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Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review.

Miranda AR, Ue AP, Sabbag DV, Furlani Wde J, Souza PK, Rotta O - An Bras Dermatol (2013 Jul-Aug)

Bottom Line: The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy.The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms.A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

View Article: PubMed Central - PubMed

Affiliation: Federal University of São Paulo, EPM, São PauloSPBrazil.

ABSTRACT
In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

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Algorithm of diagnosis of hereditary angioedema (HAE)
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f03: Algorithm of diagnosis of hereditary angioedema (HAE)

Mentions: Laboratory tests are required for the diagnosis of HAE when there is a clinicalsuspicion and / or family history of angioedema. The serum C4 and INHC1 dosages arerecommended (quantitative and qualitative). The C4 and INHC1 should also be evaluated incrises when the results are normal. There is no indication of dosing CH50 orC3.14Figure 3 shows the algorithm diagnosis of HAE. Ifboth the C4 and the quantitative INCH1 are low, then the diagnosis is compatible withType I HAE. If the C4 is below normal and the quantitative INCH1 is normal, it issuggested the dosage of qualitative INHC1; if it's low, the diagnosis is compatible withHAE type II. All tests should be repeated on another occasion to confirm thediagnosis.14If both the C4 andthe quantitative INCH1 are normal, types I and II can be excluded and the hypothesis ofHAE type III should be considered. In this case, the repetition of the tests during theangioedema crisis is always recommended.14


Hereditary angioedema type III (estrogen-dependent) report of three cases and literature review.

Miranda AR, Ue AP, Sabbag DV, Furlani Wde J, Souza PK, Rotta O - An Bras Dermatol (2013 Jul-Aug)

Algorithm of diagnosis of hereditary angioedema (HAE)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3760933&req=5

f03: Algorithm of diagnosis of hereditary angioedema (HAE)
Mentions: Laboratory tests are required for the diagnosis of HAE when there is a clinicalsuspicion and / or family history of angioedema. The serum C4 and INHC1 dosages arerecommended (quantitative and qualitative). The C4 and INHC1 should also be evaluated incrises when the results are normal. There is no indication of dosing CH50 orC3.14Figure 3 shows the algorithm diagnosis of HAE. Ifboth the C4 and the quantitative INCH1 are low, then the diagnosis is compatible withType I HAE. If the C4 is below normal and the quantitative INCH1 is normal, it issuggested the dosage of qualitative INHC1; if it's low, the diagnosis is compatible withHAE type II. All tests should be repeated on another occasion to confirm thediagnosis.14If both the C4 andthe quantitative INCH1 are normal, types I and II can be excluded and the hypothesis ofHAE type III should be considered. In this case, the repetition of the tests during theangioedema crisis is always recommended.14

Bottom Line: The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy.The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms.A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

View Article: PubMed Central - PubMed

Affiliation: Federal University of São Paulo, EPM, São PauloSPBrazil.

ABSTRACT
In this article, three cases of hereditary angioedema (HAE) type III (estrogen-dependent or with normal C1 inhibitor) are reported. The HAE was initially described in women of the same family in association with high-leveled estrogenic conditions such as the use of oral contraceptives and pregnancy. There is no change in the C1 inhibitor as happens in other types of hereditary angioedema, and mutations are observed in the encoding gene of the XII factor of coagulation in several patients. The current diagnosis is mainly clinical and treatment consists in the suspension of the triggering factors and control of acute symptoms. A brief review of physiopathology, clinical features, genetic alterations and treatment are also presented.

Show MeSH
Related in: MedlinePlus