Case Rep Cardiol 2017 27;2017:6421208. Epub 2017 Aug 27.
Cardiology, John D. Dingell VA Medical Center, Wayne State University, Detroit, MI, USA.
There have been multiple reports of allergic reactions associated with acute coronary syndromes. This has been classically described as Kounis syndrome. We present an unusual case of 70-year-old male with multiple prior hypersensitivity reactions and history of coronary artery bypass grafting who presented recurrent episode of severe angioedema and anaphylaxis. Read More
Background: Recombinant human C1 esterase inhibitor (rhC1-INH) is approved for treatment of hereditary angioedema (HAE)in adolescents and adults. HAE attacks that involve the upper airway can be life threatening, and data on the administration of rhC1-INH for these types of attacks are currently limited.
Objective: To evaluate the efficacy and safety of rhC1-INH for treatment of acute HAE attacks with upper airway involvement. Read More
A 16-year-old male patient, presented with one month-history of complaints of an acute pain in the neck, accompanied by relapsing acute headache, sometimes irradiating forward the left arm. The patient had been diagnosed with hereditary angioedema (HAE) type I at age of 7, because of recurrent, non-pruritic edemas, affecting the skin of the face, neck, chest and abdomen, accompanied by colic-like intestinal spasms and relapsing bronchospasms, occurring without provocation and laboratory confirmed low serum levels of C1 inhibitor (Fig. 1a,b). Read More
Background: Severe attacks of hereditary angioedema (HAE) are debilitating and potentially life threatening, and can increase anxiety and the use of medical resources.
Objective: This post hoc assessment evaluated recombinant human C1 esterase inhibitor (rhC1-INH) used to treat acute severe HAE attacks.
Methods: In a double-blind, randomized-controlled trial (RCT), patients with an HAE attack (baseline visual analog scalescore of greater than or equal to 50 mm, with severe attacks defined as greater than or equal to 75 mm) were randomly assigned to receive rhC1-INH (50 IU/kg for patients who weighed <84 kg; 4200 IU for patients who weighed more than or equal to 84 kg) or placebo. Read More
Background: Idiopathic non-histaminergic acquired angioedema (InH-AAE) is a rare disease for which there are no available laboratory parameters to clearly define the disorder. Therapy is often difficult and various treatment options have been proposed. In this paper, we have evaluated the most effective therapies for InH-AAE on the basis of current literature and report the therapeutic effect of omalizumab in three patients with InH-AAE. Read More
Purpose: The understanding of microstructural and metabolic changes in the post-traumatic brain injury is the key to brain damage suppression and repair in clinics.
Methods: Ten female Wistar rats were traumatically injured in the brain CA1 region and above the cortex. Next, diffusion tensor magnetic resonance imaging (DTI) and proton magnetic resonance spectroscopy ((1)H MRS) were used to analyze the microstructural and metabolic changes in the brain within the following 2 weeks. Read More
Background: Health-related quality of life (HR-QoL) is impaired in patients with hereditary angioedema (HAE) but has notyet been satisfactorily described.
Objective: To study HR-QoL in patients with HAE by combining different HR-QoL instruments with disease activity assessment.
Methods: All adults in the Swedish HAE registry were invited to take part in this questionnaire study, which used the generic HR-QoL instruments, EuroQol 5 Dimensions 5 Level (EQ-5D-5L) and the RAND Corporation Short Form 36 (RAND-36), the disease-specific Angioedema Quality of Life instrument (AE-QoL), the recently introduced Angioedema Activity Score (AAS) form, and questionnaires on sick leave and prophylactic medication. Read More
Triazole antifungal drugs may rarely cause serious allergic reactions including angioedema. No standardized tests are available to predict cross-reactivity within the azole class and little guiding information on whether to change therapy within the class or to another class after a serious allergic reaction. Herein we report the first successful use, to our knowledge, of graded isavuconazole introduction for treatment of aspergillosis in a liver transplant recipient with severe voriconazole allergy. Read More
Angioedema has been observed in a few cases secondary to systemic lupus erythematosus (SLE). Herein, we report a rare case where a young healthy male initially presented with angioedema, lymphadenopathy and parotitis and later on developed neuropsychiatric manifestations at the very onset of his SLE disease. This case illustrates the importance of prompt clinical consideration of lupus with unusual and atypical preceding manifestations. Read More
The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage. Read More
Background: Acute angioedema of the upper airways can be life-threatening. An important distinction is drawn between mast-cell-mediated angioedema and bradykinin-mediated angioedema; the treatment of these two entities is fundamentally different.
Methods: This review is based on pertinent articles retrieved by a selective search in PubMed and on guidelines concerning the treatment of angioedema. Read More
Histamine intolerance (pseudoallergy) is a poorly investigated type of food hypersensitivity. The main enzyme responsible for histamine degradation in the extracellular matrix is diamine oxidase (DAO). Disturbances in the concentration or activity of DAO may lead to the development of clinical signs of allergy. Read More
Division of Pulmonary, Critical Care, Allergy and Immunology, Department of Medicine, Wake Forest University School of Medicine and Wake Forest Baptist Medical Center, Winston-Salem, NC; Division of Allergy and Clinical Immunology, Department of Medicine, W.G. (Bill) Hefner VA Medical Center, Salisbury, NC; Division of Allergy and Clinical Immunology, Department of Medicine, Kernersville Health Care Center, Kernersville, NC. Electronic address:
Anaphylaxis is a systemic, life-threatening disorder triggered by mediators released by mast cells and basophils activated via allergic (IgE-mediated) or nonallergic (non-IgE-mediated) mechanisms. It is a rapidly evolving, multisystem process involving the integumentary, pulmonary, gastrointestinal, and cardiovascular systems. Anaphylaxis and angioedema are serious disorders that can lead to fatal airway obstruction and culminate in cardiorespiratory arrest, resulting in hypoxemia and/or shock. Read More
Anaphylaxis is the most severe form of allergic reaction, resulting from the effect of mediators and chemotactic substances released by activated cells. Mast cells and basophils are considered key players in IgE-mediated human anaphylaxis. Beyond IgE-mediated activation of mast cells/basophils, further mechanisms are involved in the occurrence of anaphylaxis. Read More
Background: Hereditary angioedema (HAE) with normal C1-INH (HAEnCI) may be linked to specific mutations in the coagulation factor 12 (FXII) gene (HAE-FXII) or functional mutations in other genes that are still unknown. We sought to identify and characterize a hitherto unknown type of HAE with normal C1-INH and without mutation in the F12 gene.
Methods: The study comprised analysis of whole-exome sequencing, Sanger sequencing, and clinical data of patients. Read More
Purpose Of Review: The aims of this study are to update the clinician on current understanding of angioedema as it presents in the pediatric population and to review proper diagnostic techniques and treatment modalities for various types of angioedema.
Recent Findings: Angioedema is still best classified by whether it is likely histaminergic or kinin-mediated. New guidelines have been published around the world to help diagnose and treat both forms (urticaria/angioedema and hereditary angioedema). Read More
Hereditary angioedema (HAE) with C1-inhibitor (C1-Inh) deficiency (C1-Inh-HAE) is a rare, life-threatening, and disabling genetic disorder characterized by self-limited tissue swelling caused by deficiency or dysfunction of C1-Inh. Our aim in this update is to discuss new advances in HAE therapy, focusing mainly on the various treatment options that have become available recently and also drugs that are under trial for prophylaxis to prevent attacks. There is a paradigm shift to where the treatment of HAE is headed, focusing now on prophylactic treatment rather than abortive management. Read More
Background: A non-interventional study suggested that use of angiotensin-converting enzyme inhibitors (ACEIs) or aliskiren was associated with an angioedema risk three times that of beta-blockers (BBs).
Objective: The aim was to assess angioedema incidence rates (IRs) and the relative angioedema risk of aliskiren compared to other antihypertensive drugs (AHDs).
Methods: A cohort study in hypertensive patients with an AHD prescription between 2007 and 2012 was conducted using data from the US PharMetrics Plus™ claims database. Read More
Atlas Oral Maxillofac Surg Clin North Am 2017 Sep;25(2):127-132
Department of Diagnostics and Biological Sciences, University of Colorado School of Dental Medicine, 13065 East 17th Avenue, Mail Stop F844, Aurora, CO 80045, USA; Department of Family Medicine, University of Colorado School of Medicine, 12631 East 17th Avenue, Aurora, CO 80045, USA. Electronic address:
Angiotensin-converting enzyme inhibitor induced angioedema commonly involves the head and neck area. We report a case of angiotensin-converting enzyme inhibitor induced intestinal angioedema in a heart transplant recipient on mTOR immunosuppression. A 36-year-old Caucasian woman with history of heart transplantation on sirolimus, tacrolimus and prednisone presented to the Emergency Department with abdominal pain, one day following lisinopril initiation. Read More
The purpose of this study was to assess the impact of age and sex on the reporting of cough and angioedema related to renin-angiotensin system (RAS) inhibitors. A case/noncase study was performed in VigiBase. Two case groups were identified, reports of cough and reports of angioedema, and noncases were all reports of all other adverse events. Read More
Autoimmune progesterone dermatitis is a rare cyclic premenstrual reaction to progesterone produced during the luteal phase of the menstrual cycle. The clinical symptoms of autoimmune progesterone dermatitis overlap with other forms of dermatosis such as erythema multiforme, eczema, fixed drug eruption, urticaria, and angioedema. We experienced 3 cases of autoimmune progesterone dermatitis. Read More
Summary: Monoclonal anti-IgE antibodies (omalizumab) are able to induce clinically significant benefits in patients with severe chronic spontaneous urticaria (CS). Those results led clinicians and investigators to reconsider a possible pathogenic role not previously supported for IgE and its receptors in this disease, and to investigate additional approaches for understanding its pathogenesis. IgE antibodies to unknown environmental allergens able to trigger chronic urticaria are not generally regarded as the etiologic factor for the disease. Read More
Chronic urticaria is a spontaneous or inducible group of diseases characterized by the occurrence of wheals (and, in about half of cases, angioedema) for more than 6 weeks. These are rather frequent conditions that may severely affect patients' quality of life and sometimes represent a challenge for doctors as well. The causes of chronic urticaria are still poorly defined, although there is growing evidence that different biologic systems including immunity, inflammation, and coagulation may take part in the pathomechanism eventually leading to mast cell and basophil degranulation and hence to wheal formation. Read More
Background: Omalizumab, a recombinant anti-IgE antibody, effectively treats chronic spontaneous urticaria. Evidence is lacking in patients with chronic inducible urticarias (CIndUs), which are frequently H1-antihistamine resistant.
Objective: From the current published literature, we aimed to determine the strength of evidence for omalizumab efficacy and safety in the treatment of CIndUs. Read More
Urticaria is a common, mast cell-driven disease presenting with wheals or angioedema or both. In the last years, urticaria has increasingly attracted notice to clinicians and researchers, last but not least inspired by the approval of omalizumab, an anti-IgE antibody, for urticaria treatment. There is wide consensus on the clinical classification based on duration and elicitation. Read More
Aim: To assess the required characteristics (cost, sensitivity and specificity) of a pharmacogenomic test for being a cost-effective prevention of angiotensin-converting enzyme inhibitors induced angioedema. Furthermore, we assessed the influence of only testing high-risk populations.
Materials & Methods: A decision tree was used. Read More
Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany; Clinical Chemistry, Department of Molecular Medicine and Surgery, Karolinska Institutet and University Hospital, 17176 Stockholm, Sweden. Electronic address:
The contact system is a potent procoagulant and proinflammatory plasma protease cascade that is initiated by binding ("contact")-induced, auto-activation of factor XII zymogen. Formed active serine protease FXIIa then cleaves plasma prekallikrein to kallikrein that in turn liberates the mediator bradykinin from its precursor high molecular weight kininogen. Bradykinin induces inflammation with implications for host defense and innate immunity. Read More
Macroglossia is classified as true macroglossia, which exhibits abnormal histology with clinical findings, and relative macroglossia, in which normal histology does not correlate with pathologic enlargement. This report describes an atypical case of morbidity with massive macroglossia secondary to myxedema; the macroglossia enlarged over a 3-month period before being presented to the Department of Oral and Maxillofacial Surgery, University of Texas Health Science Center at Houston (Houston, TX). Substantial enlargement of the tongue (16 cm long × 10 cm wide) was first attributed to angioedema, which was refractory to the discontinuation of lisinopril and a C1 esterase inhibitor. Read More
Chronic urticaria (CU) is the occurrence of urticaria with or without angioedema for at least 6 weeks. Management has traditionally involved antihistamines as first-line therapy with various alternative therapies for refractory cases. Largely based on the success of biologics for various diseases, this class of drugs has come to the forefront of medical research. Read More
Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Read More
Mast cells (MCs) are physiologically activated by binding of stem cell factor (SCF) to the extracellular domains of the Kit receptor. This binding increases the proliferation and prolongs the survival of normal mature MCs, as well as intensifies the release of mediators. In mastocytosis, somatic mutations of the coding Kit gene cause autocrine dysregulation and lead to constitutive KIT activation even in the absence of its ligand SCF. Read More
Purpose: Assess angioedema risk with exposure to angiotensin converting enzyme inhibitors (ACEIs) or angiotensin II receptor blockers (ARBs) compared with beta-blockers, by race/ethnicity.
Methods: New-user cohorts of Medicare beneficiaries 65 years or older initiating ACEI, ARB, or beta-blocker treatment from March 2007 to March 2014 were constructed. Angioedema incidence rates by drug and race/ethnicity were computed for 1-30 and 31-365 days of treatment. Read More
From the Department of Pharmacy, Clinical and Administrative Sciences, University of Oklahoma Health Sciences Center, Oklahoma City (REO); Clinical Pharmacy Specialist, Cardiology/Anticoagulation, Methodist University Hospital, Memphis, TN (CSO); and Department of Clinical Pharmacy, University of Tennessee College of Pharmacy, Nashville (CSO).
Incorporation of neprilysin inhibition into heart failure pharmacotherapy regimens has recently been recommended by U.S. guidelines, based on results from the PARADIGM-HF trial comparing sacubitril/valsartan to enalapril. Read More
Eur Urol Focus 2017 Feb 5;3(1):89-93. Epub 2016 Feb 5.
Department of Urology, Loyola University Medical Center, Maywood, IL, USA; 1:MAP Surgical Analytics Group, Loyola University Medical Center, Maywood, IL, USA; Department of Surgery, Loyola University Medical Center, Maywood, IL, USA.
Adverse reactions (ARs) to intravenous (IV) radiographic contrast range from mild urticaria to life-threatening anaphylaxis. Intraluminal contrast dye is routinely used in the urinary tract with a minimal perceived risk of AR. We used the Healthcare Cost and Utilization Project State Inpatient Databases for California and Florida from 2007 to 2011 to identify patients who received urinary tract contrast dye for retrograde pyelography, percutaneous pyelography, retrograde/other cystogram, and ileal conduitogram. Read More
Importance: Hereditary angioedema (HAE) is a rare but severe disease, with high risk of death, and attacks have been associated to high estrogen levels. Polycystic ovary syndrome (PCOS) is a common hyperandrogenic condition, which is frequently treated with combined oral contraceptives.
Objective: The aim of this study was to describe 2 clinical cases of young women diagnosed as having PCOS who developed HAE attacks after the introduction of combined estrogen-progestin pills to treat PCOS symptoms. Read More
Background: The severe life-threatening characteristics of hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) can affect anxiety levels among pediatric patients. This emotional burden together with the physical restrictions of C1-INH-HAE may decrease children's health-related quality of life (HRQoL).
Objectives: (i) To compare anxiety state and trait between children with C1-INH-HAE and healthy controls; (ii) to examine the relationship between the level of anxiety of children with C1-INH-HAE, their disease activity/affected sites and their HRQoL; and (iii) to predict the HRQoL of children with C1-INH-HAE based on their anxiety level and disease activity/affected sites METHODS: Thirty-three children with C1-INH-HAE (aged 5-18 years) and 52 healthy controls were recruited from Israel and Hungary. Read More
Background: Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. However, despite the use of long-term prophylaxis (LTP), breakthrough attacks are known to occur. We used data from the Icatibant Outcome Survey (IOS) to evaluate the characteristics of breakthrough attacks and the effectiveness of icatibant as a treatment option. Read More