Blue Rubber Bleb Nevus Syndrome: Successful Treatment With Sirolimus
Küçük Resim Yok
Tarih
2012
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
AMER ACAD PEDIATRICS
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic vascular malformations of the skin, gastrointestinal system, and, less often, other organ systems. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. The most serious complication is abundant gastrointestinal bleeding. We describe the case of an 8-year-old girl with diagnosed BRBNS who had multiple venous malformations all over her body, importantly, throughout the gastrointestinal tract, mouth, esophagus, stomach, small bowel, and colon. She presented with recurrent massive gastrointestinal bleeding and soft tissue hematoma despite prednisolone and a-interferon therapy. We started low-dose sirolimus as an antiangiogenic agent. The vascular masses were reduced rapidly and there was no gastrointestinal bleeding and muscular hematoma after sirolimus therapy. There was no drug adverse reaction at 20-month follow-up. To the best of our knowledge, this is the first report related to the use of sirolimus in a patient with BRBNS. Pediatrics 2012;129:e1080-e1084
Açıklama
Anahtar Kelimeler
gastrointestinal bleeding, hemangiomatosis, sirolimus, blue rubber bleb nevus syndrome
Kaynak
PEDIATRICS
WoS Q Değeri
Q1
Scopus Q Değeri
Q1
Cilt
129
Sayı
4
