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Pulmonary alveolar microlithiasis.

Udaya B S Prakash1

  • 1Pulmonary, Critical Care, and Internal Medicine, Mayo Medical School and Mayo Medical Center, Rochester, Minnesota 55905-0001, USA. prakash.udaya@mayo.edu

Seminars in Respiratory and Critical Care Medicine
|August 10, 2005
PubMed
Summary
This summary is machine-generated.

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Pulmonary alveolar microlithiasis is a rare lung disease where calcium phosphate deposits form in the alveoli. This condition progresses slowly and currently has no cure, though lung transplants are an option for severe cases.

Area of Science:

  • Pulmonology
  • Rare Diseases
  • Medical Diagnosis

Background:

  • Pulmonary alveolar microlithiasis (PAM) is a rare lung disorder of unknown cause.
  • Characterized by intra-alveolar deposition of calcium phosphate microliths.
  • Approximately 400 cases reported globally, affecting individuals from newborns to 80 years.

Purpose of the Study:

  • To summarize the key features, diagnosis, and management of Pulmonary Alveolar Microlithiasis.
  • To highlight the diagnostic modalities and current therapeutic limitations.

Main Methods:

  • Literature review of reported cases of Pulmonary Alveolar Microlithiasis.
  • Analysis of patient demographics, clinical presentation, diagnostic findings, and treatment outcomes.

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Main Results:

  • PAM typically presents with a slow progression and a restrictive pattern on pulmonary function tests.
  • Diagnosis is aided by imaging (chest X-ray, HRCT, Tc-99m lung scan) and confirmed by bronchoalveolar lavage or biopsy.
  • No specific therapy exists; lung transplantation is considered for end-stage disease with respiratory insufficiency and cor pulmonale.

Conclusions:

  • Pulmonary Alveolar Microlithiasis is a rare, slowly progressing lung disease with no established cure.
  • Early diagnosis through imaging and biopsy is crucial.
  • Management focuses on supportive care and considering lung transplantation for advanced cases.