Hereditary angioedema: approaches to diagnosis and treatment, analysis of a clinical family case
Hereditary angioedema belongs to the group of rare, orphan, genetically determined defects that represent a significant medical and social problem due to the pronounced impact on the quality of life and potential mortality, as well as the emerging ...
O. V. Skorohodkina +3 more
doaj +1 more source
The hereditary angioedema burden of illness study in Europe (HAE-BOIS-Europe) : background and methodology [PDF]
Background: Hereditary angioedema (HAE) is a rare but serious disease marked by swelling attacks in the extremities, face, trunk, airway, or abdominal areas that can be spontaneous or the result of trauma and other triggers.
Aygören-Pürsün, Emel +7 more
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Lymphoproliferative disease and acquired C1 inhibitor deficiency
Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal ...
Roberto Castelli +4 more
doaj +1 more source
Paths reunited: initiation of the classical and lectin pathways of complement activation [PDF]
Understanding the structural organisation and mode of action of the initiating complex of the classical pathway of complement activation (C1) has been a central goal in complement biology since its isolation almost 50 years ago.
Keeble, Anthony H. +4 more
core +1 more source
Plasma complement biomarkers distinguish multiple sclerosis and neuromyelitis optica spectrum disorder [PDF]
Background: Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are autoimmune demyelinating diseases distinguished clinically by selective involvement in NMOSD of optic nerves and spinal cord. Early clinical manifestations are
Evans, David R. S. +6 more
core +1 more source
Amniotic fluid embolism pathophysiology suggests the new diagnostic armamentarium: β-tryptase and complement fractions C3-C4 are the indispensable working tools [PDF]
Amniotic fluid embolism (AFE) is an uncommon obstetric condition involving pregnant women during labor or in the initial stages after delivery. Its incidence is estimated to be around 5.5 cases per 100,000 deliveries.
Busardo', FRANCESCO PAOLO +3 more
core +2 more sources
Antimicrobial peptides and complement in neonatal hypoxia-ischemia induced brain damage [PDF]
Hypoxic-ischemic encephalopathy (HIE) is a clinical condition in the neonate, resulting from oxygen deprivation around the time of birth. HIE affects 1-5/1000 live births worldwide and is associated with the development of neurological deficits ...
Hristova, M, Rocha-Ferreira, E
core +1 more source
The authors wish to make corrections to their article: Nicola S, Rolla G, Brussino L. Breakthroughs in hereditary angioedema management: a systematic review of approved drugs and those under research. Drugs in Context 2019; 8: 212605. DOI: 10.7573/dic.
Stefania Nicola +2 more
doaj +1 more source
Background. Hereditary angioedema with C1 inhibitor deficiency (HAE-C1INH) is caused by dysfunctional C1-INH protein due to mutations in the SERPING1 gene encoding C1-INH.
Ezgi Topyıldız +4 more
doaj +1 more source
Secreted phospholipases A2 in hereditary angioedema with C1-inhibitor deficiency [PDF]
BackgroundHereditary angioedema (HAE) caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein (C1-INH-HAE) is a disabling, potentially fatal condition characterized by recurrent episodes of swelling. We have recently found that
Andrea Zanichelli +16 more
core +3 more sources

