{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T14:11:08Z","timestamp":1787839868299,"version":"build-2784847793"},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T00:00:00Z","timestamp":1664409600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>There are no recommended guidelines or clinical studies on safety of COVID\u201019 vaccines in patients with inborn errors of metabolism (IEMs). Here, we aimed to examine the relationship between COVID\u201019 vaccination and metabolic outcome in paediatric IEM patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with IEM between the ages of 12 and 18 were enrolled. Term metabolic decompensation was defined as acute disruption in metabolic homeostasis due to vaccination. Clinical and biochemical markers were compared between pre\u2010 and post\u2010vaccination periods.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Data from a total of 36 vaccination episodes in 18 patients were included. Thirteen patients had intoxication\u2010type metabolic disorders including organic acidemia (OA), urea cycle disorders (UCDs), maple syrup urine disease (MSUD) and phenylketonuria (PKU); 4 patients had energy metabolism disorders including fatty acid metabolism disorders and LIPIN 1 deficiency; and 1 patient had glycogen storage disorder (GSD) type 5. Seventeen patients received BNT162b2, and 1 received CoronaVac because of an underlying long QT syndrome. Fatty acid metabolism disorders, LIPIN 1 deficiency and GSD type 5 were included in the same group named \u2018metabolic myopathies\u2019. In two PKU patients, plasma phenylalanine level increased significantly within 24\u2009h following the second dose of vaccination. None of the OA, UCD, MSUD and metabolic myopathy patients experienced acute metabolic attack and had emergency department admission due to metabolic decompensation within 1\u2009month after vaccination.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>COVID\u201019 vaccines did not cause acute metabolic decompensation in a cohort of 18 children with IEM.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jpc.16231","type":"journal-article","created":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T12:26:16Z","timestamp":1664454376000},"page":"41-46","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Safety of<scp>COVID<\/scp>\u201019 vaccines in children with inborn errors of metabolism in terms of developing metabolic decompensation"],"prefix":"10.1111","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7159-4008","authenticated-orcid":false,"given":"Tanyel","family":"Zubarioglu","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sinem","family":"Oral\u2010Cebeci","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Emergency Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Duhan","family":"Hopurcuoglu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esma","family":"Uygur","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saffa","family":"Ahmadzada","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00f6zde","family":"Uzunyayla\u2010Inci","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ece","family":"Oge\u2010Enver","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sedanur","family":"Akca","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirsaid","family":"Aghalarov","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gizem","family":"Yilmaz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ertugrul","family":"Kiykim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cigdem","family":"Aktuglu\u2010Zeybek","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Nutrition and Metabolism Istanbul University\u2010Cerrahpasa, Cerrahpasa Medical Faculty Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,9,29]]},"reference":[{"key":"e_1_2_6_2_1","unstructured":"World Health Organization. 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