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Nonne–Milroy–Meige syndrome
Classification and external resources
ICD-10 Q82.0
ICD-9 757.0
OMIM 153100
DiseasesDB 8228
eMedicine med/1482
MeSH D008209

Nonne–Milroy–Meige syndrome is a congenital lymphangiectasia that causes swelling of the soft tissues in which an excessive amount of lymph has accumulated,[1]:849 including a protein-losing enteropathy.[2] It is also known as Nonne-Milroy-Meige syndrome, hereditary lymphedema, and intestinal lymphangiectasia with peripheral lymphedema.

It is named for William Milroy.[3][4]

Description

It usually begins in childhood. Steatorrhea caused by impaired transport of chylomicrons by the intestinal lymphatics. Lymphopenia, impaired cell-mediated immunity and Chylous ascites are also seen. The most obvious clinical presentation is unilateral lower extremity edema usually in young females that can progress to bilateral edema.

An association with FLT4 has been described.[5]

External Links

References

  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.  
  2. ^ David Lowell Strayer; Raphael Rubin (2007). Rubin's Pathology: Clinicopathologic Foundations of Medicine 5th Edition. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-9516-8.  
  3. ^ synd/1326 at Who Named It?
  4. ^ W. F. Milroy. An undescribed variety of herditary edema. New York Medical Journal, 1892, 56: 505-508.
  5. ^ Spiegel R, Ghalamkarpour A, Daniel-Spiegel E, Vikkula M, Shalev SA (2006). "Wide clinical spectrum in a family with hereditary lymphedema type I due to a novel missense mutation in VEGFR3". Journal of human genetics 51 (10): 846–50. doi:10.1007/s10038-006-0031-3. PMID 16924388. http://dx.doi.org/10.1007/s10038-006-0031-3.  
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