Applegate K E, Sargent S K
Department of Radiology, Children's Hospital, Boston, Massachusetts 02115, USA.
Pediatr Radiol. 1995;25(4):312-3. doi: 10.1007/BF02011113.
Familial dysautonomia, or Riley-Day syndrome, is a hereditary disturbance in the autonomic and peripheral sensory nervous systems, first described by Riley, Day, and colleagues in 1949 [1, 2]. Previous reports of myocardial infarction and avascular necrosis in bone suggest that these patients are at risk for ischemia at certain organ sites [3, 4]. We report a case of spontaneous colonic ischemia and stricture which resulted in colocutaneous fistula formation and eventual colonic resection in a child with Riley-Day syndrome.
家族性自主神经功能障碍，即赖利 - 戴综合征，是一种自主神经和周围感觉神经系统的遗传性疾病，1949年由赖利、戴及其同事首次描述[1,2]。先前关于心肌梗死和骨缺血性坏死的报道表明，这些患者在某些器官部位有缺血风险[3,4]。我们报告了一例患有赖利 - 戴综合征的儿童发生自发性结肠缺血和狭窄，导致结肠皮肤瘘形成并最终进行结肠切除术的病例。
