正铁血红素治疗急性间歇性血卟啉症  

Treatment of acute intermittent porphyria with panhematin

作　　者：徐克成[1] 叶平[2] 

机构地区：[1]深圳养和医疗中心,深圳南山518052 [2]深圳市罗湖人民医院,深圳罗湖518001

出　　处：《胃肠病学和肝病学杂志》1999年第3期216-217,219,共3页Chinese Journal of Gastroenterology and Hepatology

摘　　要：目的　探讨正铁血红素对急性间歇性血卟啉症（ＡＩＰ） 的治疗作用。方法　２ 例ＡＩＰ 均具有典型症状，尿Ｗａｔｓｏｎ － Ｓｃｈｗａｒｔｚ 试验阳性，葡萄糖疗法对其无明显效果，给予正铁血红素剂Ｐａｎｈｅｍａｔｉｎ 治疗，２００ｍｇ／ 天，静脉输注，连用５天。结果　腹绞痛在用药２ － ３ 天后均迅速缓解。例１ 抽搐症状明显减轻，例１ 在６ 个月后症状复发，再次用药仍有效，随访２ 年未复发；例２ 接受随访１ 年，未见复发。未见明显副作用。结论　正铁血红素是治疗急性间歇性血卟啉症的有效药物。Aim To evaluate the therapeutic efficacy of Panhematin for acute intermittent porphyria(AIP).Methods Two patients with AIP,who had classical symptoms and positive Watson Schwartz test of urine,were treated Panhematin intravenously in dose of 200mg daily for 5 days.Results The abdominal colic in 2 cases was significantly relieved at the third and second day respectively after administration of Panhematin.Seizure attack in patient 1 also obviously declined.Patient 1 had recurrent attacks 6 months later,and was well responded to second course of Panhematin.During the further following up period of 1 2 years,two cases did not suffer from recurrent attacks of the disease.On the above dose of Panhematin,no side effects occurred.Conclusion Panhematin is effective for improvement of porphyric attacks in AIP patients.

关 键 词：急性 间歇性 血卟啉症 正铁血红素 治疗 

分 类 号：R589.805[医药卫生—内分泌]