{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T11:51:47Z","timestamp":1785239507451,"version":"3.55.0"},"reference-count":107,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2023,4,4]],"date-time":"2023-04-04T00:00:00Z","timestamp":1680566400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Metabolites"],"abstract":"<jats:p>Organic acidurias are a heterogeneous group of rare inherited metabolic disorders (IMDs) caused by a deficiency of an enzyme or a transport protein involved in the intermediary metabolic pathways. These enzymatic defects lead to an accumulation of organic acids in different tissues and their subsequent excretion in urine. Organic acidurias include maple syrup urine disease, propionic aciduria, methylmalonic aciduria, isovaleric aciduria, and glutaric aciduria type 1. Clinical features vary between different organic acid disorders and may present with severe complications. An increasing number of women with rare IMDs are reporting successful pregnancy outcomes. Normal pregnancy causes profound anatomical, biochemical and physiological changes. Significant changes in metabolism and nutritional requirements take place during different stages of pregnancy in IMDs. Foetal demands increase with the progression of pregnancy, representing a challenging biological stressor in patients with organic acidurias as well as catabolic states post-delivery. In this work, we present an overview of metabolic considerations for pregnancy in patients with organic acidurias.<\/jats:p>","DOI":"10.3390\/metabo13040518","type":"journal-article","created":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T02:11:41Z","timestamp":1680660701000},"page":"518","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Organic Aciduria Disorders in Pregnancy: An Overview of Metabolic Considerations"],"prefix":"10.3390","volume":"13","author":[{"given":"Loai A.","family":"Shakerdi","sequence":"first","affiliation":[{"name":"National Centre for Inherited Metabolic Disorders, Mater Misericordiae University Hospital, D07 R2WY Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Gillman","sequence":"additional","affiliation":[{"name":"National Centre for Inherited Metabolic Disorders, Mater Misericordiae University Hospital, D07 R2WY Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Corcoran","sequence":"additional","affiliation":[{"name":"National Centre for Inherited Metabolic Disorders, Mater Misericordiae University Hospital, D07 R2WY Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny","family":"McNulty","sequence":"additional","affiliation":[{"name":"National Centre for Inherited Metabolic Disorders (NCIMD), Childrens Health Ireland at Temple Street, Temple Street, D01 XD99 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6226-1615","authenticated-orcid":false,"given":"Eileen P.","family":"Treacy","sequence":"additional","affiliation":[{"name":"The Irish National Rare Disease Office, Mater Misericordiae University Hospital, D07 R2WY Dublin, Ireland"},{"name":"Discipline of Medicine, School of Medicine, Trinity College Dublin, D02 PN40 Dublin, Ireland"},{"name":"University College Dublin (UCD) School of Medicine, Belfield, D04 V1W8 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,4,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1002\/jimd.12348","article-title":"An international classification of inherited metabolic disorders (ICIMD)","volume":"44","author":"Ferreira","year":"2021","journal-title":"J. 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