Tuesday, 9 December 2014
Exhibition Hall-Poster Area (Sul America)
Fabiane Milena Castro Araújo Pimenta, MD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Vivian Alves Costa, MD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Ana Karolinne Burlamaqui Melo, MD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Aline Lury Aoki, MD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Sandra Mitie Palma, MD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Neusa Falbo Wandalsen, PhD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Rosemeire Navickas Constantino-Silva, PhD
,
Laboratory of Clinical Immunology, Center of Research, Faculty of Medicine ABC, Santo Andre - SP, Brazil
Anete Grumach, MD, PhD
,
Outpatient Group of Recurrent Infections, Faculty of Medicine ABC, SP, Brazil, Santo Andre, Brazil
Background: Hereditary angioedema (HAE) with normal C1 esterase inhibitor (C1INH) was described for the first time in 2000. It was characterized by subcutaneous, gastrointestinal and laryngeal edema with familial history. Triggering factors are: stress, hormonal factors, trauma and infections. The authors evaluate response to therapy in patients with HAE without C1-INH deficiency.
Methods: It was analized therapeutical response to hereditary angioedema without deficiency of C1INH. Patients with clinical symptoms compatible with HAE have been included after normal quantitative and functional C1INH levels and positive family history for HAE, independent of factor XII mutation .
Results: Nineteen patients have been identified (2M:17F; 20-60 years old). The following therapies were oriented: combined contraceptive substitution for progestagen (10/19); treatment with progestagen (2/19); tranexamic acid (15/19): 1250mg (2), 1000mg (1), 750 (5), 500 mg (4), 250 mg (1); oxandrolon (5/19) (0.5 mg-5mg/day), danazol 200mg/day (1/19) and combined therapy woth oxandrolon and tranexamic acid in two patients. Icatibant was used in seven patients with clinical improvement. One of them reported increasing frequency of attacks after repeated use of this drug. Two patients received fresh frozen plasma during attacks with clinical improvement.
Conclusions: HAE without C1-INH deficiency has no established treatment. Clinical improvement was evident with the exclusion of combined contraceptives. The majority of the patients presented clinical response to tranexamic acid in variable doses. Icatibant was adequate for the therapy of attacks.
