doiSerbia

Acute intermittent porphyria in the puerperium

Sparić Radmila (Institut za ginekologiju i akušerstvo, Klinički centar Srbije, Beograd)
Berisavac Ivana (Institut za neurologiju, Klinički centar Srbije, Beograd)
Arsenijević Ljubica (Institut za anesteziju i reanimaciju, Klinički centar Srbije, Beograd)
Berisavac Milica (Institut za ginekologiju i akušerstvo, Klinički centar Srbije, Beograd)
Dokić Milan (Institut za ginekologiju i akušerstvo, Klinički centar Srbije, Beograd)
Savić Olivera (Institut za neurologiju, Klinički centar Srbije, Beograd)
Ilić-Mostić Tatjana (Institut za anesteziju i reanimaciju, Klinički centar Srbije, Beograd)
Kadija Saša (Institut za ginekologiju i akušerstvo, Klinički centar Srbije, Beograd)

Introduction. 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 autosomaldominant 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. Case Outline. 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. Conclusion. 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.

Keywords: acute intermittent porphyria, puerperium, haem, diagnosis