title: The dental management of five paediatric patients with a history of acute intermittent porphyria.
abstract: 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.
authors: Dental Dept., Our Lady's Children's Hospital, Crumlin, Dublin, Ireland. Eleanor.mcgovern@dental.tcd.ie
authors: E
authors: E
authors: McGovern
authors: P
authors: P
authors: Fleming
authors: A
authors: A
authors: O'Marcaigh
mesh_terms: D000293:Adolescent; D000766:Anesthesia, Dental / Q000379:methods*; D000768:Anesthesia, General; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D000779:Anesthetics, Local / Q000008:administration & dosage; D001566:Benzocaine / Q000008:administration & dosage; D002045:Bupivacaine / Q000008:administration & dosage; D002648:Child; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000628:therapy*; D005062:European Union; D017408:Guidelines as Topic; D006801:Humans; D009609:Nitrous Oxide / Q000008:administration & dosage; D017118:Porphyria, Acute Intermittent
chemical_list: D018685:Anesthetics, Inhalation; D000779:Anesthetics, Local; D009609:Nitrous Oxide; D001566:Benzocaine; D002045:Bupivacaine