title: Prognosis of acute porphyria: occurrence of acute attacks, precipitating factors, and associated diseases.
abstract: We evaluated the prognosis of acute porphyria among 206 adult Finnish patients with acute intermittent porphyria (AIP) or variegate porphyria (VP). The series represents all known patients with these porphyrias in Finland. Of the 47 patients who had a total of 117 acute attacks during the period 1967-1989, 6 died during an attack and 21 attacks were associated with paresis; the frequency of severe attacks was significantly smaller than before 1967 (p = 0.00002). Most pareses and deaths occurred because of a delay in diagnosis and inappropriate treatment of porphyria. For those patients who were symptom-free at the time of diagnosis (1365 follow-up years), the risk of the first subsequent attack was significantly smaller than for those who had had an acute attack before the diagnosis of porphyria (1047 follow-up years, p = 0.005). In addition, milder symptoms of porphyria were more common among those who had had previous attacks than among those who had not (p less than 0.00001). In AIP the risk of attacks correlated with the excretion of porphobilinogen in the urine during remission among adults (p = 0.03); a low rate of excretion predicted freedom from acute attacks. A regular use of many precipitating drugs was never associated with symptoms of porphyria. Two percent of the surgical operations and 4% of the pregnancies were associated with acute attacks. Nearly one-third of the women had symptoms of porphyria associated with the menstrual cycle, but these seldom proceeded to an acute attack. Forty-six percent of the women had used sex-hormone preparations regularly; 2 of them (4.5%) experienced associated acute attacks. Patients with AIP or VP showed increased incidences of hepatocellular carcinoma, and probably also chronic renal failure and hypertension.
authors.affiliation: Third Department of Medicine, University of Helsinki, Finland.
authors.forename: R
authors.initials: R
authors.lastname: Kauppinen
authors.forename: P
authors.initials: P
authors.lastname: Mustajoki
mesh_terms: D000208:Acute Disease; D000367:Age Factors; D002423:Cause of Death; D005387:Finland / Q000453:epidemiology; D006801:Humans; D015993:Life Tables; D011162:Porphobilinogen / Q000652:urine; D011164:Porphyrias / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D012737:Sex Factors; D011795:Surveys and Questionnaires
chemical_list: D011162:Porphobilinogen