Yamamoto Tamao, Suzuki Yoshikatsu, Kaneko Saori, Hattori Yukio, Obayashi Shintaro, Suzumori Nobuhiro, Sugiura Mayumi
Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan.
J Obstet Gynaecol Res. 2010 Oct;36(5):1125-9. doi: 10.1111/j.1447-0756.2010.01287.x.
Glycogen storage disease type Ia (GSD Ia) leads to disturbed glycogenolysis and gluconeogenesis due to a deficiency in the enzyme glucose-6-phosphatase. A patient with GSD Ia showed hypoglycemia and proteinuria without dietary management since early pregnancy. The patient's condition was complicated by hypertension with increase in proteinuria at 22 weeks of gestation. In spite of administration of antihypertensive drugs and dietary management, the disease became more severe with deterioration in the fetal status and inhibition of fetal growth. Thus, a cesarean section was performed at 26 weeks of gestation. The delivered male infant weighing 412 g died at 2 days after birth. The patient's blood pressure had normalized within 3 months after delivery, while proteinuria persisted.
Ia型糖原贮积病（GSD Ia）由于葡萄糖-6-磷酸酶缺乏导致糖原分解和糖异生紊乱。一名患有GSD Ia的患者自妊娠早期以来未进行饮食管理，出现低血糖和蛋白尿。该患者在妊娠22周时并发高血压且蛋白尿增加。尽管使用了降压药物并进行了饮食管理，但病情仍加重，胎儿状况恶化，胎儿生长受限。因此，在妊娠26周时进行了剖宫产。分娩出的男婴体重412 g，出生后2天死亡。患者产后3个月内血压恢复正常，但蛋白尿持续存在。
