An Open-Label Phase 1b/2 Trial of Navenibart, a Long-Acting Plasma Kallikrein Inhibitor for Hereditary Angioedema.

Achieving a normal life in hereditary angioedema: Quality of life and treatment gaps among German HAE patients.

Eosinophilic inflammation in hereditary angioedema: a single-center real-world retrospective chart review study.

Rethinking the management of hereditary angioedema.

Updates in Hereditary Angioedema and Chronic Spontaneous Urticaria.

Attack rate reductions following berotralstat initiation among US patients with hereditary angioedema in the real-world.

Type II hereditary angioedema with an apparently de novo SERPING1 mutation in China: A case report and family screening.

Patient-reported disease burden and health care utilization of HAE-nl-C1INH: insights from a real-world survey.

Treatment of Hereditary Angioedema With Plasma-Derived C1 Inhibitor: A Review.

Management of hereditary angioedema with normal C1Inh: a series of 163 French patients.

A sensitive and specific assay to characterize plasma kallikrein activity in plasma from patients with hereditary angioedema.

C1 esterase inhibitor (C1-INH) response as a supportive diagnostic criterion for patients with suspected hereditary angioedema with normal C1-INH.

Hereditary Angioedema With Normal C1 Esterase Inhibitor: A Case of a Late Diagnosis in a 48-Year-Old Male.

Berotralstat effectiveness and safety in patients with hereditary angioedema with normal C1 inhibitor.

French protocol for the diagnosis and management of hereditary angioedema.

Unraveling angioedema: diagnostic challenges and emerging therapies.

C1 Esterase Inhibitor Deficiency in an Indian Female: A Rare Case Report and a Review of Literature with Treatment Update.

A hidden clue behind angioedema in an elderly patient.

Real-world treatment patterns and burden-of-disease of sub-optimally controlled hereditary angioedema.

Evaluating functional C1INH with multiple laboratory methods across Hereditary Angioedema types.

Insights From the First 820 Patients From the Brazilian Multicenter Registry of Hereditary Angioedema: The Key Role of Genetic Testing and Targeted Therapies.

C1 inhibitor: from complement system to bradykinin angioedema.

Status quo and future developments in the diagnosis and treatment of hereditary angioedema.

Long-term prophylactic treatment with deucrictibant for angioedema due to acquired C1-inhibitor deficiency.

Garadacimab in hereditary angioedema due to normal C1INH with F12/PLG mutations.

Expanding the Genetic and Clinical Spectrum of Hereditary Angioedema with Normal C1 Inhibitor: Novel Variants and Treatment Insights.

The determinants of angioedema attacks related to dental and gingival procedures in hereditary angioedema patients.

Incidental findings related to genes associated to HAE-nC1INH: how to proceed?

An Atypical Case of Angioedema With Normal C1-Inhibitor Responding to Both C1-Inhibitor Concentrate and Omalizumab.

Hereditary angioedema diagnosis: Reflecting on the past, envisioning the future.

Network Meta-Analysis of Pharmacological Therapies for Long-Term Prophylactic Treatment of Patients with Hereditary Angioedema.

Angioedema without urticaria: Diagnosis and management.

Global frequency, diagnosis, and treatment of hereditary angioedema with normal C1 inhibitor.

Expert Consensus on the Diagnosis and Treatment of Hereditary Angioedema in China (2024 Edition).

[A CASE OF HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR (HEREDITARY ANGIOEDEMA TYPE III) WITH A PATHOGENIC VARIANT IN THE PLG GENE].

Comorbidities in Canadian patients with hereditary angioedema: a quantitative survey study.

Hereditary Angioedema With Normal C1 Inhibitor: A Quarter Century of Forward Progress and Persisting Obstacles.

Hereditary Angioedema with Normal C1 Inhibitor: an Updated International Consensus Paper on Diagnosis, Pathophysiology, and Treatment.

De Novo or inherited: gonosomal mosaicism in hereditary angioedema due to C1 inhibitor deficiency.

A Case of Status Epilepticus in a Patient Experiencing an Acute Attack of Hereditary Angioedema.

Omics analysis reveals galectin-3 to be a potential key regulator of allergic inflammation in hereditary angioedema.

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

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

Rare connective tissue diseases in patients with C1-inhibitor deficiency hereditary angioedema: first evidence on prevalence and distribution from a large Italian cohort study.

Unveiling the Complexities of Hereditary Angioedema.

Hereditary angioedema prevalence and satisfaction with prophylaxis in South Australia.

Physician- and patient-reported outcomes by hereditary angioedema type: Data from a real-world study.

Case Report: Early presentation of hereditary angioedema symptoms in a 2-year-old boy.

Epidemiology, economic, and humanistic burden of hereditary angioedema: a systematic review.

Normalization of C1 Inhibitor in a Patient with Hereditary Angioedema.

Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema.

Contact System Activation and Bradykinin Generation in Angioedema: Laboratory Assessment and Biomarker Utilization.

Altered levels of phospholipases C, diacylglycerols, endocannabinoids, and N-acylethanolamines in patients with hereditary angioedema due to FXII mutation.

Quantification of C1 inhibitor activity using a chromogenic automated assay: analytical and clinical performances.

Hereditary angioedema with normal C1 esterase inhibitor: Current paradigms and clinical dilemmas.

The future of therapeutic options for hereditary angioedema.

Hereditary angioedema with a normal C1 inhibitor in Japanese man.

Clinical Experience with Berotralstat in Patients with Hereditary Angioedema with Normal C1-Esterase Inhibitor: A Commented Case Series.

Hereditary Angioedema With a Normal Complement Level.

Hereditary angioedema with normal C1-inhibitor: Clinical and genetic characterization of 15 Portuguese unrelated families.

Patient outcomes associated with subcutaneous C1INH prophylaxis for hereditary angioedema: a retrospective analysis.

Plasma-derived C1 esterase inhibitor pharmacokinetics and safety in patients with hereditary angioedema.

Screening for type II hereditary angioedema-the "poor man's c1-inhibitor function".

[AN ADOLESCENT CASE OF HAE-NL-C1INH (WHO WAS) MISDIAGNOSED WITH WHEAT ALLERGY AND MULTIPLE DRUG ALLERGIES].

Multidisciplinary Prophylactic Strategies for Recurrence of Laryngeal Edema After Tooth Extraction in a Patient With Hereditary Angioedema: A Case Report.

A patient with hereditary angioedema (HAE) with normal C1-INH and SLE with pregnancy.

How Does Pregnancy and Type of Delivery Affect the Clinical Course of Hereditary Angioedema?

The US Hereditary Angioedema Association Scientific Registry: hereditary angioedema demographics, disease severity, and comorbidities.

Decreased adhesion to endothelium leads to elevated neutrophil granulocyte count in hereditary angioedema patients.

The multifactorial impact of receiving a hereditary angioedema diagnosis.

Angioedema in the Absence of C1 Esterase Inhibitor Deficiency in a Young Patient With Anti-dsDNA Negative Lupus Nephritis.

Restriction of C1-inhibitor activity in hereditary angioedema by dominant-negative effects of disease-associated SERPING1 gene variants.

Biochemistry, molecular genetics, and clinical aspects of hereditary angioedema with and without C1 inhibitor deficiency.

Managing Diagnosis, Treatment, and Burden of Disease in Hereditary Angioedema Patients with Normal C1-Esterase Inhibitor.

Hereditary angio-oedema with normal C1-INH, developing recurrent acute abdomen after taking low-dose oestrogen-progestin: A case report.

Efficacy, pharmacokinetics, and safety of subcutaneous C1-esterase inhibitor as prophylaxis in Japanese patients with hereditary angioedema: Results of a Phase 3 study.

Hereditary Angioedema with Normal C1 Esterase Inhibitor Refractory to Long-Term Prophylaxis: A Case Report.

Gene Mutations Linked to Hereditary Angioedema in Solitary Angioedema Patients With Normal C1 Inhibitor.

Hereditary Angioedema With Normal C1 Inhibitor: US Survey of Prevalence and Provider Practice Patterns.

C1 inhibitor deficiency enhances contact pathway-mediated activation of coagulation and venous thrombosis.

Mutant plasminogen in hereditary angioedema is bypassing FXII/kallikrein to generate bradykinin.

Hereditary Angioedema: Diagnosis, Clinical Implications, and Pathophysiology.

Case report: Recurrent angioedema: Diagnosing the rare and the frequent.

A Missense Mutation of the Plasminogen Gene in a Japanese Family with Hereditary Angioedema with Normal C1 Inhibitor: Third Family Survey in Asia.

Hereditary Angioedema: The Clinical Picture of Excessive Contact Activation.

Phenotypic and Genotypic Characterization of Hereditary Angioedema in Saudi Arabia.

Interventions for the long-term prevention of hereditary angioedema attacks.

Isolated angioedema: A review of classification and update on management.

Icatibant use in Brazilian patients with hereditary angioedema (HAE) type 1 or 2 and HAE with normal C1-INH levels: findings from the Icatibant Outcome Survey Registry Study.

Clinical profile and treatment outcomes in patients with hereditary angioedema with normal C1 esterase inhibitor.

Reviewing clinical considerations and guideline recommendations of C1 inhibitor prophylaxis for hereditary angioedema.

Angioedema Without Wheals: Challenges in Laboratorial Diagnosis.

Inheritance Pattern of Hereditary Angioedema Indicates Mutation-Dependent Selective Effects During Early Embryonic Development.

COVID-19 and hereditary angioedema: Incidence, outcomes, and mechanistic implications.

Specific Targeting of Plasma Kallikrein for Treatment of Hereditary Angioedema: A Revolutionary Decade.

[Diagnosis of hereditary angioedema after thirty years of clinical manifestations].

The role of C1 inhibitor and complement as acute phase reactants: are we missing the diagnosis of hereditary angioedema?

HAE patient self-sampling for biomarker establishment.

A Potentially Fatal Outcome of Oral Contraceptive Therapy: Estrogen-Triggered Hereditary Angioedema in an Adolescent.

Clinical and genetic features of hereditary angioedema with and without C1-inhibitor (C1-INH) deficiency in Japan.

The crux of C1-INH testing in everyday lab work.

Analysis of cold activation of the contact system in hereditary angioedema with normal C1 inhibitor.

The Genetics of Hereditary Angioedema: A Review.

Roles of Immune Cells in Hereditary Angioedema.

Consensus on treatment goals in hereditary angioedema: A global Delphi initiative.

Clinical profile of hereditary angioedema from a tertiary care centre in India.

Unnecessary Abdominal Surgeries in Attacks of Hereditary Angioedema with Normal C1 Inhibitor.

The Expanding Spectrum of Mutations in Hereditary Angioedema.

Survey of actual conditions of erythema marginatum as a prodromal symptom in Japanese patients with hereditary angioedema.

Periodic Severe Angioedema without Exogenous Hormone Exposure.

Population pharmacokinetics of recombinant human C1 esterase inhibitor in children with hereditary angioedema.

Long-term health-related quality of life in patients treated with subcutaneous C1-inhibitor replacement therapy for the prevention of hereditary angioedema attacks: findings from the COMPACT open-label extension study.

Leveraging Genetics for Hereditary Angioedema: A Road Map to Precision Medicine.

Acquired Angioedema with C1 Inhibitor Deficiency: Occurrence, Clinical Features, and Management: A Nationwide Retrospective Study in the Czech Republic Patients.

Pathophysiology of Hereditary Angioedema (HAE) Beyond the SERPING1 Gene.

Psychology and hereditary angioedema: A systematic review.

A robust multiplexed assay to quantify C1-inhibitor, C1q, and C4 proteins for in vitro diagnosis of hereditary angioedema from dried blood spot.

Review of icatibant use in the Winnipeg Regional Health Authority.

The Panorama of Primary Angioedema in the Brazilian Population.

Hereditary angioedema: Pathophysiology (HAE type I, HAE type II, and HAE nC1-INH).

Hereditary angioedema: Epidemiology and burden of disease.

Definition and classification of hereditary angioedema.

Hereditary Angioedema Attack in Utero and Treatment of the Mother and Fetus.

Clinical features of genetically characterized types of hereditary angioedema with normal C1 inhibitor: a systematic review of qualitative evidence.

Management of hereditary angioedema in Japan: Focus on icatibant for the treatment of acute attacks.

Orthognathic Surgery in Hereditary Angioedema With Normal C1 Inhibitor: A Clinical Response to Concentrated C1 Inhibitor Against Angioedema Attack.

Measurement of Bradykinin Formation and Degradation in Blood Plasma: Relevance for Acquired Angioedema Associated With Angiotensin Converting Enzyme Inhibition and for Hereditary Angioedema Due to Factor XII or Plasminogen Gene Variants.

Study of angiopoietin and plasminogen genes in hereditary angioedema.

Novel homozygous variants in the SERPING1 gene in two Turkish families with hereditary angioedema of recessive inheritance.

High prevalence of epilepsy in HAE with normal C1-INH.

[EFFICACY, PHARMACOKINETICS, PHARMACODYNAMICS, AND SAFETY OF INTRAVENOUS C1 INHIBITOR FOR LONG-TERM PROPHYLAXIS AND TREATMENT OF BREAKTHROUGH ATTACKS IN JAPANESE SUBJECTS WITH HEREDITARY ANGIOEDEMA: A PHASE 3 OPEN-LABEL STUDY].

A first of its kind quantitative functional C1-esterase inhibitor lateral flow assay for hereditary angioedema point-of-care diagnostic testing.

Treatment of patients with hereditary angioedema with the c.988A>G (p.Lys330Glu) variant in the plasminogen gene.

Hereditary angioedema in a single family with specific mutations in both plasminogen and SERPING1 genes.

A novel functional C1 inhibitor activity assay in dried blood spot for diagnosis of Hereditary angioedema.

Biological therapy in hereditary angioedema: transformation of a rare disease.

sgp120 and the contact system in hereditary angioedema: A diagnostic tool in HAE with normal C1 inhibitor.

Evidence for bradykinin release in chronic spontaneous urticaria.

Impaired control of the contact system in hereditary angioedema with normal C1-inhibitor.

The International/Canadian Hereditary Angioedema Guideline.

Recurrent swelling and pain in the abdomen and joints in a patient with hereditary angioedema and Ehlers-Danlos syndrome.

Hereditary and acquired angioedema.

Acquired complement C1 esterase inhibitor deficiency in a patient with a rare SERPING1 variant with unknown significance.

Subcutaneous C1 inhibitor for prevention of attacks of hereditary angioedema: additional outcomes and subgroup analysis of a placebo-controlled randomized study.

Hereditary Angio-Oedema for Dermatologists.

Increased fibrinolysis-induced bradykinin formation in hereditary angioedema confirmed using stored plasma and biotechnological inhibitors.

Hereditary Angioedema-Associated Acute Pancreatitis in C1-Inhibitor Deficient and Normal C1-Inhibitor Patients: Case Reports and Literature Review.

A randomized trial of human C1 inhibitor prophylaxis in children with hereditary angioedema.

C1-INH concentrate for prophylaxis during pregnancy in hereditary angioedema with normal C1-INH.

Oxidative stress markers in patients with hereditary angioedema.

Fixed-Dose Subcutaneous C1-Inhibitor Liquid for Prophylactic Treatment of C1-INH-HAE: SAHARA Randomized Study.

Melkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features.

A mechanism for hereditary angioedema with normal C1 inhibitor: an inhibitory regulatory role for the factor XII heavy chain.

Dominant-negative SERPING1 variants cause intracellular retention of C1 inhibitor in hereditary angioedema.

Deletions in SERPING1 Lead to Lower C1 Inhibitor Function: Lower C1 Inhibitor Function Can Predict Disease Severity.

Severity of hereditary angioedema, prevalence, and diagnostic considerations.

Repeated attacks of type III hereditary angioedema with factor XII mutation during pregnancy.

Gene therapy for C1 esterase inhibitor deficiency in a Murine Model of Hereditary angioedema.

Threshold-stimulated kallikrein activity distinguishes bradykinin- from histamine-mediated angioedema.

Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies.

Hereditary angioedema from the patient's perspective: A follow-up patient survey.

A missense mutation in the plasminogen gene, within the plasminogen kringle 3 domain, in hereditary angioedema with normal C1 inhibitor.

Use of pdC1-INH concentrate for long-term prophylaxis during pregnancy in hereditary angioedema with normal C1-INH.

[Review of a new subtype of hereditary angio-oedema with normal complement C1-inhibitor].

Hereditary Angioedema with Normal C1 Inhibitor and F12 Mutations in 42 Brazilian Families.

Clinical Features of Hereditary and Mast Cell-mediated Angioedema Focusing on the Differential Diagnosis in Japanese Patients.

Presymptomatic genetic diagnosis of two siblings with hereditary angioedema, presenting with unusual normal levels of serum C4.

Hereditary angioedema with a mutation in the plasminogen gene.

The Complex Interaction Between Polycystic Ovary Syndrome and Hereditary Angioedema: Case Reports and Review of the Literature.

Hereditary Angioedema with Normal C1 Inhibitor: Update on Evaluation and Treatment.

The role of the complement system in hereditary angioedema.

Complement factor C4 activation in patients with hereditary angioedema.

Sustained response of recombinant human C1 esterase inhibitor for acute treatment of hereditary angioedema attacks.

Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency.

Hereditary angioedema with normal C1 inhibitor: clinical characteristics and treatment response with plasma-derived human C1 inhibitor concentrate (Berinert®) in a French cohort.

Hereditary angioedema with normal C1 inhibitor in a French cohort: Clinical characteristics and response to treatment with icatibant.

Recombinant Human C1-Esterase Inhibitor to Treat Acute Hereditary Angioedema Attacks in Adolescents.

Screening for hereditary angioedema (HAE) at 13 emergency centers in Osaka, Japan: A prospective observational study.

Treatment for hereditary angioedema with normal C1-INH and specific mutations in the F12 gene (HAE-FXII).

C1-esterase inhibitor for short-term prophylaxis in a patient with hereditary angioedema with normal C1 inhibitor function.

A Case of Type 2 Hereditary Angioedema With SERPING1 Mutation.

Kininogen Cleavage Assay: Diagnostic Assistance for Kinin-Mediated Angioedema Conditions.

Efficacy of Treatment of Non-hereditary Angioedema.

Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema.

Thyroid hormones and complement parameters in hereditary angioedema with C1-inhibitor deficiency.

Hereditary angioedema with normal C1 inhibitor and factor XII mutation: a series of 57 patients from the French National Center of Reference for Angioedema.

Diagnosis and screening of patients with hereditary angioedema in primary care.

A Unique Case of Angioedema With Anti-C1 Inhibitor Antibodies and Normal C1 Inhibitor Levels.

"Nuts and Bolts" of Laboratory Evaluation of Angioedema.

Genetics of Hereditary Angioedema Revisited.

Angioedema Phenotypes: Disease Expression and Classification.

Hereditary angioedema in a Jordanian family with a novel missense mutation in the C1-inhibitor N-terminal domain.

The Levels of the Lectin Pathway Serine Protease MASP-1 and Its Complex Formation with C1 Inhibitor Are Linked to the Severity of Hereditary Angioedema.

Recombinant replacement therapy for hereditary angioedema due to C1 inhibitor deficiency.

Type I and Type II Hereditary Angioedema: Clinical and Laboratory Findings in Iranian Patients.

Current state of hereditary angioedema management: a patient survey.

Hereditary angioedema with normal C1-INH with versus without specific F12 gene mutations.

Clinical manifestations, diagnosis, and treatment of hereditary angioedema: survey data from 94 physicians in Japan.

Recurrent intestinal obstruction with acquired angio-oedema, due to C1-esterase inhibitor deficiency.