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Clin Exp Med ; 18(3): 355-361, 2018 Aug.
Article in English | MEDLINE | ID: mdl-29623491

ABSTRACT

Hereditary angioedema (HAE) is a rare autosomic-dominant disorder characterized by a deficiency of C1 esterase inhibitor which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways that are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis during attack and remission state and n = 19 healthy subjects. The samples were tested for a panel of IL (Interleukin)-17-type cytokines (IL-1ß, IL-6, IL-10, granulocyte-macrophage colony stimulating factor (GM-CSF), IL-17, IL-21, IL-22, IL-23) and transforming growth factor-beta (TGF-ß) subtypes. Data indicate that there are variations of cytokine levels in HAE subjects comparing the condition during the crisis respect to the value in the remission phase, in particular type 17 signature cytokines are increased, whereas IL-23 is unmodified and TGF-ß3 is significantly reduced. When comparing healthy and HAE subjects in the remission state, we found a significant difference for IL-17, GM-CSF, IL-21, TGF-ß1 and TGF-ß2 cytokines. These results confirm and extend our previous findings indicating that in HAE there is operating an inflammatory activation process, which involves also T helper 17 (Th17) cytokines and TGF-ß isoforms, associated with localized angioedema attacks and characterized by elevated bradykinin levels.


Subject(s)
Angioedemas, Hereditary/diagnosis , Angioedemas, Hereditary/immunology , Gene Expression Regulation/immunology , Interleukin-17/immunology , Th17 Cells/immunology , Transforming Growth Factor beta/immunology , Adolescent , Adult , Aged , Angioedemas, Hereditary/genetics , Angioedemas, Hereditary/pathology , Bradykinin/genetics , Bradykinin/immunology , Bronchi/immunology , Bronchi/pathology , Case-Control Studies , Child , Complement C1 Inhibitor Protein/genetics , Complement C1 Inhibitor Protein/immunology , Female , Granulocyte-Macrophage Colony-Stimulating Factor/genetics , Granulocyte-Macrophage Colony-Stimulating Factor/immunology , Humans , Interleukin-17/genetics , Interleukin-23/genetics , Interleukin-23/immunology , Interleukins/genetics , Interleukins/immunology , Intestines/immunology , Intestines/pathology , Male , Middle Aged , Subcutaneous Tissue/immunology , Subcutaneous Tissue/pathology , Th17 Cells/pathology , Transforming Growth Factor beta/genetics , Interleukin-22
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