Document Detail

The management of pregnancy in Gaucher disease.
MedLine Citation:
PMID:  21269752     Owner:  NLM     Status:  Publisher    
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.
Sorina Granovsky-Grisaru; Nadia Belmatoug; Stephan Vom Dahl; Eugen Mengel; Elizabeth Morris; Ari Zimran
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-24
Journal Detail:
Title:  European journal of obstetrics, gynecology, and reproductive biology     Volume:  -     ISSN:  1872-7654     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-1-28     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375672     Medline TA:  Eur J Obstet Gynecol Reprod Biol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.
Dept. of Obstetrics and Gynecology, Shaare Zedek Medical Center, Hebrew University, Jerusalem 91031, Israel; Gaucher Clinic, Shaare Zedek Medical Center, Hebrew University, Jerusalem, Israel.
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