title: [Acute intermittent porphyria in pregnancy: glucose or hematin therapy?].
abstract: The symptoms and management of acute intermittent porphyria (AIP) during pregnancy and delivery are presented on the basis of three case reports. Two patients became asymptomatic after infusion of a concentrated glucose solution (600 ml 50% glucose = 300 g glucose per day), while the third patient needed treatment with hematin (1 mg/kg body weight, 6 infusions at intervals of 12 hours) because of deterioration of psychic state in spite of glucose infusion. Whenever an attack of AIP occurs, concentrated glucose solution should be administrated as a first measure accompanied by an analgesic and possibly a neuroleptic agent after elimination of precipitating factors. However, if symptoms persist for 48-96 hours or the psychic and/or neurological state of the patient further deteriorates, administration of 1-3 mg hematin per kg body weight can be recommended.
authors.affiliation: Abteilung Gynäkologie und Geburtshilfe, Kantonsspital Fribourg.
authors.forename: M
authors.initials: M
authors.lastname: Isenschmid
authors.forename: C
authors.initials: C
authors.lastname: König
authors.forename: C
authors.initials: C
authors.lastname: Fässli
authors.forename: A
authors.initials: A
authors.lastname: Haenel
authors.forename: W
authors.initials: W
authors.lastname: Hänggi
authors.forename: H
authors.initials: H
authors.lastname: Schneider
mesh_terms: D000208:Acute Disease; D000328:Adult; D000622:Aminolevulinic Acid / Q000097:blood; D004359:Drug Therapy, Combination; D005260:Female; D005950:Glucose Solution, Hypertonic; D006427:Hemin / Q000627:therapeutic use*; D006801:Humans; D011162:Porphobilinogen / Q000097:blood; D011164:Porphyrias / Q000097:blood / Q000188:drug therapy*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy*
chemical_list: D005950:Glucose Solution, Hypertonic; D006427:Hemin; D011162:Porphobilinogen; D000622:Aminolevulinic Acid
vernacular_title: Akute intermittierende Porphyrie in der Schwangerschaft: Therapie mit Glukose oder Hämatin?