Hematin therapy for acute porphyria.
Hematin therapy for acute porphyria.
复制标题
血红素治疗急性卟啉症。
作者：
J. Lamon;B. Frykholm;R. Hess;D. Tschudy
1.介绍了一种静脉注射血红素的治疗试验。11例AIP和1例VP患者在常规治疗(高碳水化合物摄入)后无改善，接受了该新药物治疗。2.用尿ALA、PBG、尿卟啉和钴卟啉监测治疗前后的化学反应。目的观察高血压和心动过速的临床参数，以及对急性腹痛、背痛、四肢疼痛和感觉异常、虚弱、抑郁等急性门脉症状的主观评估。3.在剂量约为3 mg/kg时，每例患者的尿ALA和PBG排泄量均有减少。高血压和心动过速在与发作相关的情况下得到改善。此外，患者临床状态的主观改善也经常被观察到。4.血红素似乎是一种很有前途的治疗急性发作型卟啉病的药物。
1. A therapeutic trial of intravenous hematin is presented. Eleven cases of AIP and one of VP who did not improve with conventional treatment (high carbohydrate intake) received this new agent. 2. Urinary ALA, PBG and, when possible, uroporphyrin and coproporphyrin were used to monitor the chemical response to the treatment. Objective clinical parameters of hypertension and tachycardia were followed when present in addition to subjective estimates of acute porphyric symptomatology (abdominal pain, backache, extremity pain and paresthesias, weakness, depression, etc.). 3. At a dosage of approximately 3 mg/kg, diminution of urinary ALA and PBG excretion was achieved in every patients. Hypertension and tachycardia improved in those instances where they were observed in association with the attack. Also, subjective improvements in the clinical status of the patients were observed frequently. 4. Hematin appears to be a promising therapeutic agent for the treatment of acute attack forms of porphyria.