OBJECTIVE: The study was undertaken to investigate the outcome of pregnancies in Gaucher disease, particularly in enzyme-treated women. STUDY DESIGN: A retrospective study was performed of pregnant women evaluated at a referral clinic. RESULTS: There were 43 (17 treated, 26 untreated) women with 66 pregnancies (23 treated, 43 untreated). The live birth rate was 78.3% among treated, 86.0% among untreated. One treated woman had three spontaneous abortions; 3 untreated women had one each. Four pregnancies in each group had postpartum bleeding, 7 requiring transfusions. Postpartum infections were prevalent among treated. Cesarean sections were generally for historic reasons. There was no exacerbation of Gaucher disease, except one bone crisis. CONCLUSION: Most untreated women with milder disease enjoyed an uncomplicated course. Enzyme-treated patients (ie, with more severe disease) had more bleeding and infections post partum, but few had spontaneous abortions. Hematologic consultation is recommended. A review of world experience with pregnant patients with Gaucher disease is included.
OBJECTIVE: The study was undertaken to investigate the outcome of pregnancies in Gaucher disease, particularly in enzyme-treated women. STUDY DESIGN: A retrospective study was performed of pregnant women evaluated at a referral clinic. RESULTS: There were 43 (17 treated, 26 untreated) women with 66 pregnancies (23 treated, 43 untreated). The live birth rate was 78.3% among treated, 86.0% among untreated. One treated woman had three spontaneous abortions; 3 untreated women had one each. Four pregnancies in each group had postpartum bleeding, 7 requiring transfusions. Postpartum infections were prevalent among treated. Cesarean sections were generally for historic reasons. There was no exacerbation of Gaucher disease, except one bone crisis. CONCLUSION: Most untreated women with milder disease enjoyed an uncomplicated course. Enzyme-treated patients (ie, with more severe disease) had more bleeding and infections post partum, but few had spontaneous abortions. Hematologic consultation is recommended. A review of world experience with pregnant patients with Gaucher disease is included.
Authors: T M Cox; J M F G Aerts; N Belmatoug; M D Cappellini; S vom Dahl; J Goldblatt; G A Grabowski; C E M Hollak; P Hwu; M Maas; A M Martins; P K Mistry; G M Pastores; A Tylki-Szymanska; J Yee; N Weinreb Journal: J Inherit Metab Dis Date: 2008-05-23 Impact factor: 4.982
Authors: P Grigorescu Sido; C Drugan; V Cret; C Al-Kzouz; C Denes; C Coldea; A Zimmermann Journal: J Inherit Metab Dis Date: 2007-08-20 Impact factor: 4.982