Fridlyand Diana, Wilder Caroline, Clay E Leila Jerome, Gilbert Bruce, Pace Betty S
Department of Pediatrics, Augusta University, GA, USA.
Department of Radiology, Augusta University, GA, USA.
Pediatr Rep. 2017 Mar 22;9(1):6984. doi: 10.4081/pr.2017.6984.
Children with hemoglobin SC (HbSC) disease suffer a significant incidence of silent cerebral infarcts but stroke is rare. A 2-year-old African American boy with HbSC disease presented with focal neurologic deficits associated with magnetic resonance imaging evidence of cerebral infarction with vascular abnormalities. After the acute episode he was treated with monthly transfusions and subsequently transitioned to hydroxyurea therapy. The benefits of hydroxyurea as a fetal hemoglobin inducer in HbSC disease, to ameliorate clinical symptoms are supported by retrospective studies. This case highlights the rare occurrence of stroke in a child with HbSC disease and the use of hydroxyurea therapy.
患有血红蛋白SC(HbSC)病的儿童无症状性脑梗死的发生率很高,但中风很少见。一名患有HbSC病的2岁非裔美国男孩出现局灶性神经功能缺损,磁共振成像显示有脑梗死及血管异常。急性发作后,他接受了每月一次的输血治疗,随后转为羟基脲治疗。回顾性研究支持羟基脲作为HbSC病中胎儿血红蛋白诱导剂改善临床症状的益处。本病例突出了HbSC病患儿中风的罕见情况以及羟基脲疗法的应用。