Results 61 to 70 of about 144,374 (198)
Psychological Correlates in Subjects with Hereditary Angioedema (HAE)
Objectives: Hereditary Angioedema (HAE) is a rare serious medical condition caused by a deficiency of C1-inhibitor, due to mutations in its structural gene. The disease appears clinically as cutaneous swelling of the extremities, face, genitals, and trunk, painful swelling of the gastrointestinal mucosa and life threatening laryngeal edema.
openaire +1 more source
Background Hereditary angioedema with normal C1-inhibitor (HAE-nC1-INH) is a rare genetic disease. The symptoms can resemble other forms of hereditary angioedema (HAE), but the specific laboratory values are inconspicuous.
Robin Lochbaum +4 more
doaj +1 more source
Fresh Frozen Plasma in Hereditary Angioedema Crisis: To Give or Not To Give?
Objectives: Fresh frozen plasma (FFP) has been used in angioedema crises, however there is a risk of aggravating the symptoms as well as transmitting infections. In this report, the authors emphasize the dangers of this therapy. Materials and methods: A
Jamal Belkhouribchia, Sara Nguyen
doaj +1 more source
Switching Long‐Term Prophylaxis to Donidalorsen for Hereditary Angioedema: 1‐Year OASISplus Results
This study evaluated the long‐term safety and efficacy of donidalorsen in patients who switched from a long‐term prophylactic treatment (LTP) to donidalorsen with 1‐year outcomes. Patients who switched from LTP to donidalorsen experienced a 67.6% reduction in HAE attack rates over 52 weeks.
Marc A. Riedl +16 more
wiley +1 more source
Patient‐derived expanded potential stem cell (EPSC) hepatocytes reveal that pathogenic SERPING1 variants cause distinct cellular defects in hereditary angioedema. While most mutations reduce SERPING1 transcription and C1‐INH secretion, a large deletion induces intracellular C1‐INH retention.
Xueyan Liu +10 more
wiley +1 more source
Guideline for Hereditary Angioedema (HAE) 2010 by the Japanese Association for Complement Research - Secondary Publication [PDF]
This guideline was provided by the Japanese Association for Complement Research targeting clinicians for making an accurate diagnosis of hereditary angioedema (HAE), and for prompt treatment of the HAE patient in Japan.
Matsushita, Misao +37 more
core +1 more source
Ecallantide is a novel treatment for attacks of hereditary angioedema due to C1 inhibitor deficiency
Henriette Farkas, Lilian Varga3rd Department of Internal Medicine, Semmelweis University, Budapest, HungaryAbstract: Hereditary angioedema (HAE) resulting from the deficiency of the C1 inhibitor protein is a rare disease, characterized by paroxysms of ...
null Varga +3 more
core +1 more source
Hereditary angioedema with normal C1-inhibitor levels: a rare case report
Hereditary angioedema (HAE) is a rare, hereditary disease and its manifestations may be life-threatening. It differs from histaminergic angioedema since it shows different underlying mechanisms and, therefore, does not respond to antihistamine or ...
Filipa Rodrigues dos Santos +2 more
doaj +1 more source
Hereditary angioedema (HAE) is a rare genetic disorder, characterized by recurrent and unexpected potentially life-threatening mucosal swelling. The impairment underlying HAE could be a defect in C1-inhibitor activity, or in its serum concentration ...
Stefania Nicola +2 more
doaj +1 more source
Garadacimab for the long‐term prophylaxis of hereditary angioedema
Summary Hereditary angioedema (HAE), a rare and debilitating disease characterized by recurrent and spontaneous attacks of tissue swelling, has a high unmet therapeutic need, with many patients experiencing insufficient disease control with current prophylactic treatments.
Emel Aygören‐Pürsün +5 more
wiley +1 more source

