The management of childbirth and the postpartum period in a patient with acute intermittent porphyria, an anesthesiologist/resuscitator’s tactics

Background: Acute intermittent porphyria is a group of rare hereditary metabolic diseases caused by a genetic defect of the enzymes involved in heme biosynthesis that leads to the excessive accumulation of porphyrins in the body. Surgical intervention and the use of various medications can result in an exacerbation of acute intermittent porphyria.

Case report: The paper describes a clinical case of a 25-year-old patient diagnosed as having 38 week’s gestation, acute intermittent porphyria, a condition after two heme arginate therapy cycles started in 2019, and clinical hematological compensation, porphyritic polyneuropathy, Stage 2 chronic kidney disease. The patient was admitted in a satisfactory condition to the V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, after a telemedical consultation to be examined and prepared for surgical delivery. There was no clinically significant polyneuropathy, neurological deficit, and autonomic nervous system dysfunction during pregnancy. Three days before the date of delivery, infusion preparation was done using a 20% glucose solution of 1000 ml per day, daily serum sodium monitoring, single determination of the urinary porphyrinogen level (69 mg/l). General balanced anesthesia was the method of choice. The postoperative period occurred without respiratory and hemodynamic disorders. The investigators monitored the levels of serum sodium and iron, blood inflammation markers, creatinine, ferritin, and urea. The administration of heme arginate was not required due to her good preoperative preparation. At 24 hours after surgery, the patient was transferred to the specialized unit. She was discharged in a satisfactory condition on 7 postoperative days.

Conclusion: The described case demonstrates that there can be a favorable course of pregnancy, childbirth and the postpartum period in porphyria compensation, optimal choice of a method for delivery and anesthesia, adequate preoperative preparation.