Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease

Pregnancy and delivery are challenging in women affected by Pompe disease with respiratory involvement. We describe a 28-year-old woman, who continued to receive enzyme replacement therapy during pregnancy and had an uneventful vaginal birth. Before pregnancy the patient's vital capacity was 52...

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Main Authors: Perniconi, B, Vauthier-Brouzes, D, Morélot-Panzini, C, Dommergues, M, Nizard, J, Taouagh, N, Hogrel, J-Y, Canal, A, Servais, L, Laforêt, P
Format: Journal article
Language:English
Published: Elsevier 2016
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author Perniconi, B
Vauthier-Brouzes, D
Morélot-Panzini, C
Dommergues, M
Nizard, J
Taouagh, N
Hogrel, J-Y
Canal, A
Servais, L
Laforêt, P
author_facet Perniconi, B
Vauthier-Brouzes, D
Morélot-Panzini, C
Dommergues, M
Nizard, J
Taouagh, N
Hogrel, J-Y
Canal, A
Servais, L
Laforêt, P
author_sort Perniconi, B
collection OXFORD
description Pregnancy and delivery are challenging in women affected by Pompe disease with respiratory involvement. We describe a 28-year-old woman, who continued to receive enzyme replacement therapy during pregnancy and had an uneventful vaginal birth. Before pregnancy the patient's vital capacity was 52% in sitting position and 51% in supine position. At 32 weeks gestation her vital capacity in sitting position was 46% and 35% in supine position. Nocturnal non-invasive mechanical ventilation was introduced at this time. Labor was induced at 34 weeks following premature rupture of membranes, under epidural anesthesia. A 2590 g healthy baby was delivered by vacuum extraction. Assisted ventilation was continued throughout labor and post-partum. This observation suggests a successful pregnancy and a normal vaginal delivery can be achieved in patients with symptomatic Pompe Disease, provided multidisciplinary care is offered.
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spelling oxford-uuid:35f14932-9d85-4a7e-8a5d-ddfa36ceea9e2022-03-26T13:34:57ZMultidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe diseaseJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:35f14932-9d85-4a7e-8a5d-ddfa36ceea9eEnglishSymplectic ElementsElsevier2016Perniconi, BVauthier-Brouzes, DMorélot-Panzini, CDommergues, MNizard, JTaouagh, NHogrel, J-YCanal, AServais, LLaforêt, PPregnancy and delivery are challenging in women affected by Pompe disease with respiratory involvement. We describe a 28-year-old woman, who continued to receive enzyme replacement therapy during pregnancy and had an uneventful vaginal birth. Before pregnancy the patient's vital capacity was 52% in sitting position and 51% in supine position. At 32 weeks gestation her vital capacity in sitting position was 46% and 35% in supine position. Nocturnal non-invasive mechanical ventilation was introduced at this time. Labor was induced at 34 weeks following premature rupture of membranes, under epidural anesthesia. A 2590 g healthy baby was delivered by vacuum extraction. Assisted ventilation was continued throughout labor and post-partum. This observation suggests a successful pregnancy and a normal vaginal delivery can be achieved in patients with symptomatic Pompe Disease, provided multidisciplinary care is offered.
spellingShingle Perniconi, B
Vauthier-Brouzes, D
Morélot-Panzini, C
Dommergues, M
Nizard, J
Taouagh, N
Hogrel, J-Y
Canal, A
Servais, L
Laforêt, P
Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease
title Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease
title_full Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease
title_fullStr Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease
title_full_unstemmed Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease
title_short Multidisciplinary care allowing uneventful vaginal delivery in a woman with Pompe disease
title_sort multidisciplinary care allowing uneventful vaginal delivery in a woman with pompe disease
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