FFC#20/2016

Italian multicenter study of glucose tolerance defects in cystic fibrosis

FFC#20/2016

Italian multicenter study of glucose tolerance defects in cystic fibrosis

PRINCIPAL INVESTIGATOR

Alberto Battezzati (Centro Internazionale per lo Studio della Composizione Corporea – ICANS, DeFENS, Università degli Studi di Milano)

Partner

Carla Colombo (Dip. di Scienze Materno Infantili, Università di Milano e Centro Regionale FC, Fondazione IRCCS Ospedale Maggiore Policlinico Mangiagalli e Regina Elena, Clinica Pediatrica De Marchi, Milano); Vincenzina Lucidi (Ospedale Pediatrico Bambin Gesù, Roma); Giuseppe Magazzù (Dip. di Patologia Umana dell’adulto e dell’età evolutiva Gaetano Barresi, Università di Messina); Andrea Mari (Istituto di Neuroscienze, Consiglio Nazionale delle Ricerche IN-CNR, Padova)

RESEARCHERS

18

CATEGORY

AREA 5 Clinical and Epidemiological research

DURATION

2 years

GOAL

€ 80.000 €

RESULTS

This multicentric study allowed to perform 406 OGTTs with insulin secretory and C-peptide data. Insulin and c-peptide assays provided comparable measures among centers. Modeling is underway as well as statistical analysis for reference values and relationship with clinically relevant biomarkers. Preliminary analysis from the Milan group (inclusive of previously collected data) showed that Glucose Sensitivity and Insulin concentration at 30’ are the main correlates of lung function and long term predictors of CFRD and adult height. Sex and age-adjusted nomograms of OGTT parameters will be carried out and implemented among Italian centers: they are proposed as powerful tools to describe the glucose tolerance defects natural history, to predict future CFRD, nutritional and respiratory decay and to set a rational basis for treatment. A collaboration has been set with a US multicenter group, that will replicate the project protocol and analysis in an equal number of patients, with the aim to produce common international reference values.


Pubblications

Colombo C, Alicandro G, Gambazza S et al. “Ventilation inhomogeneity is associated with OGTT-derived insulin secretory defects in cystic fibrosis” Pediatr Pulmonol 2018 Dec 21. doi: 10.1002/ppul.24212. [Epub ahead of print]

Congress abstracts

– Nazzari E, Guarise R, Mileto P et al. “Relationship between glucose and insulin response during an oral glucose tolerance test (OGTT) and lung clearance index in cystic fibrosis patients” XIII Congresso Nazionale SIFC, Napoli, 22-25 Novembre 2017

OTHER RESULTS

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Two molecules are effective in activating Heat Shock Proteins and enhancing the action of CFTR correctors with the F508del mutation in vitro.

FFC#5/2024

Some peptide nucleic acids (PNAs) re-sensitise Pseudomonas aeruginosa to the antibiotic meropenem in vitro and reduce its virulence.

FFC#1/2023

Tezacaftor, one of the components of Kaftrio, induces an accumulation of dihydroceramides both in vitro and in vivo in animal models