Acute intermittent porphyria is a rare autosomal dominant disease caused by a mutation in the gene that codes for the heam biosynthetic enzymes porphobillinogen deaminase. Acute bouts of neuropsychiatric dysfunction, as well as neurovisceral manifestations such as acute abdomen, are common symptoms of the condition. 54 percent of pregnant women report an intensification of attacks in the form of seizures and acute abdomen as a result of hormonal changes. The frequency of acute intermittent porphyria is 1-2/200000 in general. The example of a 36-year-old lady with G4P1L2A2 who requested a pregnancy abortion at 10+4 weeks of pregnancy is presented. The patient was diagnosed with acute intermittent porphyria with porphyric polyneuropathy six months prior. Acute stomach discomfort, numbness, and weakness in all four limbs were her prominent symptoms at the time. Porphobilinogen and 5 ALA levels in her urine were both high. Her CECT and nerve conduction testing in the brain were also normal. Since then, she's been on Tablet Gabapentin 300 mg H.S. She successfully ended her pregnancy while under spinal anaesthetic via suction and evacuation, and she refused copper-T insertion. During the procedure, only safe medicines were utilised, and she was discharged with the suggestion of barrier contraception. Because acute bouts of porphyria are more common during pregnancy and are associated with a high rate of maternal and foetal mortality, it is critical to avoid triggering factors such as medications.
Author(S) Details
Pragati Meena
Department of Obstetrics & Gynaecology, JNU Medical College and Hospital, JNUIMSRC Jaipur, India.
Sandhya Jain
Department of Obstetrics & Gynaecology, University College of Medical Sciences and GTB Hospital, Delhi-110095, India.
Shalini Rajaram
Department of Obstetrics & Gynaecology, University College of Medical Sciences and GTB Hospital, Delhi-110095, India.
Neerja Goyal
Department of Obstetrics & Gynaecology, University College of Medical Sciences and GTB Hospital, Delhi-110095, India.
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