pulmonary alveolar proteinosis/hypoxia

To improve the diagnosis and treatment of pulmonary alveolar proteinosis, clinical data for the first successfully treated case of pulmonary alveolar proteinosis with severe hypoxemia by large-capacity whole lung lavage in our hospital were analyzed, and relevant literatures were reviewed. A

Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a

BACKGROUND Pulmonary alveolar proteinosis is characterized by accumulation of surfactant and phospholipids in the pulmonary alveoli. Whole lung lavage is considered the first-line therapy, which requires special techniques. To the best of our knowledge, there have only been limited reports that have

The profoundly hypoxemic child presents an interesting set of diagnostic and management challenges in the pediatric emergency department. While common pathologies including pneumonia, asthma, bronchiolitis, and pneumothoraces are managed using evidence-based algorithms, more enigmatic pathologies

Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the accumulation of proteinaceous material within alveoli. In order to evaluate the clinical features and the course of PAP we reviewed 7 cases (2F/5M) diagnosed during a 11-year period (1989-1999). The mean age of patients was

Whole lung lavage is still the most effective treatment for pulmonary alveolar proteinosis. We report a 21-year-old male diagnosed with pulmonary alveolar proteinosis by open lung biopsy and who underwent whole lung lavage with a modified technique. He showed significant improvement in clinical and

Previous studies demonstrated that antigranulocyte colony-stimulating factor autoantibody (GMAb) was consistently present in patients with autoimmune pulmonary alveolar proteinosis (aPAP), and, thus, represented candidature as a reliable diagnostic marker. However, our large cohort study suggested

Pulmonary alveolar proteinosis can result in severe hypoxemia. Treatment of symptomatic patients using unilateral or lobar staged lung lavage often results in improved oxygenation and functional capacity. Lung lavage is technically difficult in infants and small children because of inability to

In 8 patients with pulmonary alveolar proteinosis (7 with autoimmune pulmonary alveolar proteinosis and 1 with secondary pulmonary alveolar proteinosis related to Behcet disease), we performed unilateral total pulmonary lavage 41 times (including 2 sessions in which lavage was discontinued) between

BACKGROUND At present, the most effective treatment for pulmonary alveolar proteinosis (PAP) remains whole-lung lavage in spite of the usually accompanying severe hypoxemia, which is expected to be prevented by hyperoxygenated solution improving oxygen supply during lavage. In this study, the

Background: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with reduced lung function and resulting hypoxemia. It is characterized by a variable clinical course, and

Partial liquid ventilation (PLV) has been applied in various pulmonary diseases. We describe the use of partial liquid ventilation as a lavage method following normal saline (NS) lavage in an infant with pulmonary alveolar proteinosis (PAP) and severe hypoxemia. A 6 weeks old 3.4 kg former 36 weeks

Pulmonary alveolar proteinosis (PAP), which was first described by Rosen in 1958, is a rare disease characterized by impaired surfactant metabolism that provokes the accumulation of proteinaceous material in the alveoli. PAP is usually an auto-immune disease though, less commonly, may be congenital

To describe the clinical, radiological and pathological characteristics of idiopathic pulmonary alveolar proteinosis (I-PAP) and to evaluate the methods of diagnosis and treatment. Three patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed.

Pulmonary alveolar proteinosis is a rare but potentially treatable disease, characterized by impaired surfactant metabolism that leads to accumulation in the alveoli of proteinaceous material rich in surfactant protein and its component. Novel insights from an animal model aided the discovery of