pulmonary alveolar phospholipoproteinosis; alveolar proteinosis; pulmonary surfactant metabolism dysfunction; inborn error of pulmonary surfactant metabolism

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Etiology

Epidemiology

  • rare
  • affects mostly young adults
  • male:female ratio 3:1

Pathology

Genetics

  • autosomal recessive
  • associated with defects in CSF2RB
  • associated with defects in SFTPB (type 1)
  • associated with defects in SFTP (type 2)
  • associated with defects in ABCA3 (type 4)

Clinical manifestations

Diagnostic procedures

Radiology

Complications

Differential diagnosis

(similar histopathology)

Management

More general terms

Additional terms

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 756
  2. 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 14, American College of Physicians, Philadelphia 2006
  3. 3.0 3.1 Abdelmalak BB, Khanna AK, Culver DA, Popovich MJ. Therapeutic Whole-Lung Lavage for Pulmonary Alveolar Proteinosis: A Procedural Update. J Bronchology Interv Pulmonol. 2015 Jul;22(3):251-8 PMID: https://www.ncbi.nlm.nih.gov/pubmed/26165897

Database