Parker Torrey M, Ward Leanne M, Johnston Donna L, Ventureya Enrique, Klaassen Robert J
Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.
Pediatr Blood Cancer. 2009 Mar;52(3):422-4. doi: 10.1002/pbc.21850.
Moyamoya syndrome is a rare diagnosis that has been linked to a small number of hemoglobinopathies. Children with Moyamoya syndrome tend to present with transient ischemic attacks, mental deficiency, and/or neurological deficits. We describe a case of a 15-year-old Cambodian male with HbE/beta-thalassemia who was found to have left Moyamoya syndrome as part of an evaluation for growth hormone deficiency. The link between Moyamoya syndrome and HbE/beta-thalassemia may be multifactorial, but Moyamoya syndrome is an important consequence to consider in children with HbE/beta-thalassemia.
烟雾病综合征是一种罕见的诊断,已与少数血红蛋白病相关联。患有烟雾病综合征的儿童往往表现为短暂性脑缺血发作、智力缺陷和/或神经功能缺损。我们描述了一例15岁柬埔寨男性,患有HbE/β地中海贫血,在评估生长激素缺乏症时被发现患有左侧烟雾病综合征。烟雾病综合征与HbE/β地中海贫血之间的联系可能是多因素的,但烟雾病综合征是HbE/β地中海贫血患儿需要考虑的一个重要后果。