Background

Catamenial precipitation of attacks of acute intermittent porphyria (AIP) is commonly treated with gonadotropin-releasing hormone analogues (GnRHas). However, this leads to various adverse effects that might necessitate “add-back” therapy with estrogen. The literature on the efficacy and safety of such therapy is scarce.

Case

A 15-year-old girl presented to us with recurrent catamenial attacks of AIP. GnRHa therapy led to near-complete amelioration of the episodes but her bone density worsened as an adverse effect. To circumvent this, low-dose estrogen was added to her regimen as an “add-back” therapy, which was later coupled with cyclical progesterone. She continues to do well using this regimen, with no new episodes.

Summary and Conclusion

GnRHa therapy with estrogen “add-back” is an attractive option for treating catamenial AIP episodes.

中文翻译：

---

用GnRH类似物以及雌激素和孕酮加回疗法管理的Catamenial急性间歇性卟啉症。

背景

通常用促性腺激素释放激素类似物（GnRHas）来治疗急性间歇性卟啉症（AIP）发作的突然沉淀。但是，这会导致各种不良反应，可能需要对雌激素进行“补充治疗”。关于这种疗法的有效性和安全性的文献很少。

案件

一名15岁女孩向我们展示了AIP反复月经期发作。GnRHa治疗导致发作几近完全缓解，但由于不利影响，她的骨密度恶化。为了避免这种情况，在她的治疗方案中加入了低剂量的雌激素，作为“补充疗法”，后来又联合使用了周期性孕激素。在没有新发作的情况下，她继续使用这种方案表现良好。

总结与结论

带有雌激素“加成”的GnRHa治疗是治疗月经期AIP发作的有吸引力的选择。