title: The anaesthetic management of patients with congenital insensitivity to pain with anhidrosis.
abstract: BACKGROUND
  Congenital insensitivity to pain with anhidrosis (CIPA, or hereditary sensory and autonomic neuropathy type IV) is a rare, autosomal recessive disease, related to a mutation in the TrkA gene, characterized by inability to sweat, insensitivity to pain and recurrent episodes of hyperpyrexia. There are two Bedouin tribes in Israel with different mutations of the TrkA gene: one in the southern region and the other in the northern region. The Soroka University Medical Center is the referral centre for the entire southern region of Israel. One in 4500 anaesthesia cases involves a patient with CIPA.
  
  
  METHODS
  We reviewed 40 anaesthesia records of 20 patients with CIPA for anaesthetic technique and incidence of side-effects.
  
  
  RESULTS
  Sixteen patients developed complications in the immediate perioperative period: mild hypothermia in one patient and cardiovascular events in 15 others with one case of cardiac arrest. These complications were unrelated to the anaesthetic drug administered. There were no events of hyperthermia or postoperative nausea.
  
  
  CONCLUSIONS
  Cardiovascular complications following anaesthesia are common in patients with the southern Israel variant of CIPA. Hyperthermia, previously recognized as a major concern in patients with congenital insensitivity to pain with anhydrous, was not seen in our patients. We conclude that cardiovascular involvement is frequently encountered in CIPA patients following anaesthesia and is the major concern in their anaesthetic management.
authors.affiliation: Division of Anesthesiology and Critical Care Medicine, Soroka University Medical Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel.
authors.forename: V
authors.initials: V
authors.lastname: Rozentsveig
authors.forename: A
authors.initials: A
authors.lastname: Katz
authors.forename: N
authors.initials: N
authors.lastname: Weksler
authors.forename: A
authors.initials: A
authors.lastname: Schwartz
authors.forename: M
authors.initials: M
authors.lastname: Schilly
authors.forename: M
authors.initials: M
authors.lastname: Klein
authors.forename: G M
authors.initials: GM
authors.lastname: Gurman
mesh_terms: D000293:Adolescent; D000768:Anesthesia, General / Q000009:adverse effects; D018685:Anesthetics, Inhalation / Q000008:administration & dosage / Q000009:adverse effects; D018686:Anesthetics, Intravenous / Q000008:administration & dosage / Q000009:adverse effects; D001919:Bradycardia / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006323:Heart Arrest / Q000209:etiology; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D007022:Hypotension / Q000209:etiology; D007035:Hypothermia / Q000209:etiology; D007223:Infant; D007557:Israel; D008297:Male; D011183:Postoperative Complications; D012189:Retrospective Studies
chemical_list: D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous