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急性卟啉病的血红素疗法。

Hematin therapy for acute porphyria.

作者信息

Lamon J M, Frykholm B C, Hess R A, Tschudy D P

出版信息

Medicine (Baltimore). 1979 May;58(3):252-69. doi: 10.1097/00005792-197905000-00005.

Abstract
  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.
摘要
  1. 本文介绍了一项静脉注射血红素的治疗试验。11例急性间歇性卟啉病(AIP)患者和1例变异型卟啉病(VP)患者在接受传统治疗(高碳水化合物摄入)后病情未改善,接受了这种新药治疗。2. 尿δ-氨基-γ-酮戊酸(ALA)、卟胆原(PBG),以及在可能的情况下,尿卟啉和粪卟啉被用于监测治疗的化学反应。除了对急性卟啉症状(腹痛、背痛、肢体疼痛和感觉异常、虚弱、抑郁等)的主观评估外,还对高血压和心动过速等客观临床参数进行了跟踪。3. 在大约3mg/kg的剂量下,每位患者的尿ALA和PBG排泄量均有所减少。在与发作相关的情况下观察到的高血压和心动过速有所改善。此外,还经常观察到患者临床状况的主观改善。4. 血红素似乎是治疗急性发作型卟啉病的一种有前景的治疗药物。

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