Coincidence of hereditary angioedema (HAE) with Crohn's Disease
Immunological Investigations, 1999A patient with two diseases, based presumably on different immunopathological mechanisms, hereditary angioedema (HAE) and Crohn's disease, was followed for 8 years. For more than three years of this observation period, detailed laboratory data were also available and could be analyzed.
H, Farkas +10 more
openaire +2 more sources
New Instrument for the Evaluation of Prodromes and Attacks of Hereditary Angioedema (HAE-EPA)
Clinical Reviews in Allergy & Immunology, 2021A disease-specific, patient-reported outcome instrument suitable for evaluation of prodromes and attacks of hereditary angioedema (HAE) is a clinical unmet need. We constructed such instrument and examined its validity, acceptability, and discriminative ability.
Iris Leibovich-Nassi +4 more
openaire +2 more sources
Urine-histamine levels in patients with hereditary angioedema (HAE)
Journal of Allergy and Clinical Immunology, 1988Hereditary angioedema (HAE) is defined clinically by recurrent, self-limited episodes of angioedema. The disease is defined biochemically by a deficiency in the functional activity of C1 esterase inhibitor. To date, the actual serum or tissue mediator(s) responsible for the angioedematous lesion remains controversial. Although antihistaminics have been
C M, Brickman, M M, Frank, M, Kaliner
openaire +2 more sources
Hereditary angioedema: Pathophysiology (HAE type I, HAE type II, and HAE nC1-INH)
Allergy and Asthma Proceedings, 2020The pathophysiology of hereditary angioedema (HAE) in virtually all cases is the result of the uncontrolled production of the vasoactive peptide bradykinin. C1 inhibitor (C1-INH) is a serine protease inhibitor, which, under normal circumstances, is the regulator of critical enzymes that are active in the cascades that result in bradykinin generation ...
openaire +2 more sources
Icatibant for laryngeal hereditary angioedema (HAE) attacks
2014Objective: The rare autosomal dominant disease HAE manifests as attacks of cutaneous or submucosal oedema. Laryngeal attacks can cause asphyxiation and may require emergency care and intubation. We analysed (posthoc) laryngeal attacks treated with icatibant (Firazyr®, Shire, Eysins, Switzerland)[for full text, please go to the a.m. URL]
Bas, M +7 more
openaire +2 more sources
A Decade of Change: Recent Developments in Pharmacotherapy of Hereditary Angioedema (HAE)
Clinical Reviews in Allergy & Immunology, 2016Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH) is a rare but medically significant disease that can be associated with considerable morbidity and mortality. Research into the pathogenesis of HAE-C1-INH has expanded greatly in the last six decades and has led to new clinical trials with novel therapeutic agents
openaire +2 more sources
Diagnosis and management of hereditary angioedema (HAE).
Annals of allergy, 1979In summary, HAE is a dominantly inherited form of angioedema which is manifested by nonpainful, nonerythematous, nonpruritic and nonpitting swelling of the extremities, face, gastrointestinal and respiratory tracts unaccompanied by urticaria. These patients have deficient activity of the C1 INH and the laboratory diagnosis can be easily made by finding
openaire +1 more source
M099 WORSENING ANGIOEDEMA IN A PATIENT WITH A DIAGNOSIS OF HEREDITARY ANGIOEDEMA (HAE)
Annals of Allergy, Asthma & Immunology, 2021M. Kim, N. Mambetsariev, L. Grammer
openaire +1 more source
Icatibant in the Treatment of Acute Attacks of Hereditary Angioedema (HAE) in Adults
Clinical Medicine Reviews in Vascular Health, 2011Introduction: Hereditary Angioedema (HAE) is a potentially life-threatening condition consisting of recurrent episodes of limb, abdominal, genital, facial or laryngeal swelling. Unregulated bradykinin activity is known to be the main driving mechanism for the characteristic edema flares in HAE.
openaire +1 more source
Complement, coagulation and fibrinolytic parameters in hereditary angioedema (HAE).
Clinical and experimental immunology, 1983The intrinsic clotting, the kinin generating and the fibrinolytic systems were investigated in 10 patients with hereditary angioedema (HAE), 10 patients with chronic urticaria and 18 healthy volunteers. In spite of the fact that patients suffering from HAE severely lack C1 INH, neither the intrinsic coagulation nor the fibrinolytic systems are impaired.
W, Cullmann +3 more
openaire +1 more source

