Short-term prophylaxis in hereditary angioedema due to deficiency of the C1-inhibitor - a long-term survey

Allergy ◽  
2012 ◽  
pp. n/a-n/a ◽  
Author(s):  
H. Farkas ◽  
Z. Zotter ◽  
D. Csuka ◽  
E. Szabó ◽  
Z. Nébenfűhrer ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
C1 Inhibitor ◽  
Short Term ◽  
Short Term Prophylaxis ◽  
Long Term Survey

scholarly journals Short-term prophylaxis (STP) with plasma derived human C1 inhibitor concentrate (pdhC1-INH) in two pregnant women with hereditary angioedema (HAE): an experience in Rio de Janeiro – Brazil

2015 ◽  
Vol 8 ◽  
pp. A127
Author(s):  
Solange Oliveira Rodrigues Valle ◽  
Maria Luiza Oliva Alonso ◽  
Sérgio Duarte Dortas ◽  
Soloni Afra Pires Levy ◽  
Ana Paula Ferracciú Coutinho Millet ◽  
...  
Keyword(s):  
Pregnant Women ◽  
Hereditary Angioedema ◽  
Rio De Janeiro ◽  
C1 Inhibitor ◽  
Short Term ◽  
Short Term Prophylaxis

Short-term Prophylaxis With C1-inhibitor Concentrate In Patients With Hereditary Angioedema Prior To Surgical Procedures

2010 ◽  
Vol 125 (2) ◽  
pp. AB164 ◽  
Author(s):  
E. Rusicke ◽  
I. Martinez-Saguer ◽  
E. Aygören-Pürsün ◽  
T. Klingebiel ◽  
W. Kreuz
Keyword(s):  
Surgical Procedures ◽  
Hereditary Angioedema ◽  
C1 Inhibitor ◽  
Short Term ◽  
Short Term Prophylaxis

scholarly journals Short-term Prophylaxis for Delivery in Pregnant Women with Hereditary Angioedema with Normal C1-Inhibitor

2020 ◽  
Vol 42 (12) ◽  
pp. 845-848
Author(s):  
Caroline Guth de Freitas Batista de Moraes ◽  
Liya Regina Mikami ◽  
Lilian Pereira Ferrari ◽  
João Bosco Pesquero ◽  
Herberto José Chong-Neto ◽  
...  
Keyword(s):  
Case Report ◽  
Pregnant Women ◽  
Hereditary Angioedema ◽  
Genetic Test ◽  
Pathogenic Mutation ◽  
Normal Serum ◽  
C1 Inhibitor ◽  
Short Term ◽  
Exon 9 ◽  
Short Term Prophylaxis

Abstract Objective To verify the efficacy of short-term prophylaxis for vaginal or cesarean section childbirth with plasma-derived C1-inhibitor concentrate in pregnant women. They should have hereditary angioedema (HAE) and normal plasma C1-inhibitor. Methods Case report of pregnant women diagnosed with HAE with normal C1-inhibitor who had been treated with intravenous C1-inhibitor concentrate for prophylaxis of angioedema attacks when hospitalized for delivery. The exon 9 of the Factor 12 (F12) genotyping gene was performed by automatic sequencing in all patients. Results Three cases of pregnant women with HAE with normal serum level of C1-inhibitor are reported. The genetic test detected the presence of a pathogenic mutation in the F12 gene. Deliveries occurred uneventfully and patients had no HAE symptoms in the following 72 hours. Conclusion C1-inhibitor concentrate could be useful to prevent angioedema attacks during and after delivery.

scholarly journals Diagnosis and treatment of hereditary angioedema with normal C1-inhibitor level

2020 ◽  
Vol 92 (12) ◽  
pp. 86-90
Author(s):  
A. V. Emelyanov ◽  
E. V. Leshenkova ◽  
G. A. Kameneva
Keyword(s):  
Hereditary Angioedema ◽  
Autosomal Dominant ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Genetic Mutation ◽  
C1 Inhibitor ◽  
Short Term ◽  
Inhibitor Level ◽  
Life Threatening

Hereditary angioedema (HAE) with normal C1-inhibitor level is a rare potentially life-threatening disorder with autosomal dominant inheritance which was first described in 2000. Its clinical presentation is similar to HAE with C1-deficiency. The review is summarized data about its prevalence, mechanisms, genetics and diagnostic criteria. Different subtypes and treatment options (on demand, short term and long-term prophylaxis) are discussed. We describe family clinical cases of 2 female patients with normal C1-inhibitor and plasminogen gene mutation. Their features were late diagnosis (in 10 and 25 years after the onset of symptoms), family history (similar genetic mutation in 3 female members of the same family, including 1-asymtomatic) and combination of face, tongue, larynx and abdominal angioedema in patient and her sibling.

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