{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T14:40:49Z","timestamp":1753886449716,"version":"3.40.5"},"reference-count":8,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,3,22]],"date-time":"2022-03-22T00:00:00Z","timestamp":1647907200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JIMD Reports"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Glycogen storage disease type IIIa (GSD\u2010IIIa) is an autosomal recessive disorder that impairs glycogenolysis, producing ketotic hypoglycaemia, hepatomegaly, cardiac and skeletal myopathy. During pregnancy, increased metabolic demand requires careful management. There are few case reports about pregnancy in GSD\u2010IIIa, however none detail management during caesarean section. This case describes a 25\u2010year\u2010old women with GSD\u2010IIIa diagnosed at 5\u00a0months of age. She had modest metabolic control with complications including hepatomegaly, mild skeletal myopathy and poor enteral function requiring multiple operative interventions. She had a planned pregnancy managed by a multidisciplinary team, which included a metabolic geneticist, maternal\u2013fetal medicine specialist and metabolic dietitian. Nocturnal cornstarch was provided to meet basal carbohydrate requirements and a high protein diet with regular carbohydrates was consumed throughout the day. The woman remained well during the antenatal period and had an induction of labour at 38\u2009weeks gestation. She had an emergency caesarean section in early labour due to an abnormal cardiotocography (CTG). The intraoperative and postoperative period were uncomplicated. A live baby boy was born in good condition, weighing 2440\u2009g with APGARs of 9 and 9 at 1 and 5\u00a0min. She was managed in labour with glucose 10% IV at 3.5\u00a0mg\/kg\/min, hourly blood sugar level (BSL) monitoring and early epidural anaesthetic. The aim of the first 24\u2010h post\u2010partum was prevention of hypoglycaemia, which required strict management with dextrose 10% IV at 3.5\u00a0mg\/kg\/min, oral carbohydrate supplementation and BSL monitoring. This case highlights the complexity of GSD\u2010IIIa as well as provides a proposed plan for management during pregnancy.<\/jats:p>","DOI":"10.1002\/jmd2.12282","type":"journal-article","created":{"date-parts":[[2022,3,23]],"date-time":"2022-03-23T05:31:13Z","timestamp":1648013473000},"page":"216-220","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Glycogen storage disease type <scp>IIIa<\/scp> in pregnant women: A guide to management"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9076-7269","authenticated-orcid":false,"given":"Demi","family":"Beneru","sequence":"first","affiliation":[{"name":"Maternal Fetal Medicine Unit, Department of Obstetrics and Gynaecology Westmead Hospital  Sydney Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9700-7898","authenticated-orcid":false,"given":"Michel C.","family":"Tchan","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine Westmead Hospital  Sydney Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kate","family":"Billmore","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine Westmead Hospital  Sydney Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roshini","family":"Nayyar","sequence":"additional","affiliation":[{"name":"Maternal Fetal Medicine Unit, Department of Obstetrics and Gynaecology Westmead Hospital  Sydney Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,3,22]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2017.08.010"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddz133"},{"volume-title":"Gene Reviews","year":"1993","author":"Schreuder AB","key":"e_1_2_8_4_1"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-016-9932-2"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9384-7"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijoa.2011.09.004"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1479-828x.2006.00549.x"},{"key":"e_1_2_8_9_1","unstructured":"Policy H.A.S. Maternity\u2010Fetal Heart Rate Monitoring N. Health Editor.2018: New South Wales."}],"container-title":["JIMD Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jmd2.12282","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jmd2.12282","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jmd2.12282","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,23]],"date-time":"2023-08-23T22:02:21Z","timestamp":1692828141000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jmd2.12282"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,22]]},"references-count":8,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,5]]}},"alternative-id":["10.1002\/jmd2.12282"],"URL":"https:\/\/doi.org\/10.1002\/jmd2.12282","archive":["Portico"],"relation":{},"ISSN":["2192-8312","2192-8312"],"issn-type":[{"type":"print","value":"2192-8312"},{"type":"electronic","value":"2192-8312"}],"subject":[],"published":{"date-parts":[[2022,3,22]]},"assertion":[{"value":"2021-10-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-03-09","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-03-22","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}