Mastocytosis in Children: A Protocol for Management

Abstract

Mastocytosis is characterized by an

Anesthetic considerations in pediatric mastocytosis: a review

“…It is unclear which disease subtype seen in adolescence will progress into the adult form of the disease [25]. All adult cases are assumed to be systemic until clinical evaluation proves otherwise [26].…”

“…There are also ''familial'' KIT mutations, A533D, K509I, del419, but their clinical relevance is unknown [12,15]. Although there seems to be a correlation between the C -KIT mutation and the more severe forms of the disease, patients' genotypes have not been shown to consistently correlate with a particular phenotype [26,31,34].…”

“…So far, no known investigation has been found to consistently predict mast cell degranulation in these patients [4,26]. Furthermore, there are no evidence-based guidelines for preoperative prophylaxis of patients (adult or pediatric) with mastocytosis [26].…”

“…Furthermore, there are no evidence-based guidelines for preoperative prophylaxis of patients (adult or pediatric) with mastocytosis [26]. Pretreatment regimens have been based on accumulated case reports and, in the past, antihistamine prophylaxis was standard practice following reports of cardiovascular collapse in adult patients undergoing anesthesia [41,42].…”

“…H 1 -receptors facilitate preformed histamine release and are also involved in changes in capillary permeability [52]. In addition, H 1 -receptors interact with H 2 -receptors during vasodilation, prompting a recommendation that H 1 and H 2 -receptor antagonists be given together as prophylaxis [3,15,26,[52][53][54]. Glucocorticoids, as anti-inflammatories and mast cell stabilizers, are recommended in specific circumstances, for example anesthesia and surgery, or the rare case of recurrent cardiovascular collapse [12,15,27,30].…”