Howell R R, Stevenson R E, Ben-Menachem Y, Phyliky R L, Berry D H
JAMA. 1976 Sep 27;236(13):1481-4.
In older patients with glucose-6-phosphatase deficiency adenomatous nodules develop within the liver parenchyma. Investigation of eight such patients, age 3 to 28 years, using radioisotopic scans, has demonstrated areas of depressed isotope uptake in the liver in all except the one preteenaged child. Three patients were further studied with hepatic angiograms and liver biopsy specimens. A diffuse nodularity, more widespread than apparent on isotopic scans, was demonstrated on angiograms. Although initial histologic study in each case showed adenomatous tissue without evidence of neoplasm, the development of hepatocellular carcinoma in one of our patients and in others from the literature suggests that the nodules may be premalignant.
在患有葡萄糖-6-磷酸酶缺乏症的老年患者中，肝实质内会出现腺瘤样结节。对8名年龄在3至28岁的此类患者进行放射性同位素扫描研究，结果显示，除了一名十几岁前的儿童外，其他所有患者的肝脏中均存在同位素摄取降低的区域。对其中3名患者进一步进行了肝血管造影和肝活检标本研究。血管造影显示出弥漫性结节，其范围比同位素扫描所显示的更为广泛。尽管每例患者的初始组织学研究均显示为腺瘤样组织，未发现肿瘤证据，但我们的一名患者以及文献中其他患者发生肝细胞癌的情况表明，这些结节可能是癌前病变。
