Pulmonary alveolar proteinosis is such an extremely rare disease in Korea, that only a few cases have been reported. Meanwhile five cases were experienced at Seoul National University Hospital over ten years since 1987. We summarized the clinical characteristics and courses of them. Seven cases reported in the literature were included to add data about clinical characteristics and courses although only a few case reports mentioned patient's course. Middle aged male patients were mainly affected. No association with particular environmental or occupational exposure was identified. Dyspnea on exertion was the main symptom. Bilateral crackles were consistent, and bilateral parahilar hazy infiltrations on plain chest radiograph and ground glass opacity on high-resolution CT were characteristic. Superimposed infection was not identified in any patient at the time of diagnosis. Decreased diffusing capacity and hypoxia were present in almost every case. Whole lung lavage proved to be an effective therapeutic measure. The response to treatment was good. Long-term course of the disease, e.g. recurrence rate, is not yet known.
Adult ; Age Distribution ; Aged ; Female ; Hospitals, Public* ; Hospitals, University* ; Human ; Korea ; Male ; Middle Age ; Pulmonary Alveolar Proteinosis/therapy ; Pulmonary Alveolar Proteinosis/radiography ; Pulmonary Alveolar Proteinosis/physiopathology* ; Pulmonary Alveolar Proteinosis/diagnosis ; Sex Distribution ; Smoking/adverse effects

Pulmonary alveolar proteinosis (PAP) is a noninflammatory diffuse lung disease, characterized by a dense accumulation of lipoproteinaceous material within the alveoli, causing hypoxemia, restrictive lung disease, and abnormalities on chest radiograph. The etiology of PAP is uncertain and various forms, including idiopathic and disease secondary to dust or fume exposure. Bronchopulmonary lavage (BPL) is a safe and effective treatment in PAP, and a unique procedure which requires general anesthesia and separation of the lung with a double lumen endobronchial tube. We experienced anesthetic management of BPL for the successful treatment of a 33 years old female patient with PAP.
Adult ; Anesthesia, General ; Anoxia ; Bronchoalveolar Lavage* ; Dust ; Female ; Humans ; Lung ; Lung Diseases ; Pulmonary Alveolar Proteinosis* ; Radiography, Thoracic

We had performed six times (4 in right lung, 2 in left lung) bronchoalveolar lavages in a patient who has hypertension and suffered cerebral vascular accident previously. The diagnosis of PAP was confirmed by an open lung biopsy and multiple bronchoalveolar lavages were done for two and a half months. Even though she showed slight improvement in chest radiographs, she showed minimal improvement in her clinical course until the third lavage was done. She had to receive tracheostomy and prolonged ventilatory care because her PAP was progressed rapidly and complicated with superinfection. In addition, she sustained pneumothorax as a complication of the prolonged ventilatory care and the lavages. She was discharged one month after the last lavage and her clinical course has been uneventful 10 months thereafter.
Biopsy ; Bronchoalveolar Lavage* ; Diagnosis ; Humans ; Hypertension ; Lung ; Pneumothorax ; Pulmonary Alveolar Proteinosis* ; Radiography, Thoracic ; Superinfection ; Therapeutic Irrigation ; Tracheostomy

Adult ; Aspergillosis ; etiology ; Humans ; Male ; Pulmonary Alveolar Proteinosis ; etiology ; Radiography, Thoracic ; Tomography, X-Ray Computed ; Tuberculosis ; complications

Pulmonary alveolar proteinosis is a rare disease, which hallmark is a dense accumulation of PAS positive phospholipid material within alveolar sac. Pulmonary alveolar proteinosis is classified as primary form of unknown etiology and secondary form associated with other diseases. We report a case of secondary pulmonary alveolar proteinosis associated with acute erythroleukemia. A C year old male patient complained of nonproductive cough and general weakness, and presented fine inspiratory crackles at both lower lung field. Chest radiographs and high resolution CT scans showd a lobular pattern of ground-grass opacity with interlobular septal thickening in the center field of the both lungs, Bone marrow aspiration and biopsy revealed acute erythroleukemia. Open lung biopsy revealed PAS positive eosinophilic granular material filled in alveoli. He was treated with TAD chemotherapy, but died from multiorgan failure with pneumonia 22days after chemotherapy.
Biopsy ; Bone Marrow ; Cough ; Drug Therapy ; Eosinophils ; Humans ; Leukemia, Erythroblastic, Acute* ; Lung ; Male ; Pneumonia ; Pulmonary Alveolar Proteinosis* ; Radiography, Thoracic ; Rare Diseases ; Respiratory Sounds ; Tomography, X-Ray Computed