title: Intensive care management of patients with acute intermittent porphyria: Clinical report of four cases and review of literature.
abstract: Acute intermittent porphyria (AIP), the most common and the most severe form of acute hepatic porphyria, is an autosomal dominant condition. It results from lower-than-normal levels (less than 50%) of porphobilinogen (PBG) deaminase. Patients may present commonly with gastrointestinal complaints and neuropsychiatric manifestations. Diagnosis may be confirmed with the presence of intermediary metabolites of haem synthesis, amino levulinic acid (ALA) and PBG in urine or with specific enzyme assays. Abdominal pain is the most common symptom (90%). Peripheral polyneuropathy, primarily motor with flaccid paresis of proximal musculature, with or without autonomic involvement, is characteristic. Respiratory failure necessitates ventilator and intensive care support. Avoidance of precipitating factors and the use of haem preparations and intravenous dextrose form the basis of management. Gabapentin and propofol, rather than the conventional antiepileptics appear to be the appropriate choice for seizure control. Here, we present intensive care management of four cases of AIP with varying clinical presentation.
authors.affiliation: Department of Neuroanaesthesiology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Madhur
authors.initials: M
authors.lastname: Mehta
authors.forename: Girija P
authors.initials: GP
authors.lastname: Rath
authors.forename: Uma P
authors.initials: UP
authors.lastname: Padhy
authors.forename: Manish
authors.initials: M
authors.lastname: Marda
authors.forename: Charu
authors.initials: C
authors.lastname: Mahajan
authors.forename: Hari H
authors.initials: HH
authors.lastname: Dash