–ivacaftor), the potential for additional benefit from restoring protein unclear.
Early and detailed characterisation of coronavirus disease 2019 (COVID-19) has emerged principally through publications from China, where the was first identified.1Guan W-J Ni Z-Y Hu Y et al.Clinical characteristics in China.N Engl J Med. 2020; (published online Feb 28.)DOI:10.1056/NEJMoa2002032Crossref Google Scholar As pandemic spread to Lombardia, Northern Italy then globally,2Remuzzi A Remuzzi G COVID-19 Italy: what next?.Lancet. March...
Incidence of liver disease (LD) associated with cystic fibrosis (CF) and its clinical characterization still is unsettled. We have assessed prospectively the incidence risk factors this complication, impact on course CF. Between 1980 1990, we enrolled 177 CF patients without LD in a systematic clinical, laboratory, ultrasonography screening program at least 10-year duration. During 14-year median follow-up (2,432 patient-years), 48 developed LD, cirrhosis already present 5. rate (number...
Liver disease is increasingly recognized as a major cause of morbidity in cystic fibrosis (CF). Preliminary data suggest that ursodeoxycholic acid (UDCA) may be beneficial for treatment this manifestation. We performed double-blind, multicenter trial these patients to establish efficacy and safety UDCA terms the improvement clinical nutritional indicators besides standard liver function tests. also intended whether taurine supplementation has effect receiving UDCA. From June December 1990,...
Various definitions for distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent, and constipation in patients with cystic fibrosis (CF) are used. However, an unequivocal definition DIOS, is preferred. The aims of this study were, therefore, to seek consensus on the DIOS CF determine incidence, characteristics, treatment a cohort paediatric CF.During 2005 European Society Paediatric Gastroenterology, Hepatology, Nutrition meeting Porto group gastroenterologists discussed CF....
ABSTRACT Objectives: Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. Knowledge the underlying pathological aspects optimal clinical management is, however, sorely lacking. Methods: We provide summary lectures given by international speakers at European Society for Paediatric Gastroenterology, Hepatology, Nutrition (ESPGHAN) monothematic conference on fibrosis–related liver disease (CFLD) held Paris January 2016, to discuss status...
Previous studies from our groups have demonstrated improvements in biochemical markers of liver function when cystic fibrosis patients with associated disease were administered oral ursodeoxycholic acid. The magnitude the response was somewhat less than that found comparable doses (10 to 15 mg/kg body wt/day) acid are given other patients; this may be explained by bile malabsorption is characteristic disease. For reason a dose-response study carried out nine establish whether improved...
We have previously documented that ursodeoxycholic acid exerts a beneficial effect on liver function and bile metabolism in patients with cystic fibrosis. hypothesized the mechanism of action may be related part to choleretic properties administered acid. therefore compared hepatobiliary scintigraphic images obtained before 1 yr after initiation therapy document an improvement flow 13 fibrosis involvement. Before therapy, scintigraphy biliary stasis retention isotope intrahepatic...
Mutations in the cystic fibrosis (CF) transmembrane conductance regulator affect innate epithelial immune function of lung, resulting exaggerated and ineffective airway inflammation that fails to eradicate pathogenic fungi. The appreciation whether such fungi are primarily responsible for or a consequence is important future therapeutics development.To characterize impact tryptophan/kynurenine pathway on preventing effective fungal clearance CF.We studied expression indoleamine...