Successful Management of Blue Rubber Bleb Nevus Syndrome (BRBNS) with Sirolimus
Blue rubber bleb nevus syndrome (BRBNS) is a rare disease with vascular malformations in several systems of the body, most commonly the skin and gastrointestinal tract. Bleeding from the gastrointestinal (GI) tract is a major complication, which may lead to chronic iron deficiency anemia and the nee...
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| Main Authors: | , , , |
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| Format: | Book |
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Hindawi Limited,
2018-01-01T00:00:00Z.
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| Summary: | Blue rubber bleb nevus syndrome (BRBNS) is a rare disease with vascular malformations in several systems of the body, most commonly the skin and gastrointestinal tract. Bleeding from the gastrointestinal (GI) tract is a major complication, which may lead to chronic iron deficiency anemia and the need for frequent blood transfusions due to ongoing gastrointestinal blood loss. In this case report, we describe a now 19-year-old female with BRBNS who required six blood transfusions per year and after starting sirolimus is symptom- and transfusion-free. |
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| Item Description: | 2090-6803 2090-6811 10.1155/2018/7654278 |