[Acute intermittent porphyria: report on 17 patients with 49 attacks (author's transl)].
In 17 patients (15 women, 2 men) with acute intermittent porphyria in the incidence of 23 clinical symptoms during 49 attacks was calculated. The most frequent symptoms in percentage of attacks were: Red colour of the urine 100%, abdominal pain 92%, tachycardia 88%, hypertension 75%, vomiting 54%, peripheral neuropathy 50%. In 35% of acute attacks a transient normochromic, normocytic anemia developed which is probably due to a disturbance of heme synthesis. Oliguria was found in 25%, azotemia in 12.5% of attacks. 4 patients with an average of 5 preceding acute attacks showed a persistent reduction of renal function during the symptom-free interval, in contrast to 12 patients with an average of 1.7 previous attacks and normal renal function. During the observation period from 1960-1974 3 (= 18%) of the 17 patients died.
D000208:Acute Disease; D000328:Adult; D000740:Anemia / Q000209:etiology; D003085:Colic / Q000209:etiology; D004064:Digestive System / Q000503:physiopathology; D005260:Female; D006801:Humans; D007668:Kidney / Q000503:physiopathology; D007674:Kidney Diseases / Q000209:etiology; D008297:Male; D008598:Menstruation; D008875:Middle Aged; D009420:Nervous System / Q000503:physiopathology; D011164:Porphyrias / Q000097:blood / Q000150:complications / Q000503:physiopathology; D012189:Retrospective Studies
Akute intermittierende Porphyrie. Beobachtungen bei 17 Fällen mit 49 akuten Schüben