Department of Pediatrics, Division of Nutrition and Metabolism, Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Istanbul, Turkey.
Department of Pediatrics, Division of Pediatric Emergency, Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Istanbul, Turkey.
J Paediatr Child Health. 2023 Jan;59(1):41-46. doi: 10.1111/jpc.16231. Epub 2022 Sep 29.
There are no recommended guidelines or clinical studies on safety of COVID-19 vaccines in patients with inborn errors of metabolism (IEMs). Here, we aimed to examine the relationship between COVID-19 vaccination and metabolic outcome in paediatric IEM patients.
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- and post-vaccination periods.
Data from a total of 36 vaccination episodes in 18 patients were included. Thirteen patients had intoxication-type 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 'metabolic myopathies'. In two PKU patients, plasma phenylalanine level increased significantly within 24 h 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 month after vaccination.
COVID-19 vaccines did not cause acute metabolic decompensation in a cohort of 18 children with IEM.
目前尚无关于 COVID-19 疫苗在先天性代谢缺陷(IEM)患者中安全性的推荐指南或临床研究。在此,我们旨在研究 COVID-19 疫苗接种与儿科 IEM 患者代谢结局之间的关系。
纳入年龄在 12 至 18 岁之间的 IEM 患者。定义代谢性失代偿为疫苗接种引起的代谢稳态急性破坏。比较疫苗接种前后的临床和生化标志物。
共纳入 18 例患者 36 次接种事件的数据。13 例患者为中毒型代谢紊乱,包括有机酸血症(OA)、尿素循环障碍(UCD)、枫糖尿症(MSUD)和苯丙酮尿症(PKU);4 例患者为能量代谢障碍,包括脂肪酸代谢障碍和 LIPIN 1 缺乏症;1 例患者为糖原贮积症(GSD)5 型。17 例患者接受了 BNT162b2 疫苗接种,1 例因长 QT 综合征而接种了 CoronaVac 疫苗。脂肪酸代谢障碍、LIPIN 1 缺乏症和 GSD 5 型归入“代谢性肌病”同一组。在 2 例 PKU 患者中,第二剂疫苗接种后 24 小时内血浆苯丙氨酸水平显著升高。在疫苗接种后 1 个月内,无 OA、UCD、MSUD 和代谢性肌病患者因代谢性失代偿而发生急性代谢性攻击和紧急就诊。
在 18 例 IEM 儿童队列中,COVID-19 疫苗未引起急性代谢性失代偿。