Department of Oral Surgery, Medical University of Lodz, Lodz, Poland.
Shanxi Oral Disease Prevention and Treatment Center, Shanxi Provincial People's Hospital, Taiyuan, People's Republic of China.
Ann Allergy Asthma Immunol. 2022 Jul;129(1):95-100. doi: 10.1016/j.anai.2022.03.014. Epub 2022 Mar 26.
In the diagnosis of perioperative anaphylaxis, it is essential to define its cause to prevent future reexposures, especially in children with Noonan Syndrome who are characterized by a large number of systemic features and wide-ranging dysmorphia. From an oral surgeon's point of view, apart from an increased risk of tumor incidence, diverse hematologic anomalies are of high concern.
Perioperative management of such patients often requires the use of fibrin sealants, which contain aprotinin. A certain number of anaphylaxis cases have been observed in our daily practice during such treatment, which was the reason for this evaluation.
The study was conducted to retrospectively review perioperative anaphylaxis grade II and above in children with Noonan Syndrome who underwent surgeries in the Department of Oral Surgery, Medical University of Lodz, between 2006 and 2021.
Out of the 16 cases of suspected anaphylaxis to aprotinin, 14 were observed in children with Noonan Syndrome. The postoperative serologic screening revealed positive results for qualitative aprotinin-specific immunoglobulin (Ig) G, highly elevated quantitative aprotinin-specific IgG, and slightly elevated aprotinin-specific IgE antibodies. Interestingly, previous aprotinin administration or contact in the past 12 months was excluded.
Given that fibrin sealants are typically used in various surgical practices and although the anaphylaxis reactions in such cases are rare, it is essential to be cautious in patients with RASopathies who are at a high risk of developing anaphylaxis.
在围手术期过敏反应的诊断中，明确其病因至关重要，以避免再次暴露，尤其是在诺南综合征（Noonan Syndrome）患儿中，其特征是大量全身性表现和广泛的畸形。从口腔外科医生的角度来看，除了肿瘤发病率增加之外，各种血液学异常也令人高度关注。
此类患者的围手术期管理通常需要使用含有抑肽酶的纤维蛋白密封剂。在我们的日常实践中，在这种治疗过程中观察到一定数量的过敏反应病例，这就是进行这项评估的原因。
该研究旨在回顾性分析 2006 年至 2021 年期间在罗兹医科大学口腔外科接受手术的 Noonan 综合征患儿中发生的 II 级及以上围手术期过敏反应病例。
在 16 例疑似抑肽酶过敏反应的病例中，有 14 例发生在 Noonan 综合征患儿中。术后血清学筛查显示定性抑肽酶特异性免疫球蛋白（Ig）G 阳性、定量抑肽酶特异性 IgG 显著升高和稍高的抑肽酶特异性 IgE 抗体。有趣的是，排除了既往抑肽酶给药或过去 12 个月内接触的情况。
鉴于纤维蛋白密封剂通常用于各种外科手术，尽管此类病例中的过敏反应罕见，但对于 RAS 病变患者仍需谨慎，因为这些患者发生过敏反应的风险较高。
