Hereditary angioedema is a rare immunologic disorder, and immunoglobulin A deficiency is the most common isolated immunodeficiency. These rarely coexisting conditions may affect pregnancy. We report a gravida with the combination who developed severe acute laryngeal edema.
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Background: 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.
[Hereditary angioedema type 1, limitations for prophylactic therapy with Lanadelumab: Regarding a case].
Rev Alerg Mex
December 2024
Médica general, Facultad de Ciencias de la Salud, Universidad Militar Nueva Granada, Hospital Universitario Mayor Méderi, Colombia.
Article Synopsis
- Hereditary Angioedema (HAE) is a rare autosomal dominant disorder characterized by low levels or dysfunctional C1 inhibitor, leading to recurrent episodes of swelling and abdominal pain.
- A reported case involves a 53-year-old man with a longstanding history of recurrent edema and family history, who showed low levels of plasma proteins but faced treatment challenges despite various therapies, including a trial of Lanadelumab for prophylaxis.
- The patient's experience highlights issues in managing HAE, revealing an adverse reaction to Lanadelumab that is not commonly documented, emphasizing the need for better-informed treatment strategies.
Idiopathic non-mast cell angioedema: Treatment insights from global experts.
Allergy Asthma Proc
January 2025
From the Division of Allergy and Immunology, Department of Medicine, University of California San Diego, La Jolla, California and.
Idiopathic non-mast cell angioedema (INMA) is a rare disease typified by recurrent attacks of cutaneous and subcutaneous swelling. Every attack carries the potential for severe morbidity and, in the case of laryngeal involvement, mortality. Whereas therapies approved for hereditary angioedema (HAE) have been used in the care of patients with INMA, little is known with regard to their efficacy for the treatment of this disease.
View Article and Find Full Text PDFManaging Hereditary Angioedema in a Three-Generation Family: Danazol's Promise in Resource-Limited Settings.
Cureus
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Department of Pediatrics, Mahatma Gandhi Mission (MGM) Medical College and Hospital, Aurangabad, IND.
Background Hereditary angioedema (HAE) is a rare disorder in India, and while prevalence data is limited, it is believed that a significant number of individuals may be affected. Due to restricted access to first-line treatments, older therapies like danazol are commonly used despite associated risks in resource-constrained settings. This study aimed to assess the efficacy of danazol as an affordable long-term prophylaxis (LTP) for HAE in a three-generation family.
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Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, KU Leuven, Leuven, Belgium. Electronic address:
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