Catamenial Acute Intermittent Porphyria Managed with GnRH Analogues and Estrogen and Progesterone Add-back Therapy.
Acute intermittent porphyria
Catamenial precipitation
Estrogen ‘addback’ therapy
GnRH analogs
Journal
Journal of pediatric and adolescent gynecology
ISSN: 1873-4332
Titre abrégé: J Pediatr Adolesc Gynecol
Pays: United States
ID NLM: 9610774
Informations de publication
Date de publication:
Aug 2020
Aug 2020
Historique:
received:
01
12
2019
revised:
19
02
2020
accepted:
22
02
2020
pubmed:
3
3
2020
medline:
5
11
2020
entrez:
2
3
2020
Statut:
ppublish
Résumé
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. 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. GnRHa therapy with estrogen "add-back" is an attractive option for treating catamenial AIP episodes.
Sections du résumé
BACKGROUND
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
METHODS
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
CONCLUSIONS
GnRHa therapy with estrogen "add-back" is an attractive option for treating catamenial AIP episodes.
Identifiants
pubmed: 32113877
pii: S1083-3188(20)30162-5
doi: 10.1016/j.jpag.2020.02.009
pii:
doi:
Substances chimiques
Estrogens
0
Gonadotropin-Releasing Hormone
33515-09-2
Progesterone
4G7DS2Q64Y
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
432-434Informations de copyright
Copyright © 2020 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.