Case Of Angioedema Research Articles

Unveiling the Complexities of Hereditary Angioedema

Hereditary angioedema (HAE) is a rare and potentially life-threatening genetic disorder, constituting approximately 2% of all clinical cases of angioedema, with a global prevalence estimated between 1 in 50,000 and 1 in 150,000 individuals. The condition affects individuals of all genders and ethnic backgrounds without significant variation. HAE is classified into three types. Type I HAE, which accounts for 85% of cases, is characterized by a deficiency of the C1 esterase inhibitor (C1-INH) gene. Type II HAE, making up 15% of cases, involves a dysfunctional C1-INH. Type III HAE, which represents about 5% to 10% of cases, is often estrogen-dependent and although several mutations have been identified, it typically involves normal C1-INH activity. Despite the differences in C1-INH functionality, all three types of HAE manifest with similar clinical symptoms. HAE leads to recurrent episodes of non-pruritic angioedema, which occurs in the absence of urticaria. Breakthroughs in understanding HAE pathophysiology have revolutionized treatment, leading to the development of highly targeted therapies for both acute management and long-term prevention. Meanwhile, cutting-edge advancements in omics technologies are unlocking new possibilities for biomarker discovery, paving the way for more precise diagnoses and personalized treatment strategies that could significantly enhance patient outcomes. This review will delve into the intricate pathophysiology, diverse clinical presentations, and diagnostic challenges of HAE while exploring emerging biomarkers and innovative approaches to therapeutic management and prevention strategies. Additionally, it will underscore the vital importance of screening family members of affected individuals, even when symptoms are not present.

Risperidone-induced angioedema

Abstract Risperidone is a second-generation antipsychotic with common adverse effects, such as extrapyramidal symptoms, weight gain, hyperprolactinemia, and sedation. Angioedema, although generally considered to be uncommon, has previously been documented to occur following administration of some antipsychotics, including risperidone. This report describes a case of risperidone-induced angioedema in an older male patient.

Assessment of emergency physicians’ awareness and knowledge of hereditary angioedema

Aims: Hereditary angioedema can occur with life-threatening attacks of severe laryngeal edema, and epinephrine is insufficient in the treatment of attacks. We sought an answer to the question, 'Do emergency physicians, who frequently encounter angioedema cases that are so important for the emergency department, have sufficient awareness about this issue?' Methods: In this study, the online questionnaire was conducted among physicians working in adult emergency departments between April and August 2022. The questionnaire form consisted of two parts. The first part contained three questions about medical experience, academic degree, and encounter with hereditary angioedema patients. The second part of the questionnaire contained seven questions about the diagnosis and treatment of hereditary angioedema. Results: A total of 103 physicians working in emergency departments participated in the survey. The proportion of physicians is as follows: The percentage of physicians with less than 15 years of experience was 92.2%. Research assistants represented the largest group of participants at 51.5%. When asked "What is not a symptom of hereditary angioedema?" Only 40.6% of physicians could answer the question correctly. While 39.8% of physicians thougt that epinephrine and antihistamines were useful in treating these attacks, 53.4% felt that epinephrine and antihistamines were not. While there was a difference in hereditary angioedema awareness between the group with more than 15 years of professional experience and the group with less experience, there was no meaningful difference between research assistants, specialists, and academicians. According to this survey, professional experience was important, but academic title did not make a difference in terms of disease awareness. Conclusion: It is necessary to increase the awareness of emergency physicians about hereditary angioedema, which can cause fatal attacks and whose diagnosis and treatment are different from other angioedemas.

Two pediatric hereditary angioedemathe families: case report and literature review

Objective: To summarize the clinical characteristics of two families with hereditary angioedema in children. Methods: A retrospective analysis was conducted on the general information, clinical manifestations, genetic variations, and laboratory test results of two families diagnosed with hereditary angioedema at Department of Rheumatology and Immunology, Shenzhen Children's Hospital from December 2022 to May 2023. And using keywords such as "children" "adolescent" "infant" "toddler" "pediatric" "hereditary angioedema" search for relevant literature from VIP, Wanfang, CNKI, PubMed, Web of Science and Google Scholar from the establishment of the database until January 2024 and summarize the diagnosis and clinical characteristics of hereditary angioed in children. Results: Case 1, female, 12 years old, presented with intermittent abdominal pain for 8 years, aggravated with vomiting for 2 days. Eight years ago, without a clear cause, case 1 experienced abdominal pain and underwent two abdominal surgeries. The concentration of C1 esterase inhibitor was 0.46 g/L, and the function of C1 esterase inhibitor was less than 0.01. The SERPING1 gene had a c.1396C>G variation, and she was diagnosed with hereditary angioedema type Ⅱ. Among the other 13 family members, 8 had swelling in different parts, and 2 had died. Case 2, female, 17 years old, was diagnosed with hereditary angioedema type Ⅰ due to intermittent limb swelling for more than 2 years. She had C4 of 0.09 g/L, C1 esterase inhibitor concentration of 0.05 g/L, C1 esterase inhibitor function <0.01, SERPING1 gene c.882C>G variation. Two out of the other 12 family members experienced intermittent skin swelling. Literature review meets the search criteria with 0 Chinese literature and 15 English literature, including a total of 524 cases of hereditary angioedema in children. Combined with 2 families in this group, there are 5 families with hereditary angioedema in children as the proband. The onset time is 1-15 years old, and the diagnosis delay time is -0.9 to 20.0 years. Two hundred and sixty-three cases (50.2%) of the children had a family history survey, of which 229 cases (87.1%) had a positive family history. Two hundred and fifty-seven cases (49.0%) had clear disease classification, with type Ⅰ being the main type, accounting for 234 cases (91.1%). The clinical symptoms of 296 children (56.5%) were described in detail, including 262 cases (88.5%) with skin edema in different parts, 176 cases (59.5%) with abdominal pain, and 61 cases (20.6%) with upper respiratory tract edema in the throat. Conclusions: Hereditary angioedema is mainly characterized by episodic skin and mucosal swelling, with individualized differences in the locations and characteristics of the onset. Hereditary angioedema in children is often overlooked, and patients with a positive family history of episodic skin and mucosal swelling need to actively undergo complement level testing.

P148 EFFICACY AND SAFETY OF SACUBITRIL/ALLISARTAN FOR THE TREATMENT OF PRIMARY HYPERTENSION: A PHASE 3 RANDOMIZED, DOUBLE-BLIND STUDY

Background: This randomized, double-blind, phase 3 study assessed the efficacy and safety of sacubitril/allisartan, an angiotensin receptor neprilysin inhibitor, compared with olmesartan in Chinese patients with mild to moderate hypertension. Methods: Eligible patients aged 18-75 years (n=1197) with mild to moderate hypertension were randomized to receive sacubitril/allisartan 240 mg (n=399), sacubitril/allisartan 480 mg (n=399), or olmesartan 20 mg (n=399) once daily for 12 weeks. Patients who completed the 12-week treatment then received another 12-week extended treatment (n=1084) and 28-week prolonged treatment (n=189). The primary end point was a reduction in clinic mean sitting systolic blood pressure (msSBP) from baseline with various doses of sacubitril/allisartan versus olmesartan at 12 weeks. Secondary efficacy variables included clinic and 24-h ambulatory blood pressure for the comparison between sacubitril/allisartan and olmesartan at week 12, 24, and 52. Results: Sacubitril/allisartan 240 mg/day provided a greater reduction in msSBP than olmesartan at 12 weeks (between-group difference: -1.9 mmHg [95% confidence interval -4.2 to 0.4 mmHg], P=0.0007, for non-inferiority). Sacubitril/allisartan 480 mg/day provided a significantly greater reduction in msSBP than olmesartan at 12 weeks (between-treatment difference: -5.0 mmHg [95% confidence interval -7.3 to -2.8 mmHg], P&lt;0.001, for superiority). Greater reductions in 24-h, daytime and nighttime systolic and diastolic blood pressure were also observed with both doses of sacubitril/allisartan compared with olmesartan (P≤0.001 for 480 mg/day). The blood pressure reductions tended to be dose-dependent for sacubitril/allisartan. Sacubitril/allisartan was well tolerated, and no cases of angioedema or death were reported. Conclusions: Sacubitril/allisartan is effective for the treatment of hypertension and well tolerated in Chinese patients.

Efficacy and Safety of Sacubitril/Allisartan for the Treatment of Primary Hypertension

BackgroundThe prevalence of hypertension still increases with the very rapidly increasing longevity in some countries, such as China. The control rate remains low. ObjectivesThis randomized, double-blind, phase 3 study assessed the efficacy and safety of sacubitril/allisartan, compared with olmesartan in Chinese patients with mild-to-moderate hypertension. MethodsEligible patients aged 18 to 75 years (n = 1,197) with mild-to-moderate hypertension were randomized to receive sacubitril/allisartan 240 mg (n = 399), sacubitril/allisartan 480 mg (n = 399), or olmesartan 20 mg (n = 399) once daily for 12 weeks. Patients who completed the 12-week treatment then received another 12-week extended treatment (n = 1,084) and 28-week prolonged treatment (n = 189). The primary end point was a reduction in clinic mean sitting systolic blood pressure (msSBP) from baseline at 12 weeks. ResultsSacubitril/allisartan 240 mg/d provided a greater reduction in msSBP than olmesartan at 12 weeks (between-group difference: −1.9 mm Hg [95% CI: −4.2 to 0.4 mm Hg]; P = 0.0007, for noninferiority). Sacubitril/allisartan 480 mg/d provided a significantly greater reduction in msSBP than olmesartan at 12 weeks (between-treatment difference: −5.0 mm Hg [95% CI: −7.3 to −2.8 mm Hg]; P < 0.001, for superiority). Greater reductions in 24-hour, and daytime and nighttime systolic and diastolic blood pressure were also observed with both doses of sacubitril/allisartan compared with olmesartan (P ≤ 0.001 for 480 mg/d). The blood pressure reductions tended to be dose-dependent for sacubitril/allisartan. Sacubitril/allisartan was well tolerated, and no cases of angioedema or death were reported. ConclusionsSacubitril/allisartan is effective for the treatment of hypertension and well tolerated in Chinese patients.

Normalization of C1 Inhibitor in a Patient with Hereditary Angioedema

SummaryHereditary angioedema is a potentially life-threatening autosomal dominant condition, causing attacks of angioedema due to failure to regulate bradykinin. Nearly all cases of hereditary angioedema are caused by mutations in the gene encoding C1 inhibitor, SERPING1. C1 inhibitor is a multifunctional protein produced in the liver that regulates the kallikrein–kinin system at multiple points. An infant with genetically confirmed hereditary angioedema and low C1 inhibitor levels (but without previous episodes of angioedema) underwent liver transplantation for biliary atresia, an unrelated condition. Liver transplantation led to normalization of the C1 inhibitor level and function. To our knowledge, this represents the first patient to be potentially cured of hereditary angioedema.

EFFICACY AND SAFETY OF SACUBITRIL/ALLISARTAN FOR THE TREATMENT OF PRIMARY HYPERTENSION: A PHASE 3 RANDOMIZED, DOUBLE-BLIND STUDY

Objective: This randomized, double-blind, phase 3 study assessed the efficacy and safety of sacubitril/allisartan, an angiotensin receptor neprilysin inhibitor, compared with olmesartan in Chinese patients with mild to moderate hypertension. Design and method: Eligible patients aged 18-75 years (n=1197) with mild to moderate hypertension were randomized to receive sacubitril/allisartan 240 mg (n=399), sacubitril/allisartan 480 mg (n=399), or olmesartan 20 mg (n=399) once daily for 12 weeks. Patients who completed the 12-week treatment then received another 12-week extended treatment (n=1084) and 28-week prolonged treatment (n=189). The primary end point was a reduction in clinic mean sitting systolic blood pressure (msSBP) from baseline with various doses of sacubitril/allisartan versus olmesartan at 12 weeks. Secondary efficacy variables included clinic and 24-h ambulatory blood pressure for the comparison between sacubitril/allisartan and olmesartan at week 12, 24, and 52. Results: Sacubitril/allisartan 240 mg/day provided a greater reduction in msSBP than olmesartan at 12 weeks (between-group difference: -1.9 mmHg [95% confidence interval -4.2 to 0.4 mmHg], P=0.0007, for non-inferiority). Sacubitril/allisartan 480 mg/day provided a significantly greater reduction in msSBP than olmesartan at 12 weeks (between-treatment difference: -5.0 mmHg [95% confidence interval -7.3 to -2.8 mmHg], P&lt;0.001, for superiority). Greater reductions in 24-h, daytime and nighttime systolic and diastolic blood pressure were also observed with both doses of sacubitril/allisartan compared with olmesartan (P&lt;=0.001 for 480 mg/day). The blood pressure reductions tended to be dose-dependent for sacubitril/allisartan. Sacubitril/allisartan was well tolerated, and no cases of angioedema or death were reported. Conclusions: Sacubitril/allisartan is effective for the treatment of hypertension and well tolerated in Chinese patients.

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 adrenaline treatment. The authors present a case of hereditary angioedema to highlight the importance of prompt referral to the allergy and clinical immunology for further evaluation and correct diagnosis in case of suspicion of this entity.

Case Report] Ramipril-Induced Angioedema in a Patient With Basal Ganglia Bleed: A Case Report

This report presents a case of ramipril-induced angioedema in a patient who had undergone surgery for basal ganglia bleed, a complication of uncontrolled hypertension that typically requires long-term antihypertensive treatment. The patient was receiving ramipril, a common hypertension medication, but developed the life-threatening side effect of angioedema. The case highlights the potential risks associated with using ramipril.

A Case of Menopause-Induced Angioedema

A Case of Menopause-Induced Angioedema

From pharmacovigilance to pharmaceutical care: About a case of urticaria and angioedema

The concept of “pharmaceutical care” was developed in 2017 in Algeria to globally define the practice of clinical pharmacy. The EHU Oran pharmacovigilance department has thus benefited from the integration of pharmaceutical care to improve drug management. We report a clinical case of urticaria and angioedema in a 22-year-old female patient in a context of polymedication with non-steroidal anti-inflammatory drugs (NSAIDs). A notification was sent to the pharmacovigilance department to estimate the causality assessment. The aim of our article is to illustrate the integration of pharmaceutical care system with the pharmacovigilance, based on this clinical case.

Acute angioedema in Cape Town emergency centres and a suggested algorithm to simplify and improve management.

Angioedema is the most common acute allergic presentation to emergency centres (EC), with hospitalisation rates increasing in high-income countries. Angioedema can complicate with life-threatening laryngeal obstruction. There are no local data; therefore, we aimed to characterise acute angioedema cases presenting to ECs and develop a simple management algorithm. To characterise the clinical presentation, management and outcomes of acute angioedema cases presenting to ECs. Based on these findings, we developed a management algorithm for acute angioedema to improve the care of acute angioedema in South Africa (SA). We conducted a retrospective folder review of all patients admitted to Groote Schuur Hospital (tertiary) and Mitchells Plain District Hospital (secondary) ECs from 1 June 2018 to 31 June 2020. Using ICD-10 coding, folders of adults ≥18 years with possible angioedema presenting to the ECs were screened. An allergist extracted demographics, medical history, management and outcome data for each angioedema event. A total of 142acute angioedema episodes were included, with a median (interquartile range) age of 42 (28- 58) years, and 62% of patients were female. The majority (124/142, 87%) of acute angioedema EC presentations involved swelling above the shoulders, with airway involvement in 20(14%) patients, with two patients requiring intubation. Nineteen (13%) patients required admission, with five (26%) admitted to high care/intensive care. Drug-induced angioedema was the most common cause, with 64/142 (45%) linked to a known offending drug, 42/64 (65.6%) being angiotensin-converting enzyme inhibitor (ACE-I). Critical information to guide angioedema management, including past personal/family allergy history, and duration of angioedema prior to EC visit, was not recorded in 64.7% and 37.8% of EC records, respectively. Unnecessary treatment with corticosteroids or antihistamines occurred in 19/53 (36%) and 16/53 (30%) cases with bradykinin-mediated angioedema ACE-I angioedema and hereditary angioedema). Overall, only 36/142 (25%) of angioedema patients were connected to allergy care. Angioedema is the most common allergy presentation to two ECs in Cape Town, SA. Bradykinin-mediated angioedema secondary to ACE-I therapy is the single most common offender, and was not appropriately managed in more than a third of cases. Based on these findings, we have developed a management algorithm that easily stratifies patients into bradykinin or mast cell-mediated angioedema with a step-by-step management approach that is applicable to the SA context. Ongoing awareness and education on allergy emergencies are required to ensure accurate diagnosis of less common causes of angioedema (particularly bradykinin-mediated angioedema) and linkage to allergy specialist care.

Orolingual angioedema as a complication of systemic thrombolytic therapy with recombinant tissue plasminogen activator

Orolingual angioedema is a rare, but extremely dangerous complication of systemic thrombolytic therapy with recombinant tissue plasminogen activator, the incidence of which reaches 2.2-5.1 %. Orolingual angioedema is a life-threatening condition, as it can lead to airway obstruction and asphyxia. An analysis of a clinical case of orolingual angioedema as a complication of systemic thrombolytic therapy using a recombinant tissue plasminogen activator is presented, risk factors for its development and treatment methods are presented. Orolingual angioedema is a rare life-threatening complication of systemic thrombolytic therapy with recombinant tissue plasminogen activator. It is necessary to assess the severity of edema every 30-60 minutes after systemic thrombolytic therapy in order not to miss the moment of escalation of the process. For the treatment of orolingual angioedema, antihistamines, glucocorticosteroids and adrenaline are used, in case of progressive edema and impaired airway patency, emergency tracheal intubation is performed, followed by artificial ventilation of the lungs.

Isolated angioedema due to face mask and other cases of isolated angioedema.

Isolated angioedema can be divided into two groups as mast cell-mediated angioedema and bradykinin-mediated angioedema according to the known mechanisms of occurrence. However, angioedema can also occur with mechanisms whose cause is unknown. Treatment varies according to the mechanism of angioedema formation. In this prospective study, we present the causes of 80 isolated angioedema cases admitted to our clinic during the pandemic period. We would like to emphasize the causes that we found in our cases but which are rare in the literature. For example: angioedema due to allergy to nickel in the mask used during the pandemic period, T cell-mediated angioedema, alpha adrenergic receptor blocker use, and patients diagnosed with collagen tissue disease presenting with angioedema as the first clinical finding.

A case of gabapentin-induced angioedema in a 31-year-old female

Angioedema is a known potential side effect of gabapentin; however, not many reports of presentation exist in literature. This report is of a 31-year-old woman who presented with signs of sciatica and was started on gabapentin. When the gabapentin dosage was increased, she developed angioedema of the right side of her face, particularly pronounced in the periorbital region. Because an acute allergic reaction was suspected at first, the patient was treated with epinephrine and Benadryl. However, when the patient did not improve, angioedema was suspected. The patient was subsequently treated with steroids and gabapentin was discontinued

A Rare Case of ACEi-associated Angioedema of the Small Bowel

A middle-aged female presented with abdominal pain, vomiting, and watery non-bloody diarrhea shortly after her lisinopril dose was increased. Extensive workup did not reveal a definite pathology however CT of the abdomen showed bowel wall thickening of the proximal jejunum. Her lisinopril was held at the start of the admission due to an acute kidney injury and hypotension. A colonoscopy was done and biopsies revealed increased intraepithelial lymphocytes at every site which is characteristic of medication induced enteropathy. She was instructed to not restart lisinopril and symptoms completely resolved. She was diagnosed with ACEi-angioedema of small bowel.

942: SUGAR-COATING ANGIOEDEMA: RAPID REDUCTION OF TONGUE SWELLING BY TOPICAL SUCROSE APPLICATION

Introduction: Medication-induced angioedema can result in rapid tongue swelling and airway compromise. Endotracheal intubation is frequently required in severe cases and extubation is attempted only when swelling has resolved. The duration for resolution of tongue swelling is highly variable. Mechanisms to reduce swelling include discontinuation of the offending agent. Here we review three cases of medication-induced angioedema where application of granulated sugar on the tongue was used as a mechanism to rapidly reduce swelling. Description: We describe three patients seen in 2021-22 for angioedema in our ICU. Patient 1 is a 51 y-o woman who was intubated for airway protection for acute ischemic stroke. She had massive swelling of her tongue 2 days after intubation. Offending agent was alteplase. Patient 2 is a 63 y-o man who presented with acute rapidly progressive tongue swelling due to Lisinopril and was intubated. Patient 3 is a 52 y-o woman with type B thoracic aortic dissection and hypertension who developed angioedema 8 hours into esmolol and nicardipine infusions. In addition to standard care for all, the following technique was applied: The tongue was layered with granulated sugar obtained from 8 to 10 small packets by placing it on gauze and wrapping it circumferentially around the tongue. This wrap was kept in place by a petroleum-jelly (Vaseline) gauze. All patient had rapid reduction of tongue swelling. Complete resolution of tongue swelling defined as ability to see lower soft palate, entire uvula and tonsillar pillars as well as ability to close the mouth, was seen in 24 hours for patient 1, 9 hours for patient 2 and 8 hours for patient 3. Patient 1 and 2 were extubated the same day and patient 3 was never intubated. Discussion: Topical application of granulated sugar reduces tissue edema by creating an osmotic gradient and thereby decreasing interstitial fluid. We hypothesized that the principle applicable to reduction of incarcerated rectal prolapse and stoma can be applied to angioedema. Lack of tongue protrusion from the mouth and the ability to visualize the palate may indicate that the risk of airway compromise is markedly reduced. The desiccating effect of sucrose rapidly reduced the duration or need of invasive airway management and may be an effective adjunct for angioedema.

AN EPISODE OF ANGIOEDEMA TRIGGERED BY DRY NEEDLING

<h3>Introduction</h3> Angioedema is a life-threatening disorder caused by extravasation of fluid into subcutaneous tissues often in the head and neck. No prior report describes angioedema after dry needling, a procedure often performed by trained physical therapists in which a thin needle without injectate is inserted into myofascial trigger points to reduce neuromuscular pain. We present a case of angioedema occurring after dry needling. <h3>Case Description</h3> A 45-year-old woman with a medical history notable for well-controlled chronic urticaria and no known food or drug allergies underwent routine dry needling into her neck and trapezius. That night, she developed progressive right sided facial and mucosal swelling without pruritis or urticaria. She sought medical care and received injectable epinephrine, antihistamines, and glucocorticoids. Her symptoms improved over the following days. Her subsequent laboratory work-up included normal complement levels, C1 esterase inhibitor level and function, and negative food-specific IgE panel. She was diagnosed with angioedema and instructed to avoid further dry needling sessions. She has had no subsequent episode of angioedema. <h3>Discussion</h3> No prior case report describes dry needling precipitating angioedema. In this case, the individual had no urticaria and did not respond immediately to injectable epinephrine. She had no other evident triggers or history of angioedema. Her laboratory work-up was unrevealing. The angioedema was likely triggered by local trauma caused by dry needling. Clinicians should be aware of dry needling as a possible traumatic trigger for patients at risk for angioedema.

Transient Small Bowel Angio-oedema Secondary to Intravenous Iodinated Contrast Medium

We report the clinical profile and computed tomography (CT) findings of a rare case of transient angio-edema of the small bowel following intravenous administration of non-ionic iodinated contrast material in a 36-year-old woman. The pre-contrast CT showed normal small bowel and large bowel wall thickness. Findings included long segment, circumferential bowel wall thickening involving the duodenum, jejunum, and almost the entire ileum on CT scan obtained following the intravenous contrast material injection. The large bowel wall appeared normal in post contrast images also. There was no mesenteric fat stranding, ascites or significant lymphadenopathy. This entity, like most contrast-induced adverse reactions, has a favorable clinical outcome with aggressive clinical monitoring and supportive measures.