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急性间歇型血卟啉病是一种罕见的遗传性疾病,为孟德尔显性基因传递。其特征为潜伏期后周期性急性发作。年轻患者首次发作可能出现于月经初期或妊娠期。本文报告2例该症患者的妊娠经过。例1,22岁。1982年因严重腹部绞孕3月痛而行人工流产,术后发生四肢麻痹和呼吸肌麻痹,并需使用呼吸机。当时诊断为急性间歇型血卟啉病。1983年该患者妊娠11周时,医生告知妊娠将有危险,建议食用高糖类食物、饮用大量液体、戒酒、服任何药物前要征得医生同意。患者遵嘱。妊娠期正常,于孕38周时正常自然分娩一健康婴儿。为预防急性发作,产后静脉输注10%果糖(每天1,000ml,连续10天)。有关血卟啉检查阴性。哺乳正常。例2,34岁。妊娠12周对反复发生急性腹部麻痹和昏迷,诊断为急性间歇型血卟啉病。妊娠期间,医生提出劝告如例1。至孕39周无异常。分娩前输
Acute intermittent hematoporphyria is a rare genetic disorder that is transmitted by Mendelian dominant genes. It is characterized by a periodic acute episode of latency. The first attack of young patients may occur in early menstruation or during pregnancy. This article reports 2 cases of the disease after pregnancy. Example 1,22 years old. In 1982 due to severe abdominal cramps March pain and artificial abortion, postoperative limb paralysis and respiratory muscle paralysis, and the need to use a ventilator. At that time diagnosed as acute intermittent hematoporphyrin disease. In 1983, when the patient was pregnant at 11 weeks of gestation, the doctor advised that the pregnancy would be dangerous. It is recommended to consume high-carbohydrate foods, drink plenty of fluids, abstain from alcohol, and seek medical approval before taking any medicine. Patient compliance. Normal pregnancy, at 38 weeks of pregnancy normal natural childbirth a healthy baby. To prevent an acute attack, a postpartum vein is infused with 10% fructose (1,000 ml daily for 10 consecutive days). Hematoporphyrin negative examination. Breastfeeding normal. Example 2,34 years old. Twelve weeks of pregnancy, recurrent acute abdominal paralysis and coma were diagnosed as acute intermittent hematoporphyrinosis. During pregnancy, the doctor made an advice as in Example 1. 39 weeks pregnant no abnormalities. Lose before delivery