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To our knowledge, there are less than three patients with successful pregnancies and deliveries found in the literature with no specific consensus on management or recommendations for HHH syndrome. We reviewed the current literature regarding pregnancy outcomes, combine it with our experience managing a patient through two successful pregnancies and identify a new concern of fetal intrauterine growth restriction. From this, recommendations for pregnancy management are made, including a detailed protocol for clinicians to use for disease management at delivery and in the post\u2010partum period.<\/jats:p>","DOI":"10.1002\/jmd2.12025","type":"journal-article","created":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T03:49:09Z","timestamp":1694404149000},"page":"28-34","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Hyperornithinemia\u2010hyperammonemia\u2010homocitrullinuria syndrome in pregnancy: Considerations for management and review of the literature"],"prefix":"10.1002","volume":"46","author":[{"given":"Bernice","family":"Ho","sequence":"first","affiliation":[{"name":"Faculty of Arts and Science Queen's University  Kingston Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer","family":"MacKenzie","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics Kingston General Hospital  Kingston Ontario Canada"},{"name":"Department of Pediatrics McMaster University  Hamilton Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jagdeep","family":"Walia","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics Kingston General Hospital  Kingston Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Geraghty","sequence":"additional","affiliation":[{"name":"Division of Metabolics, Department of Pediatrics Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Graeme","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology Kingston General Hospital  Kingston Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julie","family":"Nedvidek","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics Kingston General Hospital  Kingston Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Guerin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics Kingston General Hospital  Kingston Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,3,14]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0242-9"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpedi.1969.02100030085009"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-7-32"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-197505000-00006"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cca.2012.03.015"},{"key":"e_1_2_7_7_1","first-page":"A14","article-title":"Maternal hyperornithinemia\u2010hyperammonemia\u2010homocitrullinuria (HHH) syndrome","volume":"45","author":"Wong P","year":"1989","journal-title":"Am J Hum Genet"},{"key":"e_1_2_7_8_1","unstructured":"Government of Canada(2005) Dietary reference intakes.https:\/\/www.canada.ca\/en\/health\u2010canada\/services\/food\u2010nutrition\/healthy\u2010eating\/dietary\u2010reference\u2010intakes\/tables.html. 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