Cheung Amanda C, Hachem Christine Y, Lai Jinping
Division of Gastroenterology and Hepatology, Department of Internal Medicine, SSM Health Saint Louis University Hospital, 3635 Vista Avenue, Saint Louis, MO, 63110, USA.
Division of Anatomic Pathology, Department of Pathology, SSM Health Saint Louis University Hospital, Saint Louis, MO, USA.
Clin J Gastroenterol. 2016 Aug;9(4):238-42. doi: 10.1007/s12328-016-0661-8. Epub 2016 Jun 13.
Idiopathic hypereosinophilic syndrome (HES) is a rare diagnosis defined by the World Health Organization as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. While many patients present with nonspecific symptoms, others will present with symptoms of the affected organs, most commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with hepatitis and achalasia related to idiopathic HES.
特发性嗜酸性粒细胞增多综合征(HES)是一种罕见的诊断疾病,世界卫生组织将其定义为持续嗜酸性粒细胞增多6个月并导致终末器官功能障碍。虽然许多患者表现为非特异性症状,但其他患者会出现受累器官的症状,最常见的是涉及心脏、皮肤或神经系统的症状。据估计,高达三分之一的HES患者会出现胃肠道或肝脏受累,尽管具有临床显著疾病的患者仅限于病例报告。这是首例报告的与特发性HES相关的肝炎和贲门失弛缓症患者。