1 临床特点①幼儿,女,病程长,反复发作,进行性加重.②无明显诱因,发作时以四肢和眼部肌肉抽搐为主要表现,发作前及发作间隙伴有哭闹、烦躁、恶心、呕吐等症状.③肝脾明显肿大,肝功能正常.④血糖极度减少,二氧化碳结合力明显降低,血色素偏低:尿pH值降低,蛋白尿,原酮体明显升高.⑤输入葡萄糖及纠正酸碱失衡后好转 1 clinical features ① children, women, longer duration, recurrent, progressive increase.② no obvious incentive, seizures to limbs and eye muscle twitch as the main performance before and during the attack episodes accompanied by crying, irritability, nausea, Vomiting and other symptoms.③ obvious enlargement of liver and spleen, liver function is normal.④Glucose reduction, carbon dioxide binding decreased significantly, low hemoglobin: urinary pH decreased, proteinuria, the original ketone body was significantly increased.⑤Glucose and correction After acid-base imbalance improved