lymphangioleiomyomatosis

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Etiology

Epidemiology

Pathology

* image (lung biopsy, gross pathology)[11]

Genetics

Clinical manifestations

Laboratory

Diagnostic procedures

Radiology

* image (chest CT)[11]

Complications

  • often fatal

Management

More general terms

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 756-57
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015
  3. UniProt http://www.uniprot.org/uniprot/P49815.html
  4. McCormack FX, Inoue Y, Moss J et al Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011 Apr 28;364(17):1595-606 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21410393
  5. Meraj R, Wikenheiser-Brokamp KA, Young LR, McCormack FX. Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med. 2012 Oct;33(5):486-97. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23001803
  6. 6.0 6.1 6.2 6.3 Weinstock M, Vaidya A, El-Chemaly S, et al The Verge of Collapse. INTERACTIVE MEDICAL CASE N Engl J Med 2018;378:e18. March 29, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29590548
  7. Henske EP, McCormack FX Lymphangioleiomyomatosis - a wolf in sheep's clothing. J Clin Invest. 2012 Nov;122(11):3807-16. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23114603 Free PMC Article
  8. McCormack FX, Travis WD, Colby TV, Henske EP, Moss J. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. Am J Respir Crit Care Med. 2012 Dec 15;186(12):1210-2. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23250499 Free PMC Article
  9. Goldberg HJ, Harari S, Cottin V et al Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study.wal S, Cheng Y, Khindri S, Kovarik JM, Ma S, McCormack FX, Henske EP. Eur Respir J. 2015 Sep;46(3):783-94. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26113676 Free Article
  10. McCormack FX, Gupta N, Finlay GR et al Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. Am J Respir Crit Care Med. 2016 Sep 15;194(6):748-61. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27628078 Free PMC Article
  11. 11.0 11.1 11.2 11.3 11.4 11.5 Sclafani A, VanderLaan P. Lymphangioleiomyomatosis. N Engl J Med 2018; 378:2224. June 7, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29874537 https://www.nejm.org/doi/full/10.1056/NEJMicm1712581

Patient information

lymphangioleiomyomatosis patient information

Database