Revue des maladies respiratoires
-
The provision of ambulatory oxygen in addition to a stationary oxygen supply has been suggested to increase adherence to long-term oxygen therapy (LTOT) and promote ambulation. The degree of adherence to ambulatory oxygen therapy and the determinants of adherence remain largely unknown. ⋯ Adherence to ambulatory oxygen in this series of patients is quite low but at the higher end of data reported previously. Patients with MMRC 4 dyspnoea scale had a lower use of ambulatory oxygen despite reporting better adherence to LTOT. We could not identify any other predictive factors of adherence to ambulatory oxygen.
-
Primary ciliary dyskinesia (PCD) is an inherited disease responsible for a disruption of normal ciliary function. Its clinical presentation is usually in early childhood with pulmonary and otorhinolaryngologic symptoms. Early diagnosis is essential to avoid the development of bronchiectasis. The aim of the study was to retrospectively review the clinical features of children suspected to have PCD. ⋯ This case series highlights the key clinical features of recurrent otitis, sinusitis, and situs inversus, especially when occurring in combination with bronchitic symptoms. Measures of nasal nitric oxide are useful for the diagnosis of PCD and in the case of high levels of NO, PCD is unlikely. Results may not be definitive and TEM analysis of biopsies is still indispensable to ensure the diagnosis and guide genetic counselling.
-
Idiopathic pulmonary fibrosis (IPF) is a severe chronic lung disease. Pulmonary rehabilitation could improve the quality of life of patients with this condition. ⋯ A home-based programme of pulmonary rehabilitation is feasible in IPF patients. It significantly improved endurance parameters and physical limitation in this patient group without changing pulmonary function.