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Pulmonary alveolar microlithiasis. A review including ultrastructural and pulmonary function studies
Abstract:
Pulmonary alveolar microlithiasis is a rare disease of unknown cause in which calcium phosphate microliths are deposited throughout the lungs. These deposits are of sufficient density to be almost diagnostic on chest roentgenograms. The Mayo Clinic experience with 8 patients is added to the approximately 120 cases reported in the world literature. The age range of all patients is from newborn to 80 years, with a mean age at diagnosis of about 35 years. No sexual predominance has been noted, but in about half of the reported cases a familial pattern has been found. The progression of the disease is generally very slow, some patients having been followed up for more than 30 years without evidence of change. No specific treatment is available. Pulmonary function studies demonstrate a tendency toward a restrictive pattern. Technetium-99m scanning and scanning and transmission electron microscopy are useful procedures for analysis of pulmonary alveolar microliths.
Insights
Pulmonary alveolar microlithiasis is a rare lung disease characterized by calcium phosphate deposits. Diagnosis is often aided by chest X-rays, with no specific treatment currently available.
Area of Science:
- Pulmonology
- Rare Diseases
- Radiology
Background:
- Pulmonary alveolar microlithiasis (PAM) is a rare interstitial lung disease.
- Characterized by diffuse deposition of calcium phosphate microliths in the alveoli.
- Etiology remains largely unknown.
Purpose of the Study:
- To present the Mayo Clinic experience with 8 patients diagnosed with PAM.
- To add to the existing global case reports (approx. 120).
- To review clinical characteristics, diagnostic findings, and disease progression.
Main Methods:
- Review of 8 patient cases from Mayo Clinic.
- Analysis of worldwide literature on approximately 120 reported cases.
- Evaluation of diagnostic imaging (chest roentgenograms), pulmonary function studies, and specialized analyses (Technetium-99m scanning, transmission electron microscopy).
Main Results:
- The disease affects a wide age range (newborn to 80 years), with a mean age at diagnosis around 35 years.
- No sexual predilection observed; familial occurrence noted in about 50% of cases.
- Disease progression is typically very slow, with some patients stable for over 30 years. Pulmonary function studies often show a restrictive pattern.
Conclusions:
- Pulmonary alveolar microlithiasis is a rare, slowly progressive lung disease diagnosed via characteristic radiographic findings.
- No definitive treatment exists; management focuses on supportive care.
- Advanced imaging and microscopy aid in the analysis of pulmonary microliths.