Primary ciliary dyskinesia: A multicenter survey on clinical practice and patient management in Italy

Introduction There are no recent data on primary ciliary dyskinesia (PCD) distribution, diagnosis and treatment in Italy. Methods A descriptive study based on a survey questionnaire. It consisted of three sections (patients, diagnosis, and treatment), and sent to all the Italian PCD Centers. Results...

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Bibliographic Details
Published in:Pediatric pulmonology 2023-04, Vol.58 (4), p.1127-1135
Main Authors: Ullmann, Nicola, Santamaria, Francesca, Allegorico, Annalisa, Fainardi, Valentina, Borrelli, Melissa, Ferraro, Valentina A., Proietti, Elena, Parisi, Giuseppe F., Romagnoli, Vittorio, Lucca, Francesca, Gallucci, Marcella, Mappa, Luigi, Lelli, Mara, Amato, Doriana, Petrarca, Laura, Cimino, Giuseppe, Sacco, Oliviero, Calogero, Claudia, Patria, Maria Francesca, Acquafredda, Angelo, Ferlisi, Annalisa, Maschio, Massimo, Kantar, Ahmad, Cutrera, Renato
Format: Article
Language:English
Subjects:
Adult
Anti-Bacterial Agents - therapeutic use
Antibiotics
Child
Child, Preschool
children
Cilia
Ciliary Motility Disorders - diagnosis
Ciliary Motility Disorders - therapy
diagnosis
Dyskinesia
Humans
Italy
Kartagener Syndrome - diagnosis
Kartagener Syndrome - genetics
Kartagener Syndrome - therapy
management
Microscopy
Microscopy, Electron, Transmission
Pediatrics
primary ciliary dyskinesia
Surveys and Questionnaires
treatment
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Summary:Introduction There are no recent data on primary ciliary dyskinesia (PCD) distribution, diagnosis and treatment in Italy. Methods A descriptive study based on a survey questionnaire. It consisted of three sections (patients, diagnosis, and treatment), and sent to all the Italian PCD Centers. Results Questionnaires obtained from 20/22 centers in 12/20 regions showed that the total number of PCD patients treated at the participating centers was of 416. Out of all centers, 55% follow 40 patients, and 75% follow both pediatric and adults. Age at diagnosis was between 4 and 8 years in 45% of the centers, 50% of the patients have a known genetic profile. Patients treated at all centers receive inhaled saline solutions, corticosteroids and chest physiotherapy. Prophylactic antibiotics and mucolytics are prescribed in 95% and 50% of the centers, respectively. Pseudomonas infection is treated with oral or inhaled antibiotics. Conclusions Many Italian centers care for a small number of pediatric and adult patients, and diagnosis is often delayed. We found a great variability in the available diagnostic procedures, as well in the prescribed therapies. Our study will help to uniform diagnostic algorithm and share treatments protocols for PCD in Italy and allowed to set specific national goals.
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.26303