To avoid future reexposures, it was crucial to identify the origin of perioperative anaphylaxis when making a diagnosis. It was especially true for children with Noonan Syndrome, who exhibited various systemic symptoms and extensive dysmorphia. A variety of hematologic irregularities were of great concern from the perspective of an oral surgeon, in addition to an elevated risk of tumor incidence. The use of fibrin sealants, which contain aprotinin, was frequently necessary for the perioperative care of such patients. The reason for this assessment was to observe a specific number of anaphylactic cases with such treatment in researcher’s routine clinical practice. For a study, they sought to retrospectively examine cases of perioperative anaphylaxis grade II and higher in Noonan Syndrome kids who underwent oral surgery at the Medical University of Lodz between 2006 and 2021. About 14 of the 16 suspected cases of anaphylaxis to aprotinin were kids with Noonan Syndrome. Positive results for qualitative aprotinin-specific immunoglobulin (Ig) G, significantly elevated quantitative aprotinin-specific IgG, and marginally elevated aprotinin-specific IgE antibodies were found during the postoperative serologic screening.

Interestingly, the exclusion of prior aprotinin administration or interaction during the previous 12 months was excluded. Because fibrin sealants were frequently employed in various surgical procedures, even though anaphylactic reactions in these situations were uncommon, it was crucial to exercise caution when treating individuals with RASopathies. They had a high risk of experiencing anaphylaxis.

Source – sciencedirect.com/science/article/abs/pii/S108112062200206X