成人内源性高胰岛素血症所致的低血糖症,通常继发于胰岛细胞瘤。家族性高胰岛素血症是罕见的,却几乎均发生于多发性内分泌肿瘤1型综合征(MEN-1)患者。作者首次报告无MEN-1的两例同胞兄妹患有成年发病的严重高胰岛素性低血糖症。例1 42岁男性,在家中出现意识不清,即抽血查血糖为0.94mmol/L,静滴葡萄糖液后,神志迅速恢复。患者晕厥反复发作15年,晕厥多在剧烈运动后20分钟发作,但运动前进食可预 Hypoglycemia due to endogenous hyperinsulinemia in adults is usually secondary to islet cell tumors. Familial hyperinsulinemia is rare but occurs almost exclusively in patients with multiple endocrine neoplasia type 1 syndrome (MEN-1). The authors first reported that two siblings without MEN-1 had severe hyperinsulinemic hypoglycemia with adult onset. Example 42 42-year-old man, at home consciousness, that blood glucose check 0.94mmol / L, intravenous glucose solution, the rapid recovery of consciousness. Syncope in patients with recurrent episodes of 15 years, syncope and more in strenuous exercise after 20 minutes attack, but pre-exercise food can be pre-