Hereditary angioedema. Long-term follow-up of 88 patients. Experience of the Argentine Allergy and Immunology Institute.
Since the detection of the first patient with hereditary angioedema (HA) in 1978, 88 new patients belonging to 16 families have been referred to our clinic. Eighty patients had Type I disease, 5 Type II, and 3 Type III (secondary). We describe the clinical onset, frequent complications, diagnostic tests of the complement system, and abnormalities of the coagulation pathway linked to complement activation. Particular attention was paid to family members who could present succedaneum symptoms. The results of danazole and other therapies and protective and preventive treatment for surgery also are discussed.
Instituto Argentino de Alergia e Inmunología Potosí 3880 (1199) Capital Federal, Argentina. iaai@datamarkets.com.ar
J E
JE
Fabiani
A
A
Avigliano
J C
JC
Dupont
J E
JE
Fabiana
D000293:Adolescent; D000328:Adult; D000799:Angioedema / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D001118:Argentina; D001777:Blood Coagulation; D002648:Child; D002675:Child, Preschool; D002874:Chromosome Mapping; D002880:Chromosomes, Human, Pair 11; D003174:Complement C1 Inactivator Proteins / Q000235:genetics; D050718:Complement C1 Inhibitor Protein; D003181:Complement C4 / Q000032:analysis; D003165:Complement System Proteins / Q000502:physiology; D003613:Danazol / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male
D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D003181:Complement C4; D004965:Estrogen Antagonists; D003165:Complement System Proteins; D003613:Danazol