CAJ | 학술논문

糖原累积症(glycogen storage disease,GSD)是一组少见的遗传性糖原代谢紊乱疾病,又称糖原沉着症.因为GSD的发生率较低,世界上的研究中心缺乏大量的病例,因而对其远期治疗缺乏丰富的经验及广泛的共识.近年来,随着医疗水平的提高和GSD病人寿命的延长,继发肝腺瘤(hepatocellular adenoma,HCA)的病人越来越多.GSD继发肝腺瘤的发展会产生占位,压迫,出血,甚至癌变等并发症,需要积极的治疗以挽救病人的生命.本文就肝糖原累积症继发肝腺瘤的发病机理及外科治疗作一综述.
당원루적증(glycogen storage disease,GSD)시일조소견적유전성당원대사문란질병,우칭당원침착증.인위GSD적발생솔교저,세계상적연구중심결핍대량적병례,인이대기원기치료결핍봉부적경험급엄범적공식.근년래,수착의료수평적제고화GSD병인수명적연장,계발간선류(hepatocellular adenoma,HCA)적병인월래월다.GSD계발간선류적발전회산생점위,압박,출혈,심지암변등병발증,수요적겁적치료이만구병인적생명.본문취간당원루적증계발간선류적발병궤리급외과치료작일종술.
Glycogen storage disease (GSD) is a rela-tively rare inherited metabolic disease. However, its relative rarity implies that no metabolic centre has experience of large numbers of patients and experience with long-term manage-ment is limited. In addition, there is wide variation in meth-ods of medical treatment. With the development of medical care, life-expectancy in glycogen storage disease (GSD) has improved considerably. With ageing liver adenoma may de-velop which will bring about several complications-compres-sion, hemorrhage, or even transformation into carcinomas-and needs immediate intervention. In this paper, reviews for the mechanism and surgical intervention of GSD are presented.