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Sirolimus in the Management of Blue Rubber Bleb Nevus Syndrome: A Case Report and Review of the Literature
Blue rubber bleb nevus syndrome (BRBNS) is a rare multifocal venous malformation (VM) that may affect any tissue or organ but mainly affects the skin, subcutaneous tissue and gastrointestinal (GI) tract. Patients present with serious anemia, treated with lifelong iron supplements and frequent blood...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8436651/ https://www.ncbi.nlm.nih.gov/pubmed/34594200 http://dx.doi.org/10.1159/000511535 |
Sumario: | Blue rubber bleb nevus syndrome (BRBNS) is a rare multifocal venous malformation (VM) that may affect any tissue or organ but mainly affects the skin, subcutaneous tissue and gastrointestinal (GI) tract. Patients present with serious anemia, treated with lifelong iron supplements and frequent blood transfusion secondary to chronic GI bleeding. Variable therapeutic modalities were used in the management of BRBNS; sirolimus (SRL), a mammalian target of rapamycin (mTOR) inhibitor, is found to be a promising therapy for vascular anomalies. |
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