731 results match your criteria: "Angioedema Acquired"

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.

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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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Hereditary angioedema (HAE) in children and adolescents: New treatment options.

Allergol Select

October 2024

Hannover Medical School (MHH), Department of Dermatology, Allergology and Venereology, Interdisciplinary Allergy Center of the MHH, Treatment Center for Hereditary Angioedema of the MHH, Hannover, Germany.

Article Synopsis
  • Modern management of hereditary angioedema (HAE) emphasizes personalized treatment plans tailored to the needs of children and adolescents, as well as the severity of the disease.
  • New medications have improved quality of life for HAE patients, but not all are approved for children, leading to ongoing research into alternative treatments such as those targeting bradykinin effects.
  • Emerging options like oral medications, long-acting therapies, and potential gene therapy are being explored to better meet the care requirements for young patients with HAE type I and II.
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Angioedema is a health issue that affects parts of the body like the upper pulmonary and gastric pathways and is identified by abrupt, nonpitting enlargement of the skin, mucous membranes, or both. The swelling usually lasts a few hours to 72 hours and may appear as non-puritic, subcutaneous, or submucosal organ edema. It is characterized by localized swelling brought on by the release of histamine.

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  • MGUS-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) hasn't been specifically characterized before, prompting this study to explore its biological and clinical features over 30 years in France.
  • In a study of 41 patients, most had anti-C1INH antibodies, and treatments included acute management and long-term prophylaxis, with a significant number developing malignant blood disorders like lymphoma or myeloma.
  • The study found a link between the remission of angioedema and the underlying hematological malignancy, emphasizing the need for regular hematological evaluations in patients with MGUS-AE-C1-INH.
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  • - AAE-C1-INH is a rare condition resulting from low levels of the C1 esterase inhibitor, leading to episodes of swelling in various body parts, including the genitals and extremities.
  • - The case report details a 79-year-old man who experienced painless swelling and was evaluated in the emergency department, showing lab results indicative of AAE-C1-INH without any underlying lymphoreticular or rheumatic diseases.
  • - Accurate diagnosis of AAE-C1-INH is crucial to prevent serious complications like airway blockage, ensure appropriate treatment, and rule out other possible health issues.
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Daratumumab-based treatment of monoclonal gammopathy-associated angioedema due to acquired C1-inhibitor deficiency.

J Allergy Clin Immunol Glob

November 2024

Department of Vascular Medicine, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

Article Synopsis
  • - Daratumumab, a targeted therapy, shows promise for managing severe angioedema that doesn't respond to standard treatments.
  • - This condition is linked to a deficiency in the C1-inhibitor protein, often seen in patients with monoclonal gammopathy.
  • - The treatment could offer relief and improve the quality of life for patients facing this serious health issue.
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  • - Acquired angioedema (AAE) is a rare condition that can cause severe abdominal pain and is often misdiagnosed in emergency rooms, potentially leading to unnecessary treatments.
  • - A case study of a 47-year-old man revealed that initial suspicion of ACEI-induced angioedema was incorrect; instead, tests showed AAE with abnormal C1 inhibitor function and low complement levels.
  • - Recognizing AAE early in patients with acute abdominal symptoms is crucial, as it can indicate underlying serious conditions and lead to better treatment outcomes.
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Article Synopsis
  • Angioedema (AE) is a condition characterized by localized swelling in tissues, and this study aimed to analyze the traits of patients with recurring AE that does not include wheals.
  • The research involved 143 adult patients and documented their demographics and laboratory results from outpatient visits between August 2018 and August 2020, with follow-ups occurring between October 2023 and January 2024 to check for remission.
  • Findings revealed variations in phenotypic characteristics among different AE subtypes, with notable differences in patients' medical histories and remission rates linked to specific triggers and underlying causes of AE.
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  • Hereditary angioedema (HAE) is a rare genetic disorder causing episodes of swelling, particularly due to an increase in a substance called bradykinin, especially in patients with mutations in the F12 gene that affect C1 inhibitor activity.
  • A study comparing 40 patients with FXII-HAE to 40 healthy individuals found increased plasma levels of specific lipid mediators and enzymes, indicating an altered biochemical response in those with the condition.
  • The findings suggest that the overproduction of bradykinin impacts certain pathways in FXII-HAE, opening up potential avenues for further research on the role of these lipid mediators in the disease.
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  • Hereditary angioedema (HAE) causes serious swelling in different parts of the body, but new treatments help reduce attacks and improve patients' lives.
  • A survey with patients in six countries looked at how the time without attacks relates to their quality of life.
  • The results showed that being attack-free for longer periods made people feel better and needed less rescue medication.
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  • Chronic histaminergic angioedema (CHA) and angioedema associated with chronic spontaneous urticaria (AE-CSU) were found to be distinct conditions, despite some similarities in symptoms.
  • An observational study compared 56 CHA patients with 40 AE-CSU patients, revealing that AE-CSU had a higher frequency of attacks and different affected areas.
  • A predictive model successfully differentiated the two conditions with high accuracy, reinforcing the notion that CHA and AE-CSU are independent pathologies.
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  • Corticosteroids have been widely used in critical care for various conditions like respiratory distress, septic shock, and COVID-19, showing both benefits and risks.
  • Key advantages include improved treatment outcomes, shorter hospital stays, and lower mortality rates, while significant adverse effects include hyperglycemia, hypertension, and increased infection risk.
  • There is currently no thorough report on corticosteroid use in critical care, highlighting the need for better understanding and management of their use in therapy.
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Quantification of C1 inhibitor activity using a chromogenic automated assay: analytical and clinical performances.

Clin Chem Lab Med

November 2024

Immunology Department Laboratory, Referral Medical Biology Laboratory, 36760 Institut Fédératif de Biologie, Toulouse University Hospital Center, Toulouse, France.

Article Synopsis
  • The study aimed to evaluate the performance of a chromogenic assay (Berichrom) for quantifying functional C1 inhibitor activity (fC1-INH), which is crucial in diagnosing bradykinin-mediated angioedema.
  • The assay demonstrated excellent precision and stability under various conditions, showing no significant interference from common substances.
  • Results indicated that the Berichrom assay agreed well with a manual method and provided high diagnostic accuracy, with 100% sensitivity and 97.2% specificity for assessing patients with angioedema.
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  • Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease that causes swelling and doesn't respond well to common medications like antihistamines.
  • The study looked at 26 patients with InH-AAE to understand their symptoms and possible markers of the disease, collecting a lot of health-related information.
  • Findings included changes in specific proteins in the blood and differences in blood vessel shape, helping to improve the understanding of InH-AAE and possibly leading to better treatments in the future.
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  • Lanadelumab is a medicine used to help people with hereditary angioedema (HAE) and has been studied over 4 years for how well it works and if it's safe.
  • Out of 34 patients treated with lanadelumab, most were still doing well after 4 years, with many reporting fewer attacks and feeling satisfied with their treatment.
  • The study showed that lanadelumab is effective and safe in real life, and it suggests that patients may not need extra short-term treatments when using it.
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Extremely Late Diagnosis of Hereditary Angioedema Type I in an Elderly Female.

Cureus

March 2024

Allergy and Immunology, Aloha Allergy and Immunology, Honolulu, USA.

Article Synopsis
  • The case chronicles the delayed diagnosis of hereditary angioedema (HAE) type I in an elderly woman who had recurrent lip swelling and itchiness since her teenage years, leading to frequent ER visits.
  • Misdiagnosis as a hypersensitivity reaction, due to her environmental allergies, complicated her condition and delayed proper treatment.
  • After extensive testing, she was diagnosed with HAE type I, treated successfully with C1 esterase inhibitor therapy, which reduced the frequency of her symptoms and ER visits.
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Definition, acronyms, nomenclature, and classification of angioedema (DANCE): AAAAI, ACAAI, ACARE, and APAAACI DANCE consensus.

J Allergy Clin Immunol

August 2024

Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Institute for Translational Medicine, and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.

Article Synopsis
  • Angioedema (AE) is a condition characterized by localized swelling in the skin or mucous membranes and can be hereditary or acquired, making its classification complex due to various underlying mechanisms and taxonomies.
  • The DANCE initiative, involving 91 experts from 35 countries, aimed to create a unified consensus on the definition, acronyms, and classification of AE through an extensive online discussion and voting process over 16 months.
  • The resulting DANCE classification introduces five types of AE, standardizes terminology, and is designed to enhance research and patient care while complementing existing clinical guidelines without replacing them.
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  • Antiphospholipid syndrome (APS) involves blood clotting issues and pregnancy complications linked to antiphospholipid antibodies and is a common acquired disorder affecting clotting.
  • While strokes can occur in APS patients, there is limited data on the use of thrombolytic treatment (clot-busting drugs) and its safety during such events.
  • A clinical case is presented where thrombolysis was successfully used on an APS stroke patient without complications, highlighting the neurological symptoms associated with APS and stroke.
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  • Hereditary angioedema (HAE) is difficult to diagnose due to its variable symptoms and many overlapping conditions, especially in cases with normal C1-inhibitor levels (nC1-INH HAE).
  • A study found that traditional genetic testing was only successful in identifying pathogenic variants in a small number of HAE patients, urging the use of comprehensive sequencing methods like genome, exome, and transcriptome analysis.
  • Despite various genetic investigations, the diagnostic success for nC1-INH HAE was low, highlighting the necessity for ongoing clinical assessments and further research to better understand the complexities of this condition.
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Deucrictibant for angioedema due to acquired C1-inhibitor deficiency: A randomized-controlled trial.

J Allergy Clin Immunol

July 2024

Amsterdam University Medical Center, Department of Vascular Medicine, Amsterdam, and Cardiovascular Sciences, University of Amsterdam, Amsterdam, The Netherlands. Electronic address:

Article Synopsis
  • Angioedema from acquired C1-inhibitor deficiency is a rare condition (1 in 500,000) with no current approved treatments, making research into new options like deucrictibant crucial.
  • A study was conducted in two parts to evaluate deucrictibant's effectiveness, with results showing reduced attack severity and prevention of attacks during treatment.
  • Findings suggest deucrictibant is a promising candidate for treating and preventing angioedema attacks, with minimal side effects reported.
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