Department of Clinical Science, Obstetrics and Gynecology, Umeå University, Umeå, Sweden.
Acta Obstet Gynecol Scand. 2010;89(1):95-100. doi: 10.3109/00016340903390729.
To describe the benefits and adverse effects of gonadotropin-releasing hormone (GnRH) agonist treatment for prevention of recurrent menstrual attacks in women with acute intermittent porphyria and variegate porphyria. To describe concomitant add-back therapies with estradiol and progesterone and describe their benefits and adverse effects.
A retrospective follow-up with questionnaires, interviews and medical records.
Out-patient care at the Umeå University Hospital in Sweden.
Sixteen Caucasian women with DNA-diagnosed porphyria and menstrual-cycle-related porphyria attacks were treated with GnRH agonists during 1984-2000. Fourteen women participated. The mean age when treatment started was 33 years (17-48 years). The duration of treatment varied between 5 months and 9 years.
GnRH agonists were administered by the intranasal route or by injections. To reduce menopausal symptoms, add-back therapy with low doses of estradiol was administered, and for endometrial protection progesterone was usually administered.
Treatment effects and adverse events as detected in questionnaires, interviews and medical records.
Eleven women reported benefits from GnRH agonist treatment with less intense and/or less frequent porphyria attacks, and in four of them attacks almost disappeared. Two women reported no change. One woman had only temporary improvement. Porphyria attacks were triggered by solely estradiol add-back in two women and in five of nine women when progesterone was given.
GnRH agonist treatment can ameliorate menstrual-cycle-related attacks of porphyria. Dose findings for GnRH agonists and add-back regimes especially for progesterone are intricate.
描述促性腺激素释放激素（GnRH）激动剂治疗对预防急性间歇性卟啉症和变异性卟啉症女性月经性发作的益处和不良反应。描述雌二醇和孕激素的伴随添加治疗，并描述其益处和不良反应。
回顾性随访，使用问卷、访谈和病历。
瑞典于默奥大学医院的门诊治疗。
16 名白人女性，经 DNA 诊断患有卟啉症和月经周期相关卟啉症发作，在 1984-2000 年期间接受 GnRH 激动剂治疗。14 名女性参与。开始治疗时的平均年龄为 33 岁（17-48 岁）。治疗持续时间从 5 个月到 9 年不等。
通过鼻内途径或注射给予 GnRH 激动剂。为了减少绝经症状，给予低剂量雌二醇的添加治疗，并且通常给予孕激素以保护子宫内膜。
通过问卷、访谈和病历检测到的治疗效果和不良反应。
11 名女性报告 GnRH 激动剂治疗有获益，表现为卟啉症发作的强度和/或频率降低，其中 4 名女性的发作几乎消失。2 名女性报告无变化。1 名女性仅有暂时改善。2 名女性仅因雌二醇添加治疗而触发卟啉症发作，9 名女性中有 5 名在给予孕激素时发作。
GnRH 激动剂治疗可以改善月经周期相关的卟啉症发作。对于 GnRH 激动剂和添加治疗方案，特别是对于孕激素，剂量发现比较复杂。
