Porphyric crisis. Experience of 30 episodes.

The experience of 30 porphyric crisis is reviewed in 25 patients attended s ince 1967:21 patients had 1 crisis, 3 had 2, and 1 had 3 of these episodes. In all patients, porphyria was diagnosed in relation to one crisis, even t hough many of them had family histories and/or previous clinical symptoms o f this disease. There was clear predominance (80%) of women, but they are a lso a majority among acute porphyrias. The most frequent symptoms were: abd ominal pain, tachycardia, dark urine, neurological and psychiatric alterati ons and arterial hypertension. The neurological alterations required the us e of a respirator in 9 crisis (33%), which was maintained in 2 patients dur ing 4 months. In 6 crisis (20%) there were no neurological symptoms. Among laboratory tests, hyponatremia was notable for its frequency (53.4%) and in tensity. Increase in urinary porphobilinogen, a requirement for diagnosis, between 15 and 130 times the normal value was observed. Septic complication s, such as pneumonia, septicemia, and urinary infection, were frequent (50% ). Factors suspicious of triggering crisis episodes were: drugs, usually mo re than 2, in 50% of the cases; pregnancy in 30% of the women and in a less er proportion, intense exercise, and surgery. In 10 patients, crisis trigge ring factors were not identified or informed. The role of pregnancy, childb irth delivery or puerperium in causing a crisis is not clear, because the p atients who had a crisis related to them had 15 other pregnancies without i ncidents; besides, in the pregnancy which was accompanied by a crisis, ther e was always one or more than one potentially triggering drug present. The first therapeutic step was oral and/or parenteral administration of an over load of carbohydrates and, if there was no response, intravenous infusion o f hematin was prescribed. Four (13.3%) patients died even though they had r eceived hematin, but it had been administered too late due to a delay in di agnosis. In surviving patients, there were no organic sequels of any kind.