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Fibroadipose vascular anomaly treated with sirolimus: Successful outcome in two patients

机译:用西罗司治疗纤维型脂素血管异常:两名患者的成功结果

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摘要

Abstract Fibroadipose vascular anomaly (FAVA) is a rare, complex mesenchymal malformation combining fibrofatty replacement of the affected muscles and slow‐flow vascular malformation. The condition is characterized by localized swelling, severe pain, phlebectasia, and contracture of the affected limb. Treatment paradigms are not well established for this rare, recently recognized condition. We report two cases of FAVA in which treatment with sirolimus produced rapid, dramatic improvement in pain and quality of life.
机译:摘要纤维型胶质血糖异常(FAVA)是一种罕见的复杂的间充质畸形,结合了纤维缺陷的受影响的肌肉和缓冲血管畸形。 该病症的特征在于受影响的肢体的局部肿胀,严重的疼痛,痰湿和挛缩。 治疗范式对这种罕见的,最近认可的病症并不充分建立。 我们报告了两种美孚患者,其中西罗莫司治疗迅速,急剧提高痛苦和生活质量。

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