Genetics of Klippel-Trenaunay-Weber Syndrome Medication

Updated: Apr 08, 2022
  • Author: Ravi Sunderkrishnan, MD; Chief Editor: Maria Descartes, MD  more...
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Medication

Antiplatelet agents

Class Summary

These agents inhibit platelet function by blocking cyclooxygenase production and subsequent aggregation.

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

Inhibits prostaglandin synthesis preventing formation of platelet-aggregating thromboxane A2. May be used in low dose to inhibit platelet aggregation and improve complications of venous stases and thrombosis.

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Corticosteroids

Class Summary

These agents elicit anti-inflammatory and immunosuppressive properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisolone (Pediapred)

Used to treat coagulopathy. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.

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PI3K Inhibitors

Class Summary

As a result of gain-of-function mutations in the gene encoding the catalytic alpha subunit of phosphatidylinositol-3 kinase (PIK3CA), activation of the PI3K-alpha and Akt-signaling cellular transformation occurs. PIK3CA gene mutations have been found in many, but not all, patients with Klippel-Trenaunay syndrome. [19]  

Alpelisib (Vijoice)

Indicated for patients aged 2 years or older who have severe manifestations of PIK3CA-related overgrowth spectrum (PROS) and are in need of systemic therapy.

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