The management of pregnancy in Gaucher disease.

Abstract

Gaucher disease (GD), characterized by deficient acid β-glucosidase activity, is the most common lysosomal storage disorder. The disease is progressive with manifestations that include anemia, thrombocytopenia, organomegaly and bone disease. Pregnancy has the potential to exacerbate these manifestations, compounding the risk of complications during pregnancy, delivery and postpartum. Enzyme replacement therapy with imiglucerase, before and during pregnancy, has demonstrated benefits in reducing the risk of spontaneous abortion and GD-related complications, especially bleeding during delivery and postpartum. European Medicines Agency guidelines now indicate that treatment-naive women should be advised to consider imiglucerase therapy before conception to obtain optimal health, and that imiglucerase treatment should be considered throughout pregnancy for women already receiving therapy. Many questions remain, however, on the indications for treatment and optimal management of women with GD. Based on a comprehensive review of outcomes in the management of pregnancy in GD, we present recommendations that aim to optimize patient care around pregnancy, delivery and the postpartum period, and alert attending physicians to the possible complications of pregnancy and delivery in GD.

• Authors: Sorina Granovsky-Grisaru, Nadia Belmatoug, Stephan vom Dahl, Eugen Mengel, Elizabeth Morris, Ari Zimran
• Journal: European journal of obstetrics, gynecology, and reproductive biology (Eur J Obstet Gynecol Reprod Biol) Vol. 156 Issue 1 Pg. 3-8 (May 2011) ISSN: 1872-7654 [Electronic] Ireland
• PMID: 21269752 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Chemical References

• Recombinant Proteins
• Glucosylceramidase
• imiglucerase

Topics

• Decision Trees
• Enzyme Replacement Therapy (adverse effects)
• Female
• Gaucher Disease (drug therapy, physiopathology, therapy)
• Glucosylceramidase (adverse effects, deficiency, therapeutic use)
• Humans
• Pregnancy
• Pregnancy Complications (drug therapy, physiopathology, therapy)
• Pregnancy Outcome
• Recombinant Proteins (adverse effects, therapeutic use)