ABSTRACT
Tracheobronchopathia osteochondroplastica (TPO) is an idiopathic disease of the trachea and major bronchi characterized by multiple submucosal osteocartilaginous nodules. The nodules may be either focal or diffuse, and typically spare the membranous wall of the airways. Symptoms are non-specific, and include dry cough, dyspnea, recurrent respiratory infections and occasionally hemoptysis. TPO is rarely considered as a diagnosis in part due to lack of awareness among clinicians. The diagnosis can be based on a typical bronchoscopic appearance and generally does not require biopsy of the lesions. When available, histology reveals bone formation within the submucosa with normal overlying respiratory epithelium. TPO is a benign disorder, marked by a generally favorable clinical evolution. There is currently no established treatment for the removal of airway nodules, or the prevention of further tissue growths. Interventional bronchoscopy techniques have a role in the relief of symptomatic airway obstruction, when indicated.
Subject(s)
Bronchial Diseases/diagnosis , Osteochondrodysplasias/diagnosis , Tracheal Diseases/diagnosis , Bronchial Diseases/etiology , Bronchial Diseases/therapy , Humans , Osteochondrodysplasias/etiology , Osteochondrodysplasias/therapy , Tracheal Diseases/etiology , Tracheal Diseases/therapyABSTRACT
Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterised by intra-alveolar accumulation of surfactant components and cellular debris, with minimal interstitial inflammation or fibrosis. Since surfactant accumulates abnormally, a disturbance in the normal pathway of surfactant production, metabolism, recycling or degradation has been postulated. This disease has a variable clinical course: from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infections. PAP leading to pulmonary fibrosis is rarely seen, and few case reports describe this association. Here, we describe the case of a patient with a diagnosis of PAP confirmed by open lung biopsy, who developed interstitial pulmonary fibrosis years after disease onset.
Subject(s)
Pulmonary Alveolar Proteinosis/complications , Pulmonary Alveolar Proteinosis/pathology , Pulmonary Fibrosis/etiology , Female , Humans , Middle Aged , Pulmonary Fibrosis/diagnosisABSTRACT
Patients with obstructive sleep apnoea syndrome frequently have cognitive deficits, especially related to executive functions, which cannot be fully explained by daytime sleepiness and are partial irreversible after nasal continuous positive airway pressure treatment. The causal mechanism of these cognitive deficits is not yet known, but it has been proposed that they are associated with chemical and structural brain cell injury. The aim of this study was to investigate brain metabolism in patients with sleep apnoea syndrome. Twenty-two patients with severe sleep apnoea and 10 healthy volunteers of comparable age were studied using single voxel proton magnetic resonance spectroscopy. Magnetic resonance spectra were obtained from prefrontal cortex, parieto-occipital and frontal periventricular white matter. N-acetylaspartate-to-creatine and choline-to-creatine ratios were significantly lower in the frontal white matter of obstructive sleep apnoea patients when compared to controls. Absolute concentrations of N-acetylaspartate and choline were also significantly reduced in the frontal white matter of patients with sleep apnoea. Frontal lobe white matter lesions are known to be associated with cognitive executive dysfunction. The findings of this study may offer an explanation for the sometimes irreversible cognitive deficits associated with sleep apnoea.
