title: [Anesthetic management of a patient with erythropoietic protoporphyria for laparoscopic appendectomy].
abstract: Porphyria is a hereditary disorder due to reduction in enzyme activity of heme synthesis system, resulting in accumulation of heme precursors. Erythropoietic protoporphyria (EPP) is a type of porphyria. There are varieties of clinical expressions of EPP such as hepatic damage and photosensitivity, caused by the accumulation of protoporphyrin in the liver and the skin. Therefore it is important to prevent development of these clinical expressions. A 36-year-old woman with EPP was scheduled for a laparoscopic appendectomy. Anesthesia was induced with remifentanil, sevoflurane and rocuronium, and maintained with remifentanil, fentanyl and sevoflurane. She had performed normal daily activities without taking countermeasures against photosensitivity, so we did not change the light in the operating room, and allowed using endoscopy. The surgery was performed without any complications. No skin symptom was observed perioperatively. The level of aminotransferase was elevated temporally after the operation, while the level of protoporphyrin was unchanged. Therefore, we considered there was no manifestation of EPP perioperatively. In general, patients with EPP do not develop acute attacks induced by drugs like barbiturates. Since there was a case report of severe liver dysfunction of EPP, we did not use any contraindicated drugs in patients with acute intermittent porphyria in this patient. Considering a possibility of motor nerve damage in severe EPP, we carried out anesthetic management without epidural anesthesia.
authors.affiliation: Department of Anesthesiology and Critical Care, Hiroshima University Hospital, Hiroshima 734-8551.
authors.forename: Yoshie
authors.initials: Y
authors.lastname: Kuwahara
authors.forename: Shima
authors.initials: S
authors.lastname: Taguchi
authors.forename: Hideki
authors.initials: H
authors.lastname: Fukuda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hamada
authors.forename: Masashi
authors.initials: M
authors.lastname: Kawamoto
mesh_terms: D000328:Adult; D000768:Anesthesia, General / Q000379:methods*; D001062:Appendectomy; D005260:Female; D006801:Humans; D010535:Laparoscopy; D046351:Protoporphyria, Erythropoietic / Q000150:complications*