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A 30 year old housewife presenting with cor pulmonale was diagnosed as having pulmonary alveolar microlithiasis. Exponential analysis of static pressure-volume data obtained through whole body plethysmography revealed decreased compliance, normal shape constant and reduced extrapolated lung volume at infinite distending pressures. These changes were consistent with a restrictive ventilatory defect due to alveolar filling process, and closely correlate with the lung histology seen in this disease.
