Pediatric Neurology Unit and Magen Rare Disease Center, Wolfson Medical Center, Holon and Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
1(st) Department of Pediatrics, Hippokratio General Hospital, Aristotle University, Medical School, Thessaloniki, Greece.
Eur J Paediatr Neurol. 2021 Nov;35:93-99. doi: 10.1016/j.ejpn.2021.10.002. Epub 2021 Oct 7.
Inborn errors of metabolism (IEM) are genetic disorders due to a defective metabolic pathway. The incidence of each disorder is variable and depends on the respective population. Some disorders such as urea cycle disorders (UCD) and organic acidurias, pose a high risk for a metabolic crisis culminating in a life-threatening event, especially during infections; thus, vaccines may play a crucial role in prevention. However, there are different triggers for decompensations including the notion that vaccines themselves can activate fever and malaise. Additionally, many of the IEM include immunodeficiency, placing the patients at an increased risk for infectious diseases and possibly a weaker response to immunizations. Since metabolic crises and vaccine regimens intersect in the first years of life, the question whether to vaccinate the child occupies parents and medical staff. Many metabolic experts hesitate to vaccinate IEM patients, disregarding the higher risk from the direct infections. In this paper we summarize the published data regarding the safety and recommendations for vaccinations in IEM patients, with reference to the risk for decompensations and to the immunogenic component.
先天性代谢缺陷（IEM）是由于代谢途径缺陷引起的遗传疾病。每种疾病的发病率不同，取决于各自的人群。一些疾病，如尿素循环障碍（UCD）和有机酸血症，存在发生代谢危机的高风险，最终导致危及生命的事件，尤其是在感染期间；因此，疫苗可能在预防方面发挥关键作用。然而，代偿失调的诱因不同，包括疫苗本身可能会引发发热和不适的观点。此外，许多 IEM 包括免疫缺陷，使患者更容易感染传染病，并且可能对免疫接种的反应较弱。由于代谢危机和疫苗方案在生命的最初几年相交，是否给孩子接种疫苗的问题让父母和医务人员感到困扰。许多代谢专家不愿为 IEM 患者接种疫苗，而忽略了直接感染的更高风险。本文总结了关于 IEM 患者疫苗接种安全性和建议的已发表数据，参考了代偿失调的风险和免疫成分。
