Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis.

Lung clearance index (LCI) is a biomarker of ventilation inhomogeneity. Data are scarce on its usefulness in daily practice for monitoring the effects of treatments in older children and adults with CF. In this French observational study of lumacaftor-ivacaftor, 63 of 845 patients (7.

[National consensus regarding azithromycin use in cystic fibrosis].

Aim: To propose a formalized consensus agreement regarding the prescription of azithromycin in cystic fibrosis (CF).

Material And Methods: Application of the Delphi method in 5 thematic fields: indications, contra-indications, dosage, precautions for use and treatment follow-up.

Results: Thirty identified French CF centers participated in the process on 49 (61%), which comprised 3 rounds.

[Mild cystic fibrosis: genetics - extending follow-up is necessary].

The diagnosis of mild cystic fibrosis is first suspected on mild lung disease or absence of pancreatic insufficiency and is assessed by biological analysis. The sweat test is not always conclusive. The nasal potential difference and molecular analysis of CFTR gene allow confirming diagnosis.

[Epidemiological and clinical study of Mycoplasma pneumoniae respiratory infections in children hospitalized in a pediatric ward between 1999 and 2005 at the Reims University Hospital].

Object: To determine the frequency, clinical features, and morbidity of Mycoplasma pneumoniae infections.

Method: Retrospective study of 76 consecutive children under 16 years of age hospitalized at the Reims University Hospital from 1999 to 2005 with M. pneumoniae pneumonia.

Feasibility of nasal epithelial brushing for the study of airway epithelial functions in CF infants.

Background: For a better understanding of the early stages of cystic fibrosis (CF), it is of major interest to study respiratory epithelial cells obtained as early as possible. Although bronchoalveolar lavage has been proposed for this purpose, nasal brushing, which is a much less invasive technique, has seldom been used in CF infants. The aim of the present study was to examine in a few infants the feasibility of a nasal brushing technique for studies of airway epithelial functions in very young CF infants.

[Neonatal diagnosis of primary ciliary dyskinesia: report of one case].

Primary ciliary dyskinesia is a rare, genetic disorder resulting of an abnormal ultrastructural morphology of cilia. Such disease is rarely recognized in neonatal period. We report on a newborn who exhibited unexplained respiratory distress.