Pregnancy and the Acute Porphyrias

Abstract

An analysis is presented of the obstetric histories of 50 women with acute porphyria, comprising 39 with acute intermittent porphyria, 3 with variegate porphyria and 8 with hereditary coproporphyria. Fifty-four per cent of the women with acute intermittent porphyria had an acute attack of porphyria in pregnancy and/or the puerperium. Only one maternal death was recorded. One patient with variegate porphyria and two with hereditary coproporphyria had an attack related to pregnancy. The total fetal wastage was 13 per cent. The babies born to mothers with acute intermittent porphyria, who experienced an acute attack during pregnancy, were smaller than those in which no such attack occurred (P<O.OOl). In 13 non-porphyric primigravidae there was a rise in urinary excretion of 6-aminolaevulinic acid, porphobilinogen and coproporphyrin up to the 28th week of gestation. It is probable that pregnancy has some deleterious effects in acute porphyria but the prognosis of the porphyric pregnancy is much better than the literature suggests.

Reversible Posterior Encephalopathy Syndrome in Patient with Acute Intermittent Porphyrin: A Case Report

“…Neilson, et al [4] reported that maternal mortality was 42.5% due to attacks of porphyria during pregnancy. Brodie, et al [5] showed that 54% (27/50) of women with acute porphyria had an acute attack during pregnancy and that the babies born were significantly smaller than those who did not have a porphyria crisis. Andersson, et al [6] demonstrated that miscarriage was more common amongst women with manifest AIP.…”

Acute Intermittent Porphyria With Seizure and Paralysis in the Puerperium

“…According to the study of pregnancy outcome in patients with known porphyria, the attack rate is only 16% in the antenatal period and 8% in puerperium. 12 We believe tllat the initial porphyria attack, which took the form of a generalized seizure and hypertension in our patient, occurred after cesarean delivery and probably was related to reduced caloric intake after surgery and pyelonephritis. The use of phenytoin and nifedipine to conn'ol her seizure and hypertension further aggravated the metabolic derangement, resulting in progressive motor neuropathy.…”

Medical Termination of Pregnancy in Acute Intermittent Porphyria

“…A survey by Brodie et al [2] stated that 54% of women with the disease who become pregnant had an acute attack during pregnancy or the puerperium. Our patient had suffered acute abdominal attacks in her last pregnancy and even neurological dysfunction in the form of seizures and was unwilling to take risk of continuing pregnancy.…”

“…Given a 54-95% increased risk of acute episodes in pregnancy, the couple opted for a pregnancy termination [2] . Anaesthetist opinion was taken regarding anaesthesia and drugs to be avoided, as a large number of drugs can precipitate an acute attack.…”