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本文分析了15例2岁以下的婴儿糖原累积病(GSD)。肝肿大在肋下2.5～14cm.80%婴儿 SGPT 升高。1例出现黄疸者组织学检查同时伴有组织细胞增生症 X。12例中有9例空腹血糖降低,3例血糖正常者肝穿刺为 GSD。4/5例肾上腺素激发试验呈低平曲线,符合诊断。由于糖原沉着在肝脏使网状内皮系统功能减低,本组婴儿60%有反复呼吸道感染,53%有肠道感染。
This article analyzes 15 infants with glycogen storage disease (GSD) under 2 years of age. Hepatomegaly in the ribs 2.5 ~ 14cm.80% infant SGPT increased. One case of jaundice with histological examination accompanied by histiocytosis X. Fasting blood glucose was decreased in 9 of 12 patients and hepatic puncture in 3 patients with normal blood glucose was GSD. 4/5 cases of adrenaline provocation test showed a low level curve, in line with the diagnosis. As glycogen deposition in the liver so that the reticuloendothelial system function reduced, 60% of this group of infants have recurrent respiratory tract infections, 53% have intestinal infections.