{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T02:54:05Z","timestamp":1782442445164,"version":"3.54.5"},"reference-count":9,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2016,7,7]],"date-time":"2016-07-07T00:00:00Z","timestamp":1467849600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Obstet and Gynaecol"],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Glycogen storage disease type II (GSD II or Pompe disease; OMIM; 232\u2009300) is a rare autosomal recessive lysosomal storage disorder resulting from deficiency of \u03b1\u2010glucosidase and accumulation of glycogen in muscle. Clinical symptoms include weakness of skeletal and respiratory muscles and, in infants, cardiomyopathy. Patients with GSD II receive infusions of recombinant \u03b1\u2010glucosidase (enzyme replacement therapy; ERT), which slow the progression of the disease. ERT is given to male and female patients of all ages but as yet little is documented on the effects of continuing ERT during pregnancy. The aim of this case series was therefore to ascertain the pregnancy outcomes of women with GSD II on ERT and to describe adverse events associated with pregnancy, delivery and therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The medical records of eight women attending the Royal Free Hospital Lysosomal Storage Disorders Unit were reviewed. Four of the eight women had seven pregnancies over a period of 8 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In this series GSD II was associated with interventional deliveries but normal neonates. Cessation of ERT in early pregnancy resulted in deterioration of maternal symptoms and emergence of allergic reactions on restarting ERT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Individualized care plans are required to ensure the best neonatal and maternal outcomes. Consideration should be given to the potential benefits to mother and fetus of continuing ERT during pregnancy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jog.13055","type":"journal-article","created":{"date-parts":[[2016,7,7]],"date-time":"2016-07-07T07:46:40Z","timestamp":1467877600000},"page":"1263-1271","source":"Crossref","is-referenced-by-count":15,"title":["Pregnancy and associated events in women receiving enzyme replacement therapy for late\u2010onset glycogen storage disease type II (Pompe disease)"],"prefix":"10.1111","volume":"42","author":[{"given":"Philippa J.","family":"Rohman","sequence":"first","affiliation":[{"name":"Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free London NHS Foundation Trust University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elaine","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynaecology Royal Free London NHS Foundation Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Richfield","sequence":"additional","affiliation":[{"name":"Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free London NHS Foundation Trust University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uma","family":"Ramaswami","sequence":"additional","affiliation":[{"name":"Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free London NHS Foundation Trust University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derralynn A.","family":"Hughes","sequence":"additional","affiliation":[{"name":"Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free London NHS Foundation Trust University College London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,7,7]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909859"},{"key":"e_1_2_7_3_1","unstructured":"DeeganPB CoxTM WaldekSet al.Guidelines for the investigation and management of late onset acid maltase deficiency (type II glycogen storage disorder\/Pompe disease) version3.2007. Accessed 31 May 2016. Available from URL:http:\/\/www.specializedservices.nhs.uk\/library\/23\/Guidelines-for-Late-Onset-Pompe-Disease.pdf."},{"key":"e_1_2_7_4_1","unstructured":"Full MYOZYME\u00ae (alglucosidase alfa) Injectable for intravenous infusion prescribing information. Initial Approval: 2006. Accessed 31 May 2016. Available from URL:www.ema.eu\/docs\/en_GB\/document_library\/EPAR_-_Product_Information\/human\/000636\/wc500032125.pdf."},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)02533-2"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9384-7"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijoa.2007.11.001"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/pmj.76.901.683"},{"key":"e_1_2_7_9_1","first-page":"8","article-title":"Clinical features and predictors for disease natural progression in adults with Pompe disease: A nationwide prospective observational study","volume":"7","author":"Beek NAME","year":"2012","journal-title":"Orphanet J Rare Dis"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-0252-1"}],"container-title":["Journal of Obstetrics and Gynaecology Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjog.13055","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjog.13055","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jog.13055","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T12:42:32Z","timestamp":1696509752000},"score":1,"resource":{"primary":{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/10.1111\/jog.13055"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,7,7]]},"references-count":9,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2016,10]]}},"alternative-id":["10.1111\/jog.13055"],"URL":"https:\/\/doi.org\/10.1111\/jog.13055","archive":["Portico"],"relation":{},"ISSN":["1341-8076","1447-0756"],"issn-type":[{"value":"1341-8076","type":"print"},{"value":"1447-0756","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,7,7]]}}}