| Abstrakt: |
Acute intermittent porphyria (AIP) is a rare autosomal dominant metabolic disorder' affecting the production of heme, the oxygen-binding prosthetic group of hemoglobin. It is characterized by a deficiency of the enzyme hydroxymethylbilane synthase; without this cytoplasmic enzyme, heme synthesis cannot finish, and the metabolite porphobilinogen accumulates in the cytoplasm. Some additional factors must also be present such as drugs, hormones, dietary changes, infections diseases and surgery that trigger the appearance of symptoms, which include neurological disorders, abdominal pain, constipation, and muscle weakness. We present a perioperative course of a pregnant woman with porphyria in association with hypothyroidism and its anesthetic management. |
