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Primary Lymphedema: Update on Genetic Basis and Management

Resource type
Authors/contributors
Title
Primary Lymphedema: Update on Genetic Basis and Management
Abstract
Significance: Primary lymphedema is a chronic condition without a cure. The lower extremities are more commonly affected than the arms or genitalia. The disease can be syndromic. Morbidity includes decreased self-esteem, infections, and reduced function of the area. Recent Advances: Several mutations can cause lymphedema, and new variants continue to be elucidated. A critical determinant that predicts the natural history and morbidity of lymphedema is the patient's body mass index (BMI). Individuals who maintain an active lifestyle with a normal BMI generally have less severe disease compared to subjects who are obese. Because other causes of lower extremity enlargement can be confused with lymphedema, definitive diagnosis requires lymphoscintigraphy. Critical Issues: Most patients with primary lymphedema are satisfactorily managed with compression regimens, exercise, and maintenance of a normal body weight. Suction-assisted lipectomy is our preferred operative intervention for symptomatic patients who have failed conservative therapy. Suction-assisted lipectomy effectively removes excess subcutaneous fibro-adipose tissue and can improve underlying lymphatic function. Future Directions: Many patients with primary lymphedema do not have an identifiable mutation and thus novel variants will be identified. The mechanisms by which mutations cause lymphedema continue to be studied. In the future, drug therapy for the disease may be developed.
Publication
Advances in Wound Care
Date
2021-01-27
Journal Abbr
Adv Wound Care (New Rochelle)
Language
eng
ISSN
2162-1918
Short Title
Primary Lymphedema
Library Catalog
PubMed
Citation
Sudduth, C. L., & Greene, A. K. (2021). Primary Lymphedema: Update on Genetic Basis and Management. Advances in Wound Care. https://doi.org/10.1089/wound.2020.1338
Topic