[Porphyric crisis: experience of 30 episodes].
The experience of 30 porphyric crisis is reviewed in 25 patients attended since 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 though many of them had family histories and/or previous clinical symptoms of this disease. There was clear predominance (80%) of women, but they are also a majority among acute porphyrias. The most frequent symptoms were: abdominal pain, tachycardia, dark urine, neurological and psychiatric alterations and arterial hypertension. The neurological alterations required the use of a respirator in 9 crisis (33%), which was maintained in 2 patients during 4 months. In 6 crisis (20%) there were no neurological symptoms. Among laboratory tests, hyponatremia was notable for its frequency (53.4%) and intensity. Increase in urinary porphobilinogen, a requirement for diagnosis, between 15 and 130 times the normal value was observed. Septic complications, such as pneumonia, septicemia, and urinary infection, were frequent (50%). Factors suspicious of triggering crisis episodes were: drugs, usually more than 2, in 50% of the cases; pregnancy in 30% of the women and in a lesser proportion, intense exercise, and surgery. In 10 patients, crisis triggering factors were not identified or informed. The role of pregnancy, childbirth delivery or puerperium in causing a crisis is not clear, because the patients who had a crisis related to them had 15 other pregnancies without incidents; besides, in the pregnancy which was accompanied by a crisis, there was always one or more than one potentially triggering drug present. The first therapeutic step was oral and/or parenteral administration of an overload of carbohydrates and, if there was no response, intravenous infusion of hematin was prescribed. Four (13.3%) patients died even though they had received hematin, but it had been administered too late due to a delay in diagnosis. In surviving patients, there were no organic sequels of any kind.
Morales Ortega
X
X
Departamento de Medicina Occidente, Hospital San Juan de Dios, Universidad de Chile, Santiago, Chile.
Wolff Fernández
C
C
Leal Ibarra
T
T
Montaña Navarro
N
N
Armas-Merino
R
R
D000293:Adolescent; D000328:Adult; D005260:Female; D006427:Hemin / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011162:Porphobilinogen / Q000652:urine; D017118:Porphyria, Acute Intermittent / Q000175:diagnosis* / Q000188:drug therapy*; D011247:Pregnancy; D013997:Time Factors
D006427:Hemin; D011162:Porphobilinogen