No abstract

A total of 7 families with hereditary angioneurotic oedema (HANE) have been found in Finland. Six HANE patients have died from laryngeal oedema, 27 patients with diagnosed HANE are alive and 21 members have a haematological abnormality typical of HANE, i.e. a deficiency of the inhibitor of the activated first component of complement (C1‐INH), but no manifest symptoms. The largest family has 363 living members, 303 of whom were investigated for C1‐INH, C4 and C3. Fourteen patients had HANE, 18 relatives were symptomless but had C1‐INH deficiency, and 3 members of the family had died from laryngeal oedema. In two families only one case of HANE was diagnosed, the parents in both cases being symptomless with normal C1‐INH levels. All patients who had died from laryngeal oedema and 10 of the 27 HANE patients still alive had a typical triad of paroxysmal abdominal pain, peripheral oedema and laryngeal oedema. Six patients have had abdominal attacks alone, three peripheral oedema alone and two only laryngeal oedema. The age at onset of symptoms was 1–51 years. Three patients, who have received continuous methyltestosterone therapy, had hardly any symptoms during the treatment. Thirteen patients have received tranexamic acid, either during an attack or continuously, with positive effects in all except two. Cinnarizine treatment was beneficial in three out of four cases, both when given continuously or during an attack.

The HLA haplotypes were defined in a series including 1 1 patients with hereditary angioneurotic edema (HANE), five symptom-free subjects with pathological laboratory findings characteristic of HANE, and their 33 healthy kinsmen. The subjects belonged to two Finnish families representing the more common form of HANE which is recognized by the irnmunochernically observed decrease of the inhibitor (Cl-INH) blocking the esterase activity of the first complement component. No linkage between HANE and the HLA system was found.

Yersinia agglutinins and—when suitable samples were available—also cultural and histological evidence of Yersinia infections have been searched for from 400 hospital patients during the period 1965–70 at Lappeenranta, Finland. In 31 of them immune responses compatible with infections caused by Yersinia pseudotuberculosis (YP) serotypes I, II, III or V or Yersinia enterocolitica (YE) serotypes 3 or 9 were found. In seven patients the diagnosis was confirmed by cultural or histological findings. YE serotype 9 was involved in 20 cases. Of the 31 patients 19 had disease states commonly regarded as immunological late effects of bacterial infections. Such states included erythema nodosum, temporary arthritides or arthralgias and also pulmonary changes resembling sarcoidosis. The joint symptoms met in children were indistinguishable from so‐called benign aseptic arthritis. One patient had a typical Löfgren's syndrome.

Six out of 7 Finnish patients suffering from hereditary angioneurotic edema were helped during attacks, by treatment with tranexamic acid (AMCA, Cyklokapron, Kabi) in doses of 1.5 g 3 times daily, follow-up time 3-25 months. 3 of these patients also had continuous AMCA treatment, in the course of which 2 remained nearly symptom-free on a dosage of 1 g 2-3 times daily. Even the third one had shorter and milder attacks. One patient, however, had to stop taking AMCA after 6 weeks' treatment, because of fatigue and nausea. Follow-up time for the others was 9-11 months. For comparative purposes, 3 patients were given continuous treatment with an antihistamine, cinnarizine in a dosage of 20-30 mg daily. Two of the patients were helped by it, one becoming nearly symptom-free and the other having fewer and milder attacks; follow-up time 9-10 months.