Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients.

doi:10.1097/00005792-199207000-00003

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Article

Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients.

Medicine - 1 Jul 1992

Agostoni A, Cicardi M

Abstract excerpt

Two hundred and twenty-six patients with inherited C1 inhibitor (C1-INH) deficiency, also known as hereditary angioedema (HAE), have been studied. They belonged to 80 unrelated families, and in 11 of them C1-INH was functionally deficient but antigenically normal (type II HAE). Genetic analysis of type 1 families demonstrated restriction fragment length polymorphisms in 11% and abnormal mRNAs in 25%. In type II...

Topics

Adolescent
Adult
Aged
Aged, 80 and over
Angioedema
Autoimmune Diseases
Causality
Child
Complement C1 Inactivator Proteins
Danazol
Female
Follow-Up Studies

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Genetic Diseases, Inborn

Hospitals, University

Humans

Italy

Male

Middle Aged

Source

Open in PubMed

Identifiers

PMID: 1518394
DOI: 10.1097/00005792-199207000-00003

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-1518394-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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