Dental Treatment of Two Children with Niemann Pick Disease Type B Under General Anesthesia: Case Reports

Abstract

Niemann-Pick disease (NPD) is an autosomal recessive lysosomal lipid storage disorder with accompanying symptoms including hepatosplenomegaly and thrombocytopenia. Delayed or extensive dental treatment may need to be delivered under general anesthesia and the management of such treatments in these children may require advanced medical support including intensive care (IC). Two children with NPD type B who underwent dental treatment with general anesthesia and their post-operative follow-ups requiring in IC are were presented. Patients with NPD may present with fragile blood clots in extracted tooth socket and should be subjected to strict bleeding control standards; especially the ones that may require further respiratory assistance since oral intubation is an invasive application to the oral surgical site.

Gender-Specific Effects of Two Treatment Strategies in a Mouse Model of Niemann-Pick Disease Type C1

“…Information about gender differences in the dose of anesthetic management in patients with Niemann-Pick disease has not yet been reported. Only possible complications of anesthetic management and the necessary measures in NPC patients have been shown [ 122 , 123 , 124 , 125 , 126 , 127 , 128 ] and explained by the disease-related interference with pathologies of liver, spleen, lung, and heart [ 129 ]. Due to both the liver involvement and the probability of an alteration in drug metabolism, anesthetic drugs should be used with caution in NPC patients [ 122 ].…”