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.

Genotype-Phenotype Correlation of Hereditary Plasminogen Deficiency: Molecular Mechanisms From 18 Patients With Cerebral Infarction.

Rethinking the management of hereditary angioedema.

Updates in Hereditary Angioedema and Chronic Spontaneous Urticaria.

[Compound heterozygous plasminogen mutations causing hereditary plasminogen deficiency: a family study and mechanistic analysis].

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.

Diagnostically challenging ligneous conjunctivitis with confirmed PLG variants: clinical and genetic insights.

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.

Plasminogen mutation-associated thrombotic microangiopathy and role of anticoagulation: a single institution case series.

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.

Cross-Trait Meta-Analysis Reveals a Genetic Link between Inflammation and Aging in Giant Cell Arteritis.

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

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

Cryo-Depleted Plasma Infusions for Ligneous Conjunctivitis.

Genetic variants contribute to modulation of renal function in patients with immune thrombotic thrombocytopenic purpura.

Plasminogen Activation in Oral Mucosal Immunopathology.

Genetic Susceptibility to Periodontitis.

Ligneous periodontitis: A molecularly confirmed case of type I plasminogen deficiency.

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.

Plasminogen activation and plasmin activity are not necessary to prevent venous thrombosis/thromboembolism.

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].

Conformational and Stability Analysis of SARS-CoV-2 Spike Protein Variants by Molecular Simulation.

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

How I treat type 1 plasminogen deficiency.

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.

Exploring Potential Drug Targets in Multiple Cardiovascular Diseases: A Study Based on Proteome-Wide Mendelian Randomization and Colocalization Analysis.

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

Genetic risk variants implicate impaired maintenance and repair of periodontal tissues as causal for periodontitis-A synthesis of recent findings.

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

Oligogenic risk score for Gilles de la Tourette syndrome reveals a genetic continuum of tic disorders.

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.

Identification of novel proteins for coronary artery disease by integrating GWAS data and human plasma proteomes.

Screening of differential gene expression patterns through survival analysis for diagnosis, prognosis and therapies of clear cell renal cell carcinoma.

Hereditary angioedema prevalence and satisfaction with prophylaxis in South Australia.

Plasminogen missense variants and their involvement in cardiovascular and inflammatory disease.

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

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.

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.

Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study.

Accelerated Engineering of ELP-Based Materials through Hybrid Biomimetic-De Novo Predictive Molecular Design.

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].

Ligneous periodontitis exacerbated by Behçet's disease in a patient with plasminogen deficiency and a stop-gained variant PLG c.1468C > T: a case report.

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

Recurrent Meningitis and Its Rare Association With Ligneous Conjunctivitis and Congenital Plasminogen Deficiency.

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.

Concurrent Cobalamin C and Plasminogen Deficiencies in a Patient with Chronic Thrombotic Microangiopathy.

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

Diagnosis of Immunoglobulin G4-related disease in a child with ligneous conjunctivitis: a novel mutation in plasminogen gene and plasminogen activator inhibitor-1 polymorphism.

Plasmin-Mediated Fibrinolysis in Periodontitis Pathogenesis.

Preclinical evaluation of PHH-1V vaccine candidate against SARS-CoV-2 in non-human primates.

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.

Germline genetic variants are associated with development of insulin-dependent diabetes in cancer patients treated with immune checkpoint inhibitors.

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.

Variants in complement genes are uncommon in patients with anti-factor H autoantibody-associated atypical hemolytic uremic syndrome.

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.

Case-only design identifies interactions of genetic risk variants at SIGLEC5 and PLG with the lncRNA CTD-2353F22.1 implying the importance of periodontal wound healing for disease aetiology.

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

Molecular Genetic Screening in Patients With ACE Inhibitor/Angiotensin Receptor Blocker-Induced Angioedema to Explore the Role of Hereditary Angioedema Genes.

Ligneous conjunctivitis mimicking preseptal cellulitis in a 3-month-old infant.

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.

Severe dysplasminogenemia due to homozygous PLG Ala620Thr variant in a Korean woman without a history of venous thromboembolism: A case report and literature review.

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

PROS1 variant in sudden death case of pulmonary embolism caused by calcification in the inferior vena cava: The importance of postmortem genetic analysis.

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

Metal Ions Bound to Prion Protein Affect its Interaction with Plasminogen Activation System.

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.

Identification of four novel QTL linked to the metabolic syndrome in the Berlin Fat Mouse.

[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.

Proteolyzed Variant of IgG with Free C-Terminal Lysine as a Biomarker of Prostate Cancer.

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 validity and utility of preconception expanded carrier screening for the management of reproductive genetic risk in IVF and general population.

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

The Expanding Spectrum of Mutations in Hereditary Angioedema.

A Novel mRNA Modification Mutation in a Patient With Ligneous Conjunctivitis Coexisting With Heterozygous Familial Mediterranean Fever Mutation.

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.

[A case of inherited afibrinogenemia caused by an IVS7-12A>G splice mutation of FGG gene].

Review of icatibant use in the Winnipeg Regional Health Authority.

Pathophysiology and underlying mechanisms in hereditary angioedema.

Distinct genetic profile with recurrent population-specific missense variants in Korean adult atypical hemolytic uremic syndrome.

Deciphering the Genetics of Primary Angioedema with Normal Levels of C1 Inhibitor.

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.

Multi-omic studies on missense PLG variants in families with otitis media.

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.

Functional prediction and comparative population analysis of variants in genes for proteases and innate immunity related to SARS-CoV-2 infection.

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.

Association Study of Coronary Artery Disease-Associated Genome-Wide Significant SNPs with Coronary Stenosis in Pakistani Population.

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].

The role of inflammation and genetics in periodontal disease.

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

Plasminogen glycoforms alteration and activation susceptibility associated with the missense variant p.Lys330Glu in HAE-PLG patients.

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

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

Applying whole-genome sequencing in relation to phenotype and outcomes in siblings with cystic fibrosis.

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.

Contribution of Streptokinase-Domains from Groups G and A (SK2a) Streptococci in Amidolytic/Proteolytic Activities and Fibrin-Dependent Plasminogen activation: A Domain-Exchange Study.

In-Frame Indel Mutations in the Genome of the Blind Mexican Cavefish, Astyanax mexicanus.

Hereditary Angio-Oedema for Dermatologists.

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

Genotype-first analysis of a generally healthy population cohort supports genetic testing for diagnosis of hereditary angioedema of unknown cause.

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.

Hereditary angioedema: a Chinese perspective.

Complications of whole-exome sequencing for causal gene discovery in primary platelet secretion defects.

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.