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Angioedema due to acquired C1-inhibitor deficiency without hematological condition: a multicenter French cohort study of 34 patients.
J Allergy Clin Immunol Pract
January 2025
Sorbonne Université, service de médecine interne, AP-HP, Hôpital Saint Antoine, F-75012 Paris, France. Electronic address:
Background: Angioedema (AE) due to acquired C1-inhibitor deficiency (AAE-C1-INH) is a rare disease associating recurrent edema of mucosa and skin. Several underlying diseases have been reported, mainly lymphoproliferative diseases and monoclonal gammopathy. However, 15 to 20% of patients never exhibit such a hematological condition.
View Article and Find Full Text PDFBackground: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterisation of the AAE-C1-INH cohort in UK is required to inform management.
Objectives: To describe the disease burden of AAE-C1-INH, long-term prophylaxis (LTP) and the clinical, immunochemical and treatment profiles of AAE-associated diseases in UK.
Angioedema.
Allergy Asthma Clin Immunol
December 2024
Division of Allergy and Immunology, Department of Medicine, Western University, London, ON, Canada.
Angioedema can occur in the absence of urticaria and can be broadly divided into three main categories: mast cell-mediated (e.g., histamine), non-mast-cell-mediated (bradykinin-induced) and idiopathic angioedema.
View Article and Find Full Text PDFThe international HAE guideline under real-life conditions: From possibilities to limits in daily life - current real-world data of 8 German angioedema centers.
Allergol Select
November 2024
Department of Oto-Rhino-Laryngology. Head and Neck Surgery, Ulm University Medical Center, Ulm.
Background And Objectives: Patients with rare diseases like hereditary angioedema (HAE) are usually referred to an angioedema center to ensure guideline-compliant and experience-based therapy. Even though there are established guidelines and several approved therapeutics, there are still open questions and situations in the daily care of HAE patients, where an exchange between centers is needed.
Materials And Methods: A survey was conducted among physicians from German angioedema centers regarding challenges and issues in everyday HAE treatment.
Article Synopsis
- - Systemic lupus erythematosus (SLE) can manifest in various ways, making it hard to diagnose; this case involved a 23-year-old woman who presented with severe swelling and respiratory issues, which were later identified as symptoms of SLE.
- - Initial treatment involved high-dose steroids and pulse therapy with methylprednisolone; however, the patient developed sepsis and an SLE flare due to an infection at the tracheostomy site.
- - After broad-spectrum antibiotics and a gradual reduction of steroids, along with maintenance therapy with azathioprine, the patient's condition improved, leading to the successful reversal of the tracheostomy and her discharge from the hospital.
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