The international HAE guideline under real-life conditions: From possibilities to limits in daily life - current real-world data of 8 German angioedema centers.

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.

[Nasal hemorrhage - risk factors and management in 707 outpatient and inpatient patients].

Epistaxis is one of the most common emergencies in otorhinolaryngology. The spectrum ranges from mild, self-limiting cases to life-threatening hemorrhages. Depending on the severity and course of the condition, management can be either outpatient or involve inpatient monitoring, possibly with surgical treatment.

Establishment of the deuterium oxide dilution method as a new possibility for determining the transendothelial water permeability.

Increase in transendothelial water permeability is an essential etiological factor in a variety of diseases like edema and shock. Despite the high clinical relevance, there has been no precise method to detect transendothelial water flow until now. The deuterium oxide (DO) dilution method, already established for measuring transepithelial water transport, was used to precisely determine the transendothelial water permeability.

A clinical evaluation of patients with known mutations (plasminogen and factor XII) with a focus on prophylactic treatment.

Background: Hereditary angioedema with normal C1-inhibitor (HAE-nC1-INH) is a rare genetic disease. The symptoms can resemble other forms of hereditary angioedema (HAE), but the specific laboratory values are inconspicuous. The knowledge about treatment strategies in HAE-nC1-INH remains insufficient; most of the drugs are only licensed and approved for other types of HAE.

A Retrospective Analysis of Long-Term Prophylaxis with Berotralstat in Patients with Hereditary Angioedema and Acquired C1-Inhibitor Deficiency-Real-World Data.

Hereditary angioedema (HAE) and acquired C1-inhibitor deficiency (AAE-C1-INH) are orphan diseases. Berotralstat is a recently licensed long-term prophylaxis (LTP) and the first oral therapy for HAE patients. No approved therapies exist for AAE-C1-INH patients.

Acquired Angioedema Due to C1-Inhibitor Deficiency (AAE-C1-INH)-A Bicenter Retrospective Study on Diagnosis, Course, and Therapy.

Background: Acquired angioedema with C1-inhibitor deficiency (AAE-C1-INH) is a rare condition resembling hereditary angioedema (HAE), but with late onset and low C1-inhibitor (C1-INH) due to consumption potentially caused by autoimmune diseases and mainly lymphatic malignancies. Being about 10-fold rarer than HAE, there is limited knowledge and no licensed therapy.

Objective: To report clinical and biological data from a newly described population of 20 patients with AAE-C1-INH assessing diagnostic delay, AAE-C1-INH:HAE-ratio, underlying conditions, and therapeutic management in Germany.

Concomitant medication in patients with bradykinin-mediated angioedema - there's more than ACE inhibitors.

Bradykinin-mediated angioedema is a rare, non-allergic, potentially life-threatening disease. ACE inhibitor-induced angioedema and hereditary angioedema (HAE) are the two most common presentations. Therapeutic options, pathophysiology and diagnosis continue to be investigated, with considerable progress in HAE over the last few decades.

New Year's Eve in otorhinolaryngology: a 16-year retrospective evaluation.

Purpose: Pyrotechnics are a long-standing tradition at the turn of the year. There are little data available on New Year's Eve-associated ORL injuries. Due to restrictions during the Corona pandemic, the handling of fireworks and meetings on New Year's Eve 2020-2022 had been significantly changed.

[Functional disorders of the nose - definition, diagnostics and treatment options].

The surgical spectrum of functional rhinosurgery includes nasal septum surgery, septorhinoplasty and nasal concha surgery. Based on the German guideline "Disorders of the inner and/or external nose (with functional and/or aesthetic impairment)" published in April 2022, which was prepared on behalf of the German Society of Otorhinolaryngology, Head and Neck Surgery, we discuss the indications, diagnostic approaches, the planning of the surgery and postoperative care. The most common findings of the external nose with a functional impairment include crooked nose, saddle nose, and tension nose.

Efficacy of human C1 esterase inhibitor concentrate for treatment of ACE-inhibitor induced angioedema.

Background: ACE inhibitor (ACEi) induced angioedema predominantly affects the upper aerodigestive tract. As ACEi induced angioedema is mediated by bradykinin, therapeutic response to antihistamines and glucocorticoids remains unsatisfactory. In bradykinin mediated hereditary angioedema, C1-esterase inhibitor (C1INH) is an effective and approved treatment since many years.

[Epistaxis Requiring Intervention After Swab for SARS-CoV-2].

Introduction: Since May 2020, the first nasopharyngeal POC tests for SARS-CoV-2 diagnostic have been available. Due to the long passage through the nasal cavity, there is a risk of injury with subsequent epistaxis.

Methods: We describe the course of disease of two female patients who suffered from massive epistaxis requiring intervention after an externally performed nasopharyngeal swab.

Etiology and predictors of cluster attacks of hereditary angioedema that recur despite pharmaceutical treatment.

Hereditary angioedema (HAE) is a disease that leads to recurrent swelling of the skin and mucous membranes, including the upper airway tract. Apart from being deadly, these attacks can be debilitating, which leads to a poor quality of life in patients. Clinicians are occasionally confronted with patients who have recurrent attacks despite treatment with C1 esterase inhibitor concentrate or β₂-receptor antagonists.

The Surgical Treatment of Peritonsillar Abscess: A Retrospective Analysis in 584 Patients.

Objectives: There are three surgical treatment options for patients with peritonsillar abscess (PTA): needle aspiration, incision and drainage (ID), and abscess tonsillectomy (ATE). The updated German national guideline (2015) included changes in the treatment of PTA. The indication for tonsillectomy (TE) in patients became more stringent and preference was given to ID in certain cases.

Surgical tracheostomy in a cohort of COVID-19 patients.

Background: One of the main symptoms of severe infection with the new coronavirus‑2 (SARS-CoV-2) is hypoxemic respiratory failure because of viral pneumonia with the need for mechanical ventilation. Prolonged mechanical ventilation may require a tracheostomy, but the increased risk for contamination is a matter of considerable debate.

Objective: Evaluation of safety and effects of surgical tracheostomy on ventilation parameters and outcome in patients with COVID-19.

Prospective Analysis in Patients With HAE Under Prophylaxis With Lanadelumab: A Real-life Experience.

Background And Objectives: Patients with the rare disease hereditary angioedema (HAE) suffer from recurrent acute attacks of edema. There is no curative therapy, but the frequency of attacks and quality of life of severely affected patients can be improved by prophylactic therapy. The monoclonal antibody lanadelumab has been approved for routine prophylaxis in patients with HAE since November 2018.

[Surgery of the Nasal Septum].

Dimension, position and stability of the cartilaginous nasal septum are crucial for successful surgery of the nasal septum. The objective of septoplasty is to create sufficient space in the anterior parts of the nose, particularly in the region of the nasal valves. Therefore, the cartilaginous nasal septum should obtain a state of "balanced tension".

Symptom Control of Patients With Chronic Rhinosinusitis With Nasal Polyps Under Maintenance Therapy With Daily Acetylsalicylic Acid.

Introduction: Nonsteroidal anti-inflammatory drugs (NSAID)-exacerbated respiratory disease (NERD) is defined by intolerance to cyclooxygenase 1 inhibitors, chronic rhinosinusitis with recurrent nasal polyps, and/or intrinsic bronchial asthma. Long-term administration of acetylsalicylic acid (ASA) after desensitization has been used to mitigate these sequelae, but the optimal dose and balancing symptom relief and side effects remain unsettled.

Methods: Retrospective data analysis of 85 patients with NERD receiving maintenance therapy of 300 mg ASA was followed by questionnaires (our own, not validated and the Sino-Nasal Outcome Test-20).

Clinical features of angioedema induced by renin-angiotensin-aldosterone system inhibition: a retrospective analysis of 84 patients.

: Bradykinin-mediated angioedema (AE) induced by antihypertensive drugs primarily affect the head and neck region and may occur even after several years of uneventful treatment. Many facts about the clinical course remain unknown. Diagnosis is not easy, as the clinical appearance resembles allergic AE.

The Influence of ACE Inhibition on C1-Inhibitor: A Biomarker for ACE Inhibitor-Induced Angioedema?

Aims: Angioedema is a rare side effect of angiotensin-converting enzyme (ACE) inhibitors. It remains unclear why it is only induced in a few patients taking ACE inhibitors, often after a long period of uneventful treatment. The aim of this study was to analyze the influence of ACE inhibitor treatment on C1-inhibitor (C1-INH) levels.

Cyclooxygenase activity in bradykinin-induced dermal extravasation. A study in mice and humans.

Background: Non-allergic angioedema is largely driven by increased plasma levels of bradykinin and over-activation of bradykinin receptor type II (B2), but the specific downstream signalling pathways remain unclear. The aim of this study was to identify signal transduction events involved in bradykinin-induced dermal extravasation.

Methods: Quantification of dermal extravasation was accomplished following intradermal (i.

Bradykinin signaling regulates solute permeability and cellular junction organization in lymphatic endothelial cells.

Objective: Determine the effect of bradykinin on solute permeability and cellular junctional proteins in human dermis microvascular endothelial cells.

Methods: Cells were characterized by immunofluorescence and fluorescence-activated cell sorting. Macromolecular transport of dextran and albumin was monitored.

Successful Long-Term Prophylactic Treatment With Subcutaneous C1 Esterase Inhibitor in a Patient With Hereditary Angioedema.

Background: Hereditary angioedema (HAE) patients suffer from recurrent swellings. Current standard therapy consists of C1 esterase inhibitor (C1-INH) and bradykinin receptor B2 antagonists. Severe courses require prophylactic treatment.

Thrombosis of the internal jugular vein in the ENT-department - Prevalence, causes and therapy: A retrospective analysis.

Objective: Less than 5% of deep vein thrombosis is due to thrombosis of the internal jugular vein. Genetic, malignant or inflammatory underlying diseases as well as insertion of venous catheters can be responsible for this pathology. Due to its rare occurrence, it is difficult to find systematic research about thrombosis of the internal jugular vein.