Hereditary angioedema in Czechoslovakia. Clinical, immunological, genetic and therapeutic studies of 16 families.
The analysis of the findings in 16 families with hereditary angioedema detected in Czechoslovakia over the years 1975-1986 is being presented. In 14 families C1-inhibitor deficiency and in two families afunction of C1-inhibitor was established. Of the total number of 175 examined family members C1-inhibitor defect was registered in 66 subjects (60 with deficiency and 6 with afunction), and of these 48 suffered from clinical symptoms of edematous attacks affecting the skin, larynx, intestine and urinary tract, whereas 18 subjects were asymptomatic. Genealogical studies confirmed that the defect is inherited as an autosomal dominant trait. In 16 patients long-term prophylactic treatment with Danol was introduced. The effective minimum dosage was tested individually for each patient.
Starsia
Z
Z
Department of Microbiology, Medical Faculty, Comenius University, Bratislava.
Hegyi
E
E
Kuklinek
P
P
Cáp
J
J
Stefanovic
J
J
Lokaj
J
J
D000799:Angioedema / Q000235:genetics* / Q000276:immunology; D003174:Complement C1 Inactivator Proteins / Q000235:genetics; D003181:Complement C4 / Q000235:genetics; D003604:Czechoslovakia; D006801:Humans; D010641:Phenotype
D003174:Complement C1 Inactivator Proteins; D003181:Complement C4