The Science Journal of the American Association for Respiratory Care

2010 OPEN FORUM Abstracts

CASE REPORT: 72 YEAR OLD MALE PRESENTING WITH PULMONARY ALVEOLAR PROTEINOSIS

Michael Bocci, Ken Hargett, Kim Bloom, Jose Rodriguez, Romar Reyes; Respiratory Care Services, The Methodist Hospital, Houston, TX

Introduction: The prevalence of acquired Pulmonary Alveolar Proteinosis (PAP) has been estimated to be 0.37 per 100,000 persons. Excessive thick granular phospholipoproteinaceous material protein builds up in the alveoli of the lungs. In some cases, the cause of the Pulmonary Alveolar Proteinosis is unknown; in others, it is associated with infection or immune deficiency. Patients present with abnormal chest x-rays, decreased oxygenation and Pulmonary Function Tests show restrictive lung disease with abnormal diffusion. This rare disorder generally affects people 30-50 years old and is seen in men more than women. Treatment consists of periodically washing out the protein substance from the lung with complete lung lavage. Lung transplant may be recommended for patients with the disease. Case Summary: The patient is a 72 year-old male presenting with severe hypoxemia (PaO2 of 49 mmHg). Bronchoscopy was performed, and transbronchial biopsies obtained. The result was nondiagnostic. Subsequently an open-lung biopsy was completed which demonstrated an accumulation of granular material. These findings confirmed the diagnosis of PAP. Complete lung lavage was scheduled in 2 separate sessions. The patient was intubated with a double lumen tube. The right lung was degassed by clamping the right-sided port for 5 minutes and ventilating only the left lung. Right lung lavage was accomplished by repeatedly instilling 750 to 1000cc of warmed normal saline solution and allowing each instillation to drain by gravity. The lavage fluid initially was opaque and fairly thick. With repeated instillations, the fluid cleared and was translucent and minimally cloudy. A vibratory percussor vest was utilized to improve distribution of the fluid. The pulmonogist utilized 24 liters (L) of normal saline with a return of 23.9L. The left lung lavage was completed the following week. 28L were instilled with a return of 29.4L. The increased returned volume was secondary to what was washed out of the lung. Significant improvement in oxygenation occurred after each procedure and PaO2 increased to normal levels. The patientÂ’s dyspnea was also resolved. Seven months have passed and the patient has not needed a repeat of the lung lavages. Discussion: Though a rare disease, Respiratory Therapist should be familiar with the diagnosis and treatment of PAP. Sponsored Research - None