Limmer J, Fleig W E, Leupold D, Bittner R, Ditschuneit H, Beger H G
Department of Surgery, University of Ulm, Federal Republic of Germany.
Hepatology. 1988 May-Jun;8(3):531-7. doi: 10.1002/hep.1840080317.
Patients suffering from Type I glycogen storage disease frequently develop hepatic tumors. Some of these were classified as carcinoma, with the majority of tumors representing benign adenomata. However, no evidence exists of malignant transformation of adenomata in these patients. Here, we describe the occurrence of a hepatocellular carcinoma in the adenomata-bearing liver of the elder of two sisters suffering from Type I glycogen storage disease at the age of 20 years, 6 years after the diagnosis had been made. Surprisingly, alpha-fetoprotein levels were normal throughout the entire course of this patient, whereas the younger sister had elevated levels despite the absence of malignant lesions. Thus, the clinical significance of alpha-fetoprotein remains unclear in both cases. Nocturnal feeding, although performed continuously over the 6 years after the diagnosis, had obviously failed to prevent the development of hepatic tumors in both patients.
患有I型糖原贮积病的患者经常会发生肝肿瘤。其中一些被归类为癌，大多数肿瘤为良性腺瘤。然而，这些患者中没有腺瘤恶变的证据。在此，我们描述了一名患有I型糖原贮积病的两姐妹中的姐姐，在确诊6年后，20岁时在有腺瘤的肝脏中发生了肝细胞癌。令人惊讶的是，该患者在整个病程中α-甲胎蛋白水平均正常，而妹妹尽管没有恶性病变但α-甲胎蛋白水平却升高。因此，在这两个病例中α-甲胎蛋白的临床意义仍不清楚。夜间喂食，尽管在诊断后的6年中持续进行，但显然未能阻止两名患者肝肿瘤的发生。
