The dental management of five paediatric patients with a history of acute intermittent porphyria.

Background: Acute Intermittent Porphyria (AIP) is a rare autosomal dominant metabolic disorder resulting from partial deficiency of porphobobilinogen deaminase, the third enzyme of the haem synthetic pathway. Patients with AIP may be vulnerable to acute neurovisceral attacks if exposed to certain drugs, including some drugs used in dental practice.

Methods: This article outlines the dental management of 5 children with a diagnosis of latent AIP or a family history of AIP. The local analgesic agent used in all cases was bupivacaine or levobupivacaine. Nitrous oxide and oxygen inhalation sedation was used in 2 cases and general anaesthesia, using propofol, isoflurane with nitrous oxide and oxygen, was used in another case. The dental treatment undertaken included restorations, endodontics and extractions.

Conclusions: Dental treatment using bupivacaine or levobupivacaine as local analgesic agents was successfully and safely provided for 5 children with a diagnosis of latent AIP or a family history of AIP.