title: [Acute intermittent porphyria in the puerperium].
abstract: BACKGROUND
  Acute intermittent porphyria emerges as a result of partial defect of porphobilinogen deaminase and is manifested by repeated episodes of somatic, psychiatric and neurological disorders. The disease is conducted via the autosomal-dominant gene of variable penetration, so most of the carriers never experience seizures. Timely making of diagnosis, screening of blood relatives of the patient and education of patients on avoidance of provoking factors are the key to adequate treatment.
  
  
  METHODS
  A 23-year-old patient having born the third child was hospitalized due to pains in the abdomen and convulsive seizures nine days after the vaginal delivery. At admittance, she suffered a generalized convulsive seizure of clonic-tonic type. The patient immediately underwent a complete clinical, laboratory, bacteriological and ultrasound examination. Bearing in mind the fact that the patient had several convulsive seizures even after the given neurological therapy, haem-arginate was introduced into therapy during four days. The administration of haem-arginate led to the normalization of blood pressure, pulse and bowel function. The administration of haem-arginate led to the normalization of blood pressure, pulse and bowel function. The patient was treated by a team of doctors, in the intensive care ward, with the use of medicaments, which are allowed in the case of acute porphyria. Sixteen days after the admittance, with clean neurological status and gynaecological and ultra-sound findings, she was released for ambulatory treatment.
  
  
  CONCLUSIONS
  The presented case exhibits the gravity of making a diagnosis of acute intermittent porphyria in puerperium and the necessity of multi-disciplinary approach in treating this disease. Acute intermittent porphyria should be considered in cases of ambiguous abdominal pain, as well as in patients having abdominal pains followed by neuro-psychiatric disorders.
authors.forename: Radmila
authors.initials: R
authors.lastname: Sparić
authors.forename: Ivana
authors.initials: I
authors.lastname: Berisavac
authors.forename: Ljubica
authors.initials: L
authors.lastname: Arsenijević
authors.forename: Milica
authors.initials: M
authors.lastname: Berisavac
authors.forename: Milan
authors.initials: M
authors.lastname: Dokić
authors.forename: Olivera
authors.initials: O
authors.lastname: Savić
authors.forename: Tatjana
authors.initials: T
authors.lastname: Ilić-Mostić
authors.forename: Sasa
authors.initials: S
authors.lastname: Kadija
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D017118:Porphyria, Acute Intermittent / Q000175:diagnosis* / Q000628:therapy; D049590:Postpartum Period; D011247:Pregnancy; D055815:Young Adult