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F12-related hereditary angioedema with normal C1Inh

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Disease definition

A rare hereditary angioedema characterized by normal serum levels and function of C1 inhibitor, normal C1 activity, and, clinically, recurrent subcutaneous edema, abdominal pain attacks, and episodes of potentially life-threatening upper airway obstruction. The disorder occurs almost exclusively in women, and episodes are often precipitated or worsened by high estrogen levels (such as during pregnancy or treatment with oral contraceptives).

ORPHA:100054

Classification level: Subtype of disorder

Synonym(s):
  • Inherited estrogen-associated angioneurotic edema
  • Inherited estrogen-dependent angioedema
  • Inherited estrogen-dependent angioneurotic edema
  • HAE 3
  • HAE-III
  • Hereditary angioneurotic edema type 3
  • Inherited estrogen-associated angioedema
  • Hereditary angioedema type 3
  • F12-related HAE with normal C1 inhibitor

Source: PubMed ID 29128335

Prevalence: Unknown

Inheritance: Autosomal dominant

Age of onset: Adult

ICD-10: T78.3

ICD-11: 4A00.14

OMIM: 610618

UMLS: C1857728

MeSH: D056828

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands, Polski Ελληνικά
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2022) - Socialstyrelsen
Guidelines
Emergency guidelines
Français (2021.pdf) - Orphanet Urgences
Polski (2009.pdf) - Orphanet Urgences
Deutsch (2009.pdf) - Orphanet Urgences
English (2009.pdf) - Orphanet Urgences
Español (2017.pdf) - Orphanet Urgences
Italiano (2009.pdf) - Orphanet Urgences
Português (2009.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2010) - Allergy Asthma Clin Immunol
Diagnostic Keys
Français (2024) - Les clés du diagnostic Logo FSMR
Disability
Disability factsheet
Genetic testing
Guidance for genetic testing
English (2019) - Eur J Hum Genet
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