Rossi R, Pekolj J, Glasinovic J C, Lee A
Department of Surgery, Lahey Clinic, Burlington Mass, USA.
Rev Med Chil. 1996 Feb;124(2):225-30.
In a family composed by eight brothers, five had a type I glycogen storage disease and three presented with liver tumors complicated with hemorrhage or malignant transformation, during the follow up. The periodic ultrasonographic control allowed the early diagnosis of these neoplasms. We describe the clinical picture and treatment of two patients. Metabolic alterations were corrected during the preoperative period with parenteral nutrition. Resection was successful in both cases. It is concluded that a close follow up and early treatment of these lesions is effective and avoids complications. In patients with multiple lesions and severe metabolic alterations, liver transplantation is the treatment of choice.
在一个由八个兄弟组成的家庭中，五人患有I型糖原贮积病，三人在随访期间出现肝脏肿瘤并伴有出血或恶变。定期超声检查有助于这些肿瘤的早期诊断。我们描述了两名患者的临床表现和治疗情况。术前通过肠外营养纠正代谢紊乱。两例手术切除均成功。结论是对这些病变进行密切随访和早期治疗是有效的，可避免并发症。对于有多处病变和严重代谢紊乱的患者，肝移植是首选治疗方法。
