Chest
Clinicai Investigations: Important TopicsPulmonary Alveolar Proteinosis: Clinical Features and Outcomes
Section snippets
Materials and Methods
To ascertain eligible cases, a list of patients designated under Diagnosis-Related Group 519.2 (PAP) in the hospital database was assembled. Inclusion criteria were the presence of PAP as confirmed by a physician diagnosis and a consistent lung biopsy. All available slides were reviewed by a lung pathologist (Dr. Farver) to confirm the diagnosis of PAP.
The medical records of eligible patients were reviewed by one of the study investigators, and data elements were extracted using a standard data
Results
Twenty-four patients with PAP were identified (17 were male, 7 were female). Their mean age (± SD) was 42 ± 13.5 years (range, 21 to 67 years of age), and 18 patients were current or former smokers at the time of the initial presentation (mean pack-years, 29). All patients were white. One of the following systemic diseases was present in each of five patients (21%): breast cancer, myelodysplasia, pericardial disease, inflammatory bowel disease, and prune belly syndrome. No patients were known
Discussion
This study extends the available experience with PAP (Table 1) and also makes several new observations. As in prior reports, PAP was more common among men than women, with typical onset in the fifth decade of life. As in the reports by Rosen et al1 and Prakash et al,3 dyspnea also was a common presenting symptom among patients in the current series. Also, physical examination was largely unrevealing; the pulmonary physiologic profile of our patients was that of mild restriction with a
References (24)
- et al.
Pulmonary alveolar proteinosis: experience with 34 cases and a review
Mayo Clin Proc
(1987) - et al.
Pulmonary alveolar proteinosis
Br J Dis Chest
(1969) - et al.
Management of pulmonary alveolar proteinosis
Chest
(1980) - et al.
Pulmonary alveolar proteinosis: a case report and a review of the literature
Dis Chest
(1966) Pulmonary alveolar proteinosis
Ann Thorac Surg
(1968)- et al.
Update on the clinical diagnosis, management and pathogenesis of pulmonary alveolar proteinosis (phospholipidosis)
Chest
(1984) - et al.
Diagnosing pulmonary alveolar proteinosis
Chest
(1997) - et al.
Surfactant protein-A concentration in bronchoalveolar lavage fluids of patients with pulmonary alveolar proteinosis
Chest
(1993) - et al.
Pulmonary alveolar proteinosis
N Engl J Med
(1958) Pulmonary alveolar proteinosis: review of the literature with follow-up studies and a report of two new cases
Calif Med
(1966)
Primary pulmonary alveolar proteinosis: clinical observations of 68 patients in Japan
Jpn J Thorac Dis
Pulmonary alveolar proteinosis: CT findings
Radiology
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