Parker P, Burr I, Slonim A, Ghishan F K, Greene H
Gastroenterology. 1981 Sep;81(3):534-6.
Individuals with type Ia glycogen storage disease (glucose-6-phosphatase deficiency) frequently develop hepatic adenomas. Potential complications involving these adenomas include malignant transformation and hemorrhage. Five of 9 patients with this disease had evidence of hepatic filling defects on radionucleotide liver scan when first evaluated at our hospital. Dietary therapy aimed at preventing hypoglycemia was begun in 7 of the 9 patients. Prevention of hypoglycemia resulted in the correction of all of the metabolic abnormalities (lactic acidosis, hyperlipidemia, hyperuricemia, and growth retardation). Treatment also corrected the marked elevation in plasma glucagon concentrations. A disappearance of the hepatic lesions occurred in 2 of the treated patients, and a marked reduction in size of the adenoma occurred in the third patient. The hepatic filling defects remained present in the two untreated patients. None of the affected patients receiving dietary therapy have developed hepatic adenomas. One of these patients is now 22 yr old and has received dietary therapy for 7 yr. Early dietary therapy seems to be effective in preventing development of adenomas as well as inducing their resolution.
Ia型糖原贮积病（葡萄糖-6-磷酸酶缺乏症）患者常发生肝腺瘤。这些腺瘤的潜在并发症包括恶变和出血。在我院首次评估时，9例该疾病患者中有5例在放射性核素肝脏扫描中有肝脏充盈缺损的证据。9例患者中有7例开始了旨在预防低血糖的饮食治疗。预防低血糖导致所有代谢异常（乳酸酸中毒、高脂血症、高尿酸血症和生长发育迟缓）得到纠正。治疗还纠正了血浆胰高血糖素浓度的显著升高。2例接受治疗的患者肝脏病变消失，第3例患者的腺瘤大小显著缩小。2例未治疗的患者肝脏充盈缺损仍然存在。接受饮食治疗的所有患病患者均未发生肝腺瘤。其中1例患者现22岁，已接受饮食治疗7年。早期饮食治疗似乎对预防腺瘤的发生以及促使其消退有效。
