title: Congenital insensitivity to pain with anhydrosis: report of a family case.
abstract: Congenital Insensitivity to pain with anhydrosis (CIPA) is a rare inherited disease. It is classified as hereditary sensory and autonomic neuropathy type IV. Pain insensitivity and autonomic deficits are present, but touch and pressure sensitivity are unimpaired. Mental retardation is usually present. We report a family case of a 5 years old girl and 2 years old boy with congenital insensitivity to pain, while discussing the clinical features and the anesthetic strategy of such patients. Patients with Congenital Insensitivity to Pain with anhydrosis may undergo surgery because of susceptibility to trauma due to absence of pain. The clinical features may intrinsically possess anesthetic challenges.
authors.affiliation: Department of anesthesia and intensive care, University Hospital Hassan II, Fez, Morocco.
authors.forename: Smael
authors.initials: S
authors.lastname: Labib
authors.forename: Mohamed
authors.initials: M
authors.lastname: Adnane Berdai
authors.forename: Sanae
authors.initials: S
authors.lastname: Abourazzak
authors.forename: Mustapha
authors.initials: M
authors.lastname: Hida
authors.forename: Mustapha
authors.initials: M
authors.lastname: Harandou
mesh_terms: D000758:Anesthesia / Q000009:adverse effects; D000777:Anesthetics / Q000008:administration & dosage / Q000009:adverse effects / Q000493:pharmacokinetics; D002675:Child, Preschool; D003967:Diarrhea / Q000209:etiology; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000235:genetics*; D006801:Humans; D007007:Hypohidrosis / Q000209:etiology; D008297:Male; D008305:Malignant Hyperthermia / Q000209:etiology / Q000517:prevention & control; D009064:Mouth Protectors; D000699:Pain Insensitivity, Congenital / Q000209:etiology; D011613:Psychotherapy; D020917:Receptor, trkA / Q000172:deficiency / Q000235:genetics; D012652:Self Mutilation / Q000209:etiology*; D035781:Siblings
chemical_list: D000777:Anesthetics; D020917:Receptor, trkA