Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Benesch, M; Lackner, H; Sorantin, E; Haxhija, E; Gasparella, P; Pfleger, A.
Drug Treatment of Vascular Anomalies.
PADIATR PADOL. 2020; 55(1): 21-27.
Doi: 10.1007/s00608-019-00739-3
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- Führende Autor*innen der Med Uni Graz
- Benesch Martin
- Co-Autor*innen der Med Uni Graz
- Gasparella Paolo
- Haxhija Emir
- Lackner Herwig
- Pfleger Andreas
- Sorantin Erich
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- Abstract:
- Vascular anomalies are divided according to the International Society for the Study of Vascular Anomalies into two major groups, vascular tumors, which include the most common forms kaposiform hemangioendothelioma and hemangioma, and vascular malformations. Management of vascular anomalies requires a multidisciplinary approach and is based, depending on the entity, on surgical resection, sclero-/cryotherapy, embolization, and cytostatic and/or antiangiogenic drug therapy. Owing to expansion and mostly diffuse infiltrative growth, complete resection of vascular anomalies is achievable in only a minority of patients. Since the first description of the efficacy of propranolol in 2008, it has become established as the first-line treatment for infants with complicated hemangiomas. Sirolimus, an immunosuppressive drug well-known in organ transplantation that inhibits the mammalian/mechanistic target of rapamycin (mTOR) pathway, also exerts antiangiogenic activity, and is therefore increasingly being used for the treatment of vascular tumors and vascular malformations. Three infants with vascular anomalies (kaposiform hemangioendothelioma, mixed macro-/microcystic lymphatic malformation, and diffuse infantile hepatic hemangioma) received propranolol-/sirolimus-based treatment without developing any clinically relevant side effects. Although treatment is generally extremely well tolerated, the serious, potentially life-threatening side effects (propranolol: cardiovascular; sirolimus: infectious) that are reported in a minority of patients require special consideration.
- Find related publications in this database (Keywords)
- Kaposiform hemangioendothelioma
- Lymphatic malformation
- Infantile hepatic hemangioma
- Propranolol
- Sirolimus