Portal de Periódicos da CAPES

Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children

2009; European Respiratory Society; Volume: 34; Issue: 6 Linguagem: Inglês

10.1183/09031936.00176608

1399-3003

Antonio Barbato, Thomas Frischer, Claudia E. Kuehni, Deborah Snijders, Inês Azevedo, G Baktai, Lucia Bartoloni, Ernst Eber, Amparo Escribano, Eric G. Haarman, Bill Hesselmar, Claire Hogg, Mark Jorissen, Jane S. Lucas, Kim G. Nielsen, C O’Callaghan, Heymut Omran, Petr Pohunek, Marie‐Pierre F. Strippoli, Andrew Bush,

Neonatal Respiratory Health Research

Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, which results in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility. The diagnosis of PCD requires the presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by transmission electron microscopy or evidence of abnormal ciliary function. Although the management of children affected with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage. This European Respiratory Society consensus statement on the management of children with PCD formulates recommendations regarding diagnostic and therapeutic approaches in order to permit a more accurate approach in these patients. Large well-designed randomised controlled trials, with clear description of patients, are required in order to improve these recommendations on diagnostic and treatment approaches in this disease.