Akutko Katarzyna, Krzesiek Elzbieta, Pytrus Tomasz, Iwańczak Barbara
IInd Chair and Department of Pediatry, Gastroenterology and Feeding, Medical University of Wrocław, Poland.
Pol Merkur Lekarski. 2012 Jun;32(192):394-6.
Blue rubber bleb nevus syndrome (BRBNS or Bean's syndrome) is a rare disease characterized by the interaction of hemangiomas of rubber-like tenacity in the skin and gastrointestinal tract, rarely in other organs. Cutaneous malformations are usually asymptomatic and not require any treatment. The most common symptoms of gastrointestinal tract are iron deficiency anemia and bleeding. Hemangiomas can occur in any organ and cause a wide spectrum of symptoms. BRBNS can cause massive bleeding and even death. In this article we present a case of Bean's syndrome in a 7,5-year-old girl with bleeding from the lower gastrointestinal tract, which has been caused by hemangiomas located in the skin, large intestine, chest and armpit.
蓝色橡皮疱痣综合征(BRBNS 或 Bean 综合征)是一种罕见疾病,其特征为皮肤和胃肠道出现具有橡皮样韧性的血管瘤相互作用,很少累及其他器官。皮肤畸形通常无症状,无需任何治疗。胃肠道最常见的症状是缺铁性贫血和出血。血管瘤可发生于任何器官并引发广泛的症状。BRBNS 可导致大量出血甚至死亡。在本文中,我们报告了一例 7.5 岁女孩患 Bean 综合征的病例,其下消化道出血是由位于皮肤、大肠、胸部和腋窝的血管瘤所致。