[Clinical facets of hereditary angioedema among Swiss patients].
Hereditary angioedema (HAE) is a rare, autosomal dominant disease due to functional deficiency of C1-esterase inhibitor (C1-INH). In this observational study anamnestic, clinical and treatment data from forty patients were retrospectively analysed. Thirty nine of the patients suffered from type I of HAE and one patient from type II. Between first manifestation of the disease and correct diagnosis a median time lag of 10 years was observed. Two C1-INH deficient individuals had no symptoms so far; 36 patients suffered from recurrent, self-limiting abdominal attacks (convulsion, vomiting and diarrhea); 32 patients presented with edema of the (sub-) cutis. Thirty percent of swelling attacks involved the upper respiratory tract and two larynx attacks needed intubation. Hormonal changes in 25% of the female patients were associated with an aggravation of the attacks. Long-term therapy was established in 19 patients; treatment of acute attacks was performed in 10 patients and 11 patients needed no therapy.
Wais-Nöcker
B
B
Abteilung Hämatologie und Hämatologisches Zentrallabor, Luzerner Kantonsspital. walter.wuillemin@ksl.ch
Steiner
U
U
Späth
P
P
Wuillemin
Walter A
WA
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency / Q000235:genetics / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein; D003613:Danazol / Q000627:therapeutic use; D057210:Delayed Diagnosis; D004965:Estrogen Antagonists / Q000627:therapeutic use; D005260:Female; D056829:Hereditary Angioedema Types I and II / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D008487:Medical History Taking; D008875:Middle Aged; D012307:Risk Factors; D014148:Tranexamic Acid / Q000627:therapeutic use; D055815:Young Adult
D000933:Antifibrinolytic Agents; D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; D004965:Estrogen Antagonists; C469952:SERPING1 protein, human; D014148:Tranexamic Acid; D003613:Danazol