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Disease on EC 3.4.21.42 - complement subcomponent C1s

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis
Effects of C1 inhibitor on tissue damage in a porcine model of controlled hemorrhage.
Acquired Immunodeficiency Syndrome
A dual role for Mannan-binding lectin-associated serine protease 2 (MASP-2) in HIV infection.
Acute Kidney Injury
A Randomized Trial of Recombinant Human C1-Esterase-Inhibitor in the Prevention of Contrast-Induced Kidney Injury.
Activation of both coagulation and fibrinolysis in childhood hemolytic uremic syndrome.
An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female.
Complement Activation in Kidneys of Patients With COVID-19.
Mannan-binding lectin-associated serine protease 2 is critical for the development of renal ischemia reperfusion injury and mediates tissue injury in the absence of complement C4.
The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies.
Adenocarcinoma
Clinical significance of mannose-binding lectin-associated serine protease-2 expression in esophageal squamous cell carcinoma.
Airway Obstruction
C1-esterase inhibitor concentrate prevents upper airway obstruction in hereditary angio-oedema.
Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency.
Critical upper airway obstruction in sporadic angioedema responding to C1-esterase inhibitor.
Treatment for hereditary angioedema with normal C1-INH and specific mutations in the F12 gene (HAE-FXII).
[Episodes of angioedema in children with C1 esterase inhibitor deficiency]
Amyloidosis
Budgetary Impact of Medicinal Therapies for Rare Diseases in Bulgaria.
Anaphylaxis
An 82-year-old man with recurrent angioedema.
C1 esterase inhibitor deficiency in X-linked hypogammaglobulinaemia: an anomaly fostering anaphylactoid reactions following intramuscular gammaglobulin administration.
Chapter 25: Idiopathic anaphylaxis.
Idiopathic anaphylaxis.
Role of complement in a murine model of peanut-induced anaphylaxis.
[Idiopathic anaphylaxis]
Anemia, Hemolytic
Inhibition of complement C1s improves severe hemolytic anemia in cold agglutinin disease: a first-in-human trial.
Anemia, Hemolytic, Autoimmune
Acquired C1 esterase deficiency with cold agglutinin disease associated with monoclonal IgM antibodies.
C1-esterase inhibitor concentrate rescues erythrocytes from complement-mediated destruction in autoimmune hemolytic anemia.
Inhibition of complement C1s improves severe hemolytic anemia in cold agglutinin disease: a first-in-human trial.
Inhibition of complement C1s in patients with cold agglutinin disease: lessons learned from a named patient program.
Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma.
TNT003, an inhibitor of the serine protease C1s, prevents complement activation induced by cold agglutinin disease patient autoantibodies.
Angina, Unstable
[Evaluation of C3c, C4 and C1-esterase inhibitor (C1-INH) during unstable angina]
Angioedema
'Epinephrine-resistant' angioedema.
A Case of Angioedema Due to Acquired C1 Esterase Inhibitor Deficiency Masquerading as Suspected Peritonitis: A Case Report.
A patient with chronic lymphocytic leukemia and acquired angioedema: correlation of clinical and biochemical response to CLL therapy.
Acquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma.
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool.
Acquired angioedema: observations on the mechanism of action of autoantibodies directed against C1 esterase inhibitor.
Acquired C1 esterase inhibitor deficiency and angioedema: a review.
Acquired C1 esterase inhibitor deficiency causing intestinal angioedema: CT appearance.
Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration.
Acquired C1-esterase inhibitor deficiency: three case reports and commentary on the syndrome.
Acute upper airway angioedema secondary to acquired C1 esterase inhibitor deficiency: a case report.
Acute upper airway obstruction from acquired angioedema.
Administration of conestat alfa, human C1 esterase inhibitor and icatibant in the treatment of acute angioedema attacks in adults with hereditary angioedema due to C1 esterase inhibitor deficiency. Treatment comparison based on systematic review results.
Advances in basic and clinical immunology in 2009.
Allergic urticaria and hereditary angioedema. Independent association in the same patient.
An 82-year-old man with recurrent angioedema.
An evidence based therapeutic approach to hereditary and acquired angioedema.
An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female.
An update on the diagnosis and management of hereditary angioedema with abnormal c1 inhibitor.
Anaesthetic management of an emergency caesarean section in a complex immunologically compromised patient with pre-eclampsia and multifactorial thrombocytopaenia.
Angioedema and acquired C1 esterase inhibitor deficiency.
Angioedema as a predominant symptom of Bordetella pertussis infection.
Angioedema as the first presentation of B-cell non-Hodgkin lymphoma--an unusual case with normal C1 esterase inhibitor level: a case report.
Angioedema due to acquired C1-esterase inhibitor deficiency in a patient with Helicobacter pylori infection.
Angioedema due to Systemic Isotretinoin Therapy.
Angioedema in a patient with c1 esterase inhibitor deficiency.
Angioedema in pediatric heart transplant recipients-reporting C1-esterase inhibitor deficiency without analysing protein.
Angioedema in the emergency department: a practical guide to differential diagnosis and management.
Angioedema in the emergency department: a presentation of lymphoma.
Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency.
Angioedema related to angiotensin-converting enzyme inhibitors.
Angioedema secondary to angiotensin converting enzyme inhibitors is not due to C1 esterase inhibitor deficiency.
Angioedema suppressed by a combination of anti-histamine and leukotriene modifier.
Angioedema triggered by pulp extirpation-a case report.
Angioedema.
Angioedema: frightening and frustrating.
Angioedema: manifestations and management.
Angiotensin-converting enzyme inhibitor-associated angioedema treated with c1-esterase inhibitor: A case report and review of the literature.
Angiotensin-converting enzyme inhibitors-induced angioedema treated by C1 esterase inhibitor concentrate (Berinert®): about one case and review of the therapeutic arsenal.
Antiepileptic drug-induced lymphoproliferative disorder associated with acquired C1 esterase inhibitor deficiency and angioedema.
Autoimmune acquired form of angioedema that responded to danazol therapy.
C1 Esterase Inhibitor (Berinert) for ACE Inhibitor-Induced Angioedema: Two Case Reports.
C1 esterase inhibitor concentrate in 1085 Hereditary Angioedema attacks--final results of the I.M.P.A.C.T.2 study.
C1 Esterase Inhibitor for Ace-Inhibitor Angioedema: A Case Series and Literature Review.
C1 esterase inhibitor for angiotensin-converting enzyme inhibitor-induced angioedema at a community teaching health system: A brief retrospective propensity-matched cohort study.
C1 esterase inhibitor use in the management of lisinopril-induced angioedema: A case series.
C1 esterase inhibitor: clinical clues to the pathophysiology of angioedema.
C1-esterase inhibitor deficiency in pediatric heart transplant recipients: incidence and findings on ultrasound.
C1-esterase inhibitor transfusions in patients with hereditary angioedema.
Correction of recurrent angioedema related to C1 esterase inhibitor deficiency as a secondary event following nonmyeloablative allogeneic stem cell transplantation in a patient with myelofibrosis.
Cost-utility analysis of Ruconest(®) (conestat alfa) compared to Berinert(®) P (human C1 esterase inhibitor) in the treatment of acute, life-threatening angioedema attacks in patients with hereditary angioedema.
Critical upper airway obstruction in sporadic angioedema responding to C1-esterase inhibitor.
Does heparin prophylaxis prevent exacerbations of hereditary angioedema?
DX-88 and HAE: a developmental perspective.
Effectiveness of C1-INH therapy in angiotensin converting enzyme inhibitor induced angioedema.
Efficacy of Treatment of Non-hereditary Angioedema.
Epidemiology of Non-hereditary Angioedema.
Exacerbation of a lupus-erythematosus-like syndrome during treatment of non-C1-esterase-inhibitor-dependent angioedema with danazol.
Examination of baseline levels of carboxypeptidase N and complement components as potential predictors of angioedema associated with the use of an angiotensin-converting enzyme inhibitor.
Exposure-Response Model of Subcutaneous C1-Inhibitor Concentrate to Estimate the Risk of Attacks in Patients With Hereditary Angioedema.
Facial angioedema and systemic lupus erythematosus: case report.
Helicobacter pylori infection and skin diseases.
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.
Hereditary angioedema as a cause of transient abdominal pain.
Hereditary angioedema due to deficit of C1 esterase inhibitor.
Hereditary angioedema: a family study.
Human parvovirus infection: rheumatic manifestations, angioedema, C1 esterase inhibitor deficiency, ANA positivity, and possible onset of systemic lupus erythematosus.
Idiopathic nonhistaminergic angioedema successfully treated with ecallantide, icatibant, and C1 esterase inhibitor replacement.
Intestinal angioedema caused by an acquired C1 esterase inhibitor deficiency associated with underlying splenic marginal zone lymphoma.
Intractable acquired autoimmune angioedema in a patient with systemic lupus erythematosus.
Isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature.
Laboratory testing for C1 inhibitor deficiency: a comparison of two approaches to C1 inhibitor function.
Laparoscopic splenectomy in a patient with acquired angioneurotic edema.
Long-standing angioedema with C1 esterase inhibitor deficiency associated with occult lymphoma.
Management of dental-oral procedures in patients with hereditary angioedema due to C1 inhibitor deficiency.
Molecular diagnosis and management of hereditary angioedema in a Greek family.
Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract.
Monoclonal gammopathy with significance: case series and literature review.
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema?
Off-Label Use of Agents for Management of Serious or Life-threatening Angiotensin Converting Enzyme Inhibitor-Induced Angioedema.
Oral medicine case book 64: Some aspects of the pathophysiology of angioedema with special reference to the upper aerodigestive tract.
Perioperative course in patients with hereditary or acquired angioedema.
Perioperative management of tooth extractions for a patient with hereditary angioedema.
Peripheral edema due to increased vascular permeability: a clinical appraisal.
Prognostic factors in outcome of angioedema in the emergency department.
Recurrent angioedema and the threat of asphyxiation.
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency.
rhC1INH: a new drug for the treatment of attacks in hereditary angioedema caused by C1-inhibitor deficiency.
Safety and efficacy of C1 esterase inhibitor for acute attacks in children with hereditary angioedema.
The Genetics of Hereditary Angioedema: A Review.
The isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature.
The late onset form of C1 esterase-inhibitor deficiency presenting as food allergy.
The Role of C1-Esterase Inhibitors in the Management of Vasogenic Edema in Glioblastoma.
The Use of Plasma-Derived Complement C1-Esterase Inhibitor Concentrate (Berinert®) in the Treatment of Angiotensin Converting Enzyme-Inhibitor Related Angioedema.
Treatment of Hereditary Angioedema.
Unmasking of acquired autoimmune C1-inhibitor deficiency by an angiotensin-converting enzyme inhibitor.
Unusual Case of a Massive Macroglossia Secondary to Myxedema: A Case Report and Literature Review.
Update on the acute treatment of hereditary angioedema.
Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema.
Urticaria and angioedema.
Urticaria and angioedema: diagnosis and evaluation.
[A patient with angioedema due to acquired C1 esterase inhibitor deficiency]
[Acquired angioneurotic edema caused by acquired deficiency of C1 esterase inhibitor disclosing lymphoproliferative syndrome. Apropos of a case, review of the literature]
[Acquired C1-esterase inhibitor deficiency with angioedema in lymphoplasmocytic lymphoma]
[Acquired C1 esterase inhibitor deficiency via bradykinin-mediated angioedema: Four cases].
[Anaesthesic management of vaginal delivery in a parturient with C1 esterase deficiency]
[Anesthesia and perioperative management in a patient with acquired angioedema].
[Angioedema caused by C1 esterase inhibitor deficiency]
[Angioedema caused by C1-esterase inhibitor deficiency or ACE inhibitors?]
[Angioedema: first manifestation of non-Hodgkin's lymphoma]
[Angioneurotic edema in hereditary alpha-1-antitrypsin deficiency]
[Angioneurotic orolingual edema associated with the use of rt-PA following a stroke.]
[C1-esterase inhibitor in ACE inhibitor-induced severe angioedema of the tongue]
[Correction of deficiency of C1 esterase inhibitor in angioneurotic edema with oxymetholone]
[Episodes of angioedema in children with C1 esterase inhibitor deficiency]
[Hereditary angioedema due to C1-esterase inhibitor deficiency?: novel approaches].
[Hereditary angioedema of delayed onset]
[Hereditary deficiency of C1-esterase inhibitor presenting with recurrent abdominal pain]
[Immunocytoma with acquired C1-esterase inhibitor deficiency and recurrent angioneurotic edema]
[Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor]
Angioedemas, Hereditary
A Case of Angioedema Due to Acquired C1 Esterase Inhibitor Deficiency Masquerading as Suspected Peritonitis: A Case Report.
A case of hereditary angioedema in a 7-year-old korean girl.
A case of hereditary angioedema who presented with difficulty in urination and globe.
A Case of Type 2 Hereditary Angioedema With SERPING1 Mutation.
A Decade of Change: Recent Developments in Pharmacotherapy of Hereditary Angioedema (HAE).
A first of its kind quantitative functional C1-esterase inhibitor lateral flow assay for hereditary angioedema point-of-care diagnostic testing.
A novel functional C1 inhibitor activity assay in dried blood spot for diagnosis of Hereditary angioedema.
A novel series of arylsulfonylthiophene-2-carboxamidine inhibitors of the complement component C1s.
A rare cause of recurrent priapism: hereditary angioedema.
A recombinant C1 esterase inhibitor (Ruconest) for hereditary angioedema.
A review of kallikrein inhibitor lanadelumab in hereditary angioedema.
A review of the reported defects in the human C1 esterase inhibitor gene producing hereditary angioedema including four new mutations.
A robust multiplexed assay to quantify C1-inhibitor, C1q, and C4 proteins for in vitro diagnosis of hereditary angioedema from dried blood spot.
Acquired and hereditary forms of recurrent angioedema: Update of treatment.
Acquired C1 esterase inhibitor deficiency and angioedema: a review.
Acquired C1 esterase inhibitor deficiency as manifestation of T-cell lymphoproliferative disorder.
Acquired C1 esterase inhibitor deficiency causing intestinal angioedema: CT appearance.
Acquired C1 esterase inhibitor deficiency in a marginal zone lymphoma patient treated with rituximab.
Acquired C1 esterase inhibitor deficiency in lymphomas: prevalence, symptoms, and response to treatment.
Acquired C1 esterase inhibitor deficiency in two patients presenting with a lupus-like syndrome and anticardiolipin antibodies.
Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration.
Acquired C1 esterase inhibitor deficiency.
Acquired C1 Esterase Inhibitor Deficiency.
Acquired C1 esterase inhibitor deficiency: An atypical first presentation intra-operatively.
Acquired C1 inhibitor deficiency: postmortem diagnosis.
Acquired complement C1 esterase inhibitor deficiency in a patient with a rare SERPING1 variant with unknown significance.
Acute Genital Swelling Heralding C1 Esterase Inhibitor Deficiency in a Child.
Acute pancreatitis due to hereditary angioedema.
Acute upper airway angioedema secondary to acquired C1 esterase inhibitor deficiency: a case report.
Acute upper airway obstruction from acquired angioedema.
Administration of conestat alfa, human C1 esterase inhibitor and icatibant in the treatment of acute angioedema attacks in adults with hereditary angioedema due to C1 esterase inhibitor deficiency. Treatment comparison based on systematic review results.
Advances in basic and clinical immunology in 2009.
Allergic urticaria and hereditary angioedema. Independent association in the same patient.
An adverse reaction to angiotensin-converting enzyme inhibitors in a patient with neglected C1 esterase inhibitor deficiency.
An association between C1 esterase inhibitor deficiency and lupus erythematosus: report of two cases and review of the literature.
An evidence based therapeutic approach to hereditary and acquired angioedema.
An investigational RNAi therapeutic targeting Factor XII (ALN-F12) for the treatment of hereditary angioedema.
An update on the diagnosis and management of hereditary angioedema with abnormal c1 inhibitor.
Anaesthesia Management of a Patient with Hereditary Angioedema with Prophylactic Administration of C1 Esterase Inhibitor: Case report and literature review.
Anaesthetic Management of a Patient with Hereditary Angioedema.
Angio-oedema--? acquired C1 esterase inhibitor deficiency.
Angioedema and acquired C1 esterase inhibitor deficiency.
Angioedema in a child with a liver transplant, intussusception, and normal c4 levels.
Angioedema in a patient with c1 esterase inhibitor deficiency.
Angioedema in the emergency department: a presentation of lymphoma.
Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency.
Angioedema presenting in the retropharyngeal space in an adult.
Angioedema secondary to angiotensin converting enzyme inhibitors is not due to C1 esterase inhibitor deficiency.
Angioedema suppressed by a combination of anti-histamine and leukotriene modifier.
Angioedema: frightening and frustrating.
Angioedema: manifestations and management.
Angiotensin-converting enzyme (ACE) inhibitors and angio-oedema.
Angiotensin-converting enzyme inhibitors-induced angioedema treated by C1 esterase inhibitor concentrate (Berinert®): about one case and review of the therapeutic arsenal.
Antiepileptic drug-induced lymphoproliferative disorder associated with acquired C1 esterase inhibitor deficiency and angioedema.
Asphyxiation by laryngeal edema in patients with hereditary angioedema.
Biochemical comparison of four commercially available C1 esterase inhibitor concentrates for treatment of hereditary angioedema.
Biphenylsulfonyl-thiophene-carboxamidine inhibitors of the complement component C1s.
Budgetary Impact of Medicinal Therapies for Rare Diseases in Bulgaria.
C1 esterase inhibitor activity is reduced in the acute phase following burn injury: a prospective observational study.
C1 esterase inhibitor concentrate in 1085 Hereditary Angioedema attacks--final results of the I.M.P.A.C.T.2 study.
C1 esterase inhibitor deficiency as a cause of abdominal pain.
C1 esterase inhibitor deficiency in X-linked hypogammaglobulinaemia: an anomaly fostering anaphylactoid reactions following intramuscular gammaglobulin administration.
C1 esterase inhibitor deficiency, airway compromise, and anesthesia.
C1 esterase inhibitor deficiency: three presentations.
C1 esterase inhibitor transfusions in patients with hereditary angioedema.
C1-esterase inhibitor for short-term prophylaxis in a patient with hereditary angioedema with normal C1 inhibitor function.
C1-esterase inhibitor transfusions in patients with hereditary angioedema.
C1-esterase inhibitor treatment: preclinical safety aspects on the potential prothrombotic risk.
C1-Esterase inhibitor: an anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema.
Calcium channel blocker-associated small bowel angioedema.
Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report.
Chapter 22: Hereditary and acquired angioedema.
Chapter 25: Idiopathic anaphylaxis.
Characteristics of patients with C1 esterase inhibitor deficiency: a single center study.
Characterization of acute hereditary angioedema attacks during pregnancy and breast-feeding and their treatment with C1 inhibitor concentrate.
Cinryze as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety.
Cinryze, a Human Plasma-Derived C1 Esterase Inhibitor for Prophylaxis Of Hereditary Angioedema.
Clinical Pattern and Acute and Long-term Management of Hereditary Angioedema Due to C1-Esterase Inhibitor Deficiency.
Clinical review of hereditary angioedema: diagnosis and management.
Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency.
Co-occurrence between C1 esterase inhibitor deficiency and autoimmune disease: a systematic literature review.
Complement activation during bypass in acquired C1 esterase inhibitor deficiency.
Complement analysis in adult patients with a history of bacteremic pneumococcal infections or recurrent pneumonia.
Complement component analysis in angiodema. Diagnostic value.
Complement deficiency and disease: an update.
Complement Inhibition for Prevention and Treatment of Antibody-Mediated Rejection in Renal Allograft Recipients.
Conestat alfa (ruconest): first recombinant c1 esterase inhibitor for the treatment of acute attacks in patients with hereditary angioedema.
Contraindications to the use of ace inhibitors in patients with C1 esterase inhibitor deficiency.
Correction of recurrent angioedema related to C1 esterase inhibitor deficiency as a secondary event following nonmyeloablative allogeneic stem cell transplantation in a patient with myelofibrosis.
Cost-utility analysis of Ruconest(®) (conestat alfa) compared to Berinert(®) P (human C1 esterase inhibitor) in the treatment of acute, life-threatening angioedema attacks in patients with hereditary angioedema.
CT Findings of Acute Small-Bowel Entities.
Current and Prospective Targets of Pharmacologic Treatment of Hereditary Angioedema Types 1 and 2.
Current management options for hereditary angioedema.
Current options for prophylactic treatment of hereditary angioedema in the United States: patient-based considerations.
Danazol and C1 esterase inhibitor deficiency.
Death due to obstruction of the upper airways caused by edema of the laryngeal mucosa in the course of hereditary angioedema.
Diagnosis and management of angioedema with abdominal involvement: a gastroenterology perspective.
Diminished capacity of opsonization and immune complex solubilization, and detection of anti-C1q antibodies in sera from patients with hereditary angioedema.
Discrepancies between guidelines and international practice in treatment of hereditary angioedema.
Does heparin prophylaxis prevent exacerbations of hereditary angioedema?
DX-88 and HAE: a developmental perspective.
Ecallantide for the treatment of hereditary angiodema in adults.
Ecallantide for treatment of acute attacks of acquired C1 esterase inhibitor deficiency.
Effect of C1 esterase inhibitor in hereditary angioedema treatment.
Effect of dehydroepiandrosterone on hereditary angioedema.
Effect of time to treatment on response to C1 esterase inhibitor concentrate for hereditary angioedema attacks.
Effects of a plasma-derived C1 esterase inhibitor on hemostatic activation, clot formation, and thrombin generation.
Efficacy Assessments in Randomized Controlled Studies of Acute Therapy for Hereditary Angioedema.
Efficacy of C1 esterase inhibitor concentrate in treatment of cutaneous attacks of hereditary angioedema.
Efficacy of Different Medical Therapies for the Treatment of Acute Laryngeal Attacks of Hereditary Angioedema due to C1-esterase Inhibitor Deficiency.
Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks.
Efficacy of recombinant human C1 esterase inhibitor across anatomic locations in acute hereditary angioedema attacks.
Efficacy of recombinant human C1 esterase inhibitor for the treatment of severe hereditary angioedema attacks.
Elevated D-dimers in attacks of hereditary angioedema are not associated with increased thrombotic risk.
Escalating doses of C1 esterase inhibitor (CINRYZE) for prophylaxis in patients with hereditary angioedema.
Exacerbation of a lupus-erythematosus-like syndrome during treatment of non-C1-esterase-inhibitor-dependent angioedema with danazol.
Exposure-Response Model of Subcutaneous C1-Inhibitor Concentrate to Estimate the Risk of Attacks in Patients With Hereditary Angioedema.
Factor XII-Driven Inflammatory Reactions with Implications for Anaphylaxis.
Falsely normal C4 in a case of acquired C1 esterase inhibitor deficiency.
Feasibility of home infusion and self-administration of nanofiltered C1 esterase inhibitor for routine prophylaxis in patients with hereditary angioedema and characterization of a training and support program.
Fresh frozen plasma for on-demand hereditary angioedema treatment in South Africa and Iran.
Gene Therapy for C1 Esterase Inhibitor Deficiency in a Murine Model of Hereditary Angioedema.
General and subarachnoid anaesthesia in a patient with acquired C1 esterase inhibitor deficiency.
Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema.
Glomerulonephritis and hereditary angioedema: report of 2 cases.
Guillain-Barré syndrome following danazol and corticosteroid therapy for hereditary angioedema.
HAEdb: a novel interactive, locus-specific mutation database for the C1 inhibitor gene.
Haegarda--a subcutaneous C1 esterase inhibitor for prevention of hereditary angioedema.
Hereditary and acquired angioedema.
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.
Hereditary and acquired complement component 1 esterase inhibitor deficiency: a review for the hematologist.
Hereditary angioedema and pregnancy.
Hereditary angioedema and pregnancy: a successful outcome using C1 esterase inhibitor concentrate.
Hereditary angioedema and pregnancy: successful management of recurrent and frequent attacks of angioedema with C1-inhibitor concentrate, danazol and tranexamic acid - a case report.
Hereditary angioedema associated with heterozygous factor V Leiden mutation in a patient with Purpura fulminans.
Hereditary Angioedema Attack in Utero and Treatment of the Mother and Fetus.
Hereditary angioedema C1-esterase inhibitor replacement therapy and coexisting autoimmune disorders: findings from a claims database.
Hereditary angioedema caused by c1-esterase inhibitor deficiency: a literature-based analysis and clinical commentary on prophylaxis treatment strategies.
Hereditary angioedema due to deficit of C1 esterase inhibitor.
Hereditary angioedema managed with low-dose danazol and c1 esterase inhibitor concentrate: a case report.
Hereditary angioedema presenting as epiglottitis.
Hereditary angioedema therapy: kallikrein inhibition and bradykinin receptor antagonism.
Hereditary angioedema type 2 presented as an orbital complication of acute rhinosinusitis.
Hereditary angioedema type II--a study of two families.
Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases).
Hereditary angioedema with recurrent abdominal pain.
Hereditary angioedema.
Hereditary angioedema. Diagnosis in an asymptomatic elderly woman.
Hereditary angioedema. Report of a large kindred with a rare genetic variant of C1-esterase inhibitor.
Hereditary angioedema: a clinical review for the otolaryngologist.
Hereditary angioedema: a family study.
HEREDITARY ANGIOEDEMA: A Literature Review and National Management Guidelines.
Hereditary angioedema: a Taiwanese family with a novel gene mutation.
Hereditary angioedema: an update on causes, manifestations and treatment.
Hereditary angioedema: imaging manifestations and clinical management.
Hereditary angioedema: new hopes for an orphan disease.
Hereditary angioedema: not an allergy.
Hereditary angioedema: quality of life in Brazilian patients.
Hereditary angioedema: report of a case.
Hereditary angioedema: report of a large kindred with a rare genetic variant of C1-esterase inhibitor.
Hereditary angioedema: therapeutic effect of danazol on C4 and C1 esterase inhibitors.
Hereditary angioneurotic edema (HANE): Lack of close linkage between HLA haplotypes and C1 esterase inhibitor deficiency.
Hereditary angioneurotic edema and functional C1 esterase inhibitor.
Hereditary angioneurotic edema of the larynx.
Hereditary angioneurotic edema treated by partial uvulectomy.
Hereditary angioneurotic oedema: a neglected diagnosis.
High amounts of beta-endorphin in peripheral blood mononuclear cells from HANE patients.
Home therapy with intravenous human C1-inhibitor in children and adolescents with hereditary angioedema.
Hormonally exacerbated hereditary angioedema.
Human C1-esterase inhibitor concentrate (Berinert).
Human parvovirus infection: rheumatic manifestations, angioedema, C1 esterase inhibitor deficiency, ANA positivity, and possible onset of systemic lupus erythematosus.
Icatibant. Attacks of hereditary angioedema: continue to use C1 esterase inhibitor.
Idiopathic anaphylaxis.
In Vitro Modeling of Bradykinin-Mediated Angioedema States.
Increased fibrinolysis-induced bradykinin formation in hereditary angioedema confirmed using stored plasma and biotechnological inhibitors.
Indirect Comparison of Lanadelumab and Intravenous C1-INH Using Data from the HELP and CHANGE Studies: Bayesian and Frequentist Analyses.
Inherited disorders of complement.
Inhibition of the serine proteases of the complement system.
Interactive Web-Based Resource for Annotation of Genetic Variants Causing Hereditary Angioedema (HADA): Database Development, Implementation, and Validation.
Intestinal angioedema caused by an acquired C1 esterase inhibitor deficiency associated with underlying splenic marginal zone lymphoma.
Is there a need for clinical guidelines in the United States for the diagnosis of hereditary angioedema and the screening of family members of affected patients?
Isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature.
Kallikrein-kinin system and fibrinolysis in hereditary angioedema due to factor XII gene mutation Thr309Lys.
Laboratory testing for C1 inhibitor deficiency: a comparison of two approaches to C1 inhibitor function.
Lanadelumab for the prevention of attacks in hereditary angioedema.
Lanadelumab for the Prophylactic Treatment of Hereditary Angioedema with C1 Inhibitor Deficiency: A Review of Preclinical and Phase I Studies.
Long-standing angioedema with C1 esterase inhibitor deficiency associated with occult lymphoma.
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.
Long-term outcomes with subcutaneous C1-inhibitor replacement therapy for prevention of hereditary angioedema attacks.
Long-Term Prophylaxis of Hereditary Angioedema with a Pasteurized C1 Inhibitor Concentrate.
Low molecular weight IgM in B cell lymphoproliferative disorders.
Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma.
Lupus enteritis as the only active manifestation of systemic lupus erythematosus: A case report.
Lymphocytic lymphoma and acquired C1 esterase inhibitor deficiency.
Mast cells increase vascular permeability by heparin-initiated bradykinin formation in vivo.
Model-Based Evaluation of Similarity in Pharmacokinetics of Two Formulations of the Blood-Derived Plasma Product C1 Esterase Inhibitor.
Modern preoperative and intraoperative management of hereditary angioedema.
Modification of peripheral blood T-lymphocyte surface receptors and Langerhans cell numbers in hereditary angioedema.
Molecular diagnosis and management of hereditary angioedema in a Greek family.
Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract.
Mutation screening of the C1 inhibitor gene among Hungarian patients with hereditary angioedema.
Nanofiltered C1 esterase inhibitor (human) for hereditary angioedema attacks in pregnant women.
Nanofiltered C1 esterase inhibitor (human) for the treatment of acute attacks of hereditary angioedema: an open-label trial.
Nanofiltered C1 esterase inhibitor for treatment of laryngeal attacks in patients with hereditary angioedema.
Nanofiltered C1-Esterase Inhibitor for the Acute Management and Prevention of Hereditary Angioedema Attacks due to C1-Inhibitor Deficiency in Children.
Nephelometric and immunodiffusion assays for the fourth component of complement in acquired C1 esterase inhibitor deficiency.
New insights into hereditary angio-oedema: Molecular diagnosis and therapy.
New mutations in C1 esterase inhibitor (SERPING1) in a German family with hereditary angioedema.
New-variant hereditary angioedema in three brothers with normal C1 esterase inhibitor level and function.
Novel Use of Fresh Frozen Plasma in Treating Hereditary Angioedema: A Success Story From Pakistan.
Off-pump coronary artery bypass surgery in a patient with C1 esterase inhibitor deficiency.
Ongoing contact activation in patients with hereditary angioedema.
Optimizing hereditary angioedema management through tailored treatment approaches.
Oral Plasma Kallikrein Inhibitor for Prophylaxis in Hereditary Angioedema.
Orthodontic treatment for a patient with hereditary angiodema: a case report.
Pasteurized and nanofiltered, plasma-derived C1 esterase inhibitor concentrate for the treatment of hereditary angioedema.
Peptide inhibitor of complement c1, a novel suppressor of classical pathway activation: mechanistic studies and clinical potential.
Peri-operative management of a patient with hereditary angioedema undergoing laparoscopic cholecystectomy.
Perioperative course in patients with hereditary or acquired angioedema.
Perioperative management for patients with hereditary angioedema.
Perioperative management of a patient with hereditary angioedema during off-pump coronary artery bypass graft surgery.
Perioperative Management of Patients With Hereditary Angioedema With Special Considerations for Cardiopulmonary Bypass.
Perioperative management of tooth extractions for a patient with hereditary angioedema.
Pharmaceutical approval update.
Pharmacokinetics of plasma-derived C1-esterase inhibitor after subcutaneous versus intravenous administration in subjects with mild or moderate hereditary angioedema: the PASSION study.
Plasma bradykinin in angio-oedema.
Plasmin is a natural trigger for bradykinin production in patients with hereditary angioedema with factor XII mutations.
Population pharmacokinetics of plasma-derived C1 esterase inhibitor concentrate used to treat acute hereditary angioedema attacks.
Population pharmacokinetics of recombinant human C1 esterase inhibitor in children with hereditary angioedema.
Population pharmacokinetics of subcutaneous C1-inhibitor for prevention of attacks in patients with hereditary angioedema.
Possible disease-modifying factors: the mannan-binding lectin pathway and infections in hereditary angioedema of children and adults.
Practical approach to self-administration of intravenous C1-INH concentrate: a nursing perspective.
Practicalities of a reduced volume formulation of a C1-INH concentrate for the treatment of hereditary angioedema: real-life experience.
Pregnancy and C1 esterase inhibitor deficiency: a successful outcome.
Pregnancy-Induced Exacerbation of Hereditary Angioedema in a Multiparous Caucasian Female.
Preprocedural administration of nanofiltered C1 esterase inhibitor to prevent hereditary angioedema attacks.
Prevalence of hereditary angioedema in untested first-degree blood relatives of known subjects with hereditary angioedema.
Prognostic factors in outcome of angioedema in the emergency department.
Progress in the emergency management of hereditary angioedema: focus on new treatment options in the United States.
Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks.
Prospective study of rapid relief provided by c1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema.
Randomized Trial of the Efficacy and Safety of Berotralstat (BCX7353) as an Oral Prophylactic Therapy for Hereditary Angioedema: Results of APeX-2 Through 48 Weeks (Part 2).
Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor.
Recognition, Evaluation, and Management of Pediatric Hereditary Angioedema.
Recombinant human C1 esterase inhibitor (Conestat alfa) for prophylaxis to prevent attacks in adult and adolescent patients with hereditary angioedema.
Recombinant human C1 esterase inhibitor as short-term prophylaxis in patients with hereditary angioedema.
Recombinant human C1 esterase inhibitor for acute hereditary angioedema attacks with upper airway involvement.
Recombinant human C1 esterase inhibitor for hereditary angioedema attacks: A European registry.
Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE).
Recombinant human C1 esterase inhibitor in the management of hereditary angioedema.
Recombinant human C1 esterase inhibitor treatment for hereditary angioedema attacks in children.
Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial.
Recombinant Human C1-Esterase Inhibitor to Treat Acute Hereditary Angioedema Attacks in Adolescents.
Recombinant Human-C1 Inhibitor is Effective and Safe for Repeat Hereditary Angioedema Attacks.
Recombinant replacement therapy for hereditary angioedema due to C1 inhibitor deficiency.
Recurrent angioedema and the threat of asphyxiation.
Recurrent angioedema: Experience at a tertiary care urban medical center.
Recurrent angioedema: familial and oestrogen-induced.
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency.
Repeated attacks of type III hereditary angioedema with factor XII mutation during pregnancy.
Replacement therapy with C1 esterase inhibitors for hereditary angioedema.
Safety and efficacy of C1 esterase inhibitor for acute attacks in children with hereditary angioedema.
Safety of c1-esterase inhibitor in acute and prophylactic therapy of hereditary angioedema: findings from the ongoing international berinert patient registry.
Safety of recombinant human C1 esterase inhibitor for hereditary angioedema attacks during pregnancy.
Self-administered C1 esterase inhibitor concentrates for the management of hereditary angioedema: usability and patient acceptance.
Self-administration of intravenous C1 esterase inhibitor (Berinertâ) in patients with Hereditary Angioedema decreases number of days spent in an emergency room.
Self-administration of intravenous C1 esterase inhibitor in hereditary angioedema.
Social costs of icatibant self-administration vs. health professional-administration in the treatment of hereditary angioedema in Spain.
Some properties of proteolysis by polymorphonuclear leukocyte-granule extracts.
Sonographic appearances of the abdominal manifestations of hereditary angioedema.
Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema.
Study of angiopoietin and plasminogen genes in hereditary angioedema.
Subcutaneous C1 inhibitor for prevention of attacks of hereditary angioedema: additional outcomes and subgroup analysis of a placebo-controlled randomized study.
Subcutaneous C1-esterase inhibitor to prevent hereditary angioedema attacks: Safety findings from the COMPACT trial.
Successful anesthetic management during abdominal wall-lifting laparoscopic cholecystectomy in a patient with hereditary angioedema.
Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema.
Successful extubation with use of C1 esterase inhibitor concentrate in a patient with hereditary angioedema.
Successful Long-Term Prophylactic Treatment With Subcutaneous C1 Esterase Inhibitor in a Patient With Hereditary Angioedema.
Successful long-term treatment with the bradykinin B2 receptor antagonist icatibant in a patient with hereditary angioedema.
Successful management of hereditary angioedema during tonsillectomy: a case report.
Successful perinatal management of hereditary angioedema with normal C1 esterase inhibitor and factor XII mutation using C1 esterase inhibitor therapy.
Successful perioperative management of a patient with C1 esterase inhibitor deficiency with a novel bradykinin receptor B2 antagonist.
Successful perioperative management of three patients with hereditary angioedema without C1 esterase inhibitor therapy: A developing country perspective.
Successful resolution of bowel obstruction in a patient with hereditary angioedema.
Successful treatment of acute hereditary angioedema attacks with self-administered icatibant in patients with venous access problems.
Successful use of C1 esterase inhibitor protein in a patient with hereditary angioneurotic edema requiring coronary artery bypass surgery.
Successful use of daily intravenous infusion of C1 esterase inhibitor concentrate in the treatment of a hereditary angioedema patient with ascites, hypovolemic shock, sepsis, renal and respiratory failure.
Supplementation of C1-esterase inhibitor concentrates for a patient suffering from hereditary angioedema undergoing complex open-heart surgery.
Sustained response of recombinant human C1 esterase inhibitor for acute treatment of hereditary angioedema attacks.
The C1 esterase inhibitor and hereditary angioedema.
The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema.
The Effect of Weight on the Efficacy and Safety of C1 Esterase Inhibitor Concentrate for the Treatment of Acute Hereditary Angioedema.
The Effectiveness and Value of Lanadelumab and C1 Esterase Inhibitors for Prophylaxis of Hereditary Angioedema Attacks.
The isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature.
The spectrum and treatment of angioedema.
The thrombogenicity of C1 esterase inhibitor (human): review of the evidence.
The value of C1 esterase inhibitor in patients with aspirin-sensitive urticaria.
Therapeutic inhibition of the complement system. Y2K update.
Thrombotic Events Associated with C1 Esterase Inhibitor Products in Patients with Hereditary Angioedema: Investigation from the United States Food and Drug Administration Adverse Event Reporting System Database.
Thrombotic events associated with C1 esterase inhibitor products in patients with hereditary angioedema: investigation from the United States Food and Drug Administration adverse event reporting system database.
Tissue Plasminogen Activator-Induced Angioedema Involving a Posterior Cerebral Artery Infarct: A Case Presentation.
Training hereditary angioedema patients to self-administer intravenous C1 esterase inhibitor concentrate.
Treatment for hereditary angioedema with normal C1-INH and specific mutations in the F12 gene (HAE-FXII).
Treatment of hereditary angioedema with nanofiltered C1-esterase inhibitor concentrate (Cetor®): Multi-center phase II and III studies to assess pharmacokinetics, clinical efficacy and safety.
Treatment of hereditary angioedema with plasma-derived C1 inhibitor.
Treatment of Hereditary Angioedema.
Treatment of Hereditary Angioedema: items that need to be addressed in practice parameter.
Treatment response after repeated administration of C1 esterase inhibitor for successive acute hereditary angioedema attacks.
Treatment with C1-esterase inhibitor concentrate in type I or II hereditary angioedema: a systematic literature review.
Type I hereditary angioedema in Taiwan -- clinical, biological features and genetic study.
Unusual manifestations of hereditary angioedema.
Update on preventive therapy (prophylaxis) of hereditary angioedema.
Update on the acute treatment of hereditary angioedema.
Update on the Use of C1-Esterase Inhibitor Replacement Therapy in the Acute and Prophylactic Treatment of Hereditary Angioedema.
Urticaria and angioedema.
Urticaria and angioedema: diagnosis and evaluation.
Urticarial vasculitis and asymptomatic acquired C1 esterase inhibitor deficiency revealing an angioimmunoblastic T cell lymphoma.
Urticarial vasculitis, IgA deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report.
Use of purified C1 esterase inhibitor in patients with hereditary angioedema.
Usefulness of C1 esterase inhibitor protein concentrate in the management of hereditary angioedema of oropharyngeal tissue.
When is prophylaxis for hereditary angioedema necessary?
[67-year-old patient with speech disorder and dysphagia]
[A case of lung cancer with hereditary angioedema treated effectively by chemo-radiotherapy with C1 esterase inhibitor concentrate and danazol]
[A patient with angioedema due to acquired C1 esterase inhibitor deficiency]
[Acquired C1 esterase inhibitor deficiency and lymphoproliferative syndromes]
[Acquired C1 esterase inhibitor deficiency, Gougerot-Sjögren syndrome and B lymphoma]
[Angioedema caused by C1 esterase inhibitor deficiency]
[Attacks of abdominal pain in a young Turkish woman with C1 esterase inhibitor deficiency]
[Behavior of esterase inhibitor C1 in patients with urticaria due to aspirin hypersensitivity]
[C1 esterase inhibitor deficiency as a rare cause of recurrent acute abdominal pain]
[Caesarean section in a parturient with C1 esterase inhibitor deficiency (angioneurotic oedema)]
[Clinical facets of hereditary angioedema among Swiss patients].
[Croup syndrome]
[Efficacy of short-term danazol treatment in hereditary angioneurotic edema patients undergoing maxillofacial and dental surgical procedures]
[Episodes of angioedema in children with C1 esterase inhibitor deficiency]
[Hereditary angioedema by defict of C1 esterase. Our experience in 8 cases]
[Hereditary angioedema caused by a deficiency of C1 esterase inhibitor. A sporadic case of late appearance]
[Hereditary angioedema due to C1-esterase inhibitor deficiency]
[Hereditary angioedema in Costa Rica].
[Hereditary angioedema in the German-speaking region]
[Hereditary angioedema of delayed onset]
[Hereditary angioedema, a rare cause of recurrent abdominal pains. A report of 2 clinical cases and comments of a general nature]
[Hereditary angioedema--neglected diagnosis].
[Hereditary angioedema. A report of a case and literature review]
[Hereditary angioedema. Description of a pediatric case]
[Hereditary angioedema. Effect of danazol on C4 and functional C1INH]
[Hereditary angioneurotic edema in children]
[Hereditary angioneurotic edema: a molecular disease caused by a defect in the O-glycosylation of C1 esterase inhibitor (C1-INH)]
[Hereditary C1 esterase inhibitor deficiency type I. Divergence of clinical symptoms and laboratory chemical findings]
[Hereditary deficiency of C1-esterase inhibitor presenting with recurrent abdominal pain]
[Mixed nodular lymphoma in association with a C1 esterase inhibitor deficiency (author's transl)]
[New possibilities of treating acute angioedema caused by C1-inhibitor deficiency]
[Recurrent attacks of angioedema ascribed to the use of estrogen preparations and a pregnancy (hereditary angioedema type 3)]
[Simultaneous occurrence of hereditary angioneurotic edema and Crohn disease]
[Sonography in hereditary angioedema: typical findings demonstrated by the example of 3 cases]
[Study by means of enzymatic and immunochemical determination of Cl esterase inhibitor in 59 patients with hereditary angioneurotic edema]
[Therapeutic approach of hereditary angioedema]
Arthritis
Key Components of the Complement Lectin Pathway Are Not Only Required for the Development of Inflammatory Arthritis but Also Regulate the Transcription of Factor D.
Arthritis, Rheumatoid
Association of MASP-2 Levels and MASP2 Gene Polymorphisms with Rheumatoid Arthritis in Patients and Their Relatives.
Complement C1s activation in degenerating articular cartilage of rheumatoid arthritis patients: immunohistochemical studies with an active form specific antibody.
Complement components C1q, C1r/C1s, and C1INH in rheumatoid arthritis. Correlation of in situ hybridization and northern blot results with function and protein concentration in synovium and primary cell cultures.
[Concentration of acute phase proteins in serum of children with rheumatoid arthritis]
Asthma
Human plasma-derived C1 esterase inhibitor concentrate has limited effect on house dust mite-induced allergic lung inflammation in mice.
Atherosclerosis
Clinical and experimental approaches to the prevention of atherosclerosis by immunological regulations.
Distinct Longitudinal Associations of MBL, MASP-1, MASP-2, MASP-3, and MAp44 With Endothelial Dysfunction and Intima-Media Thickness: The Cohort on Diabetes and Atherosclerosis Maastricht (CODAM) Study.
Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction.
Atypical Hemolytic Uremic Syndrome
The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies.
Autoimmune Diseases
Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
Co-occurrence between C1 esterase inhibitor deficiency and autoimmune disease: a systematic literature review.
Hereditary angioedema C1-esterase inhibitor replacement therapy and coexisting autoimmune disorders: findings from a claims database.
Should MASP-2 Deficiency Be Considered a Primary Immunodeficiency? Relevance of the Lectin Pathway.
Bacteremia
Activation and inhibition of Hageman factor-dependent pathways and the complement system in uncomplicated bacteremia or bacterial shock.
Inherited disorders of complement.
Serum Concentrations of Mannan-Binding Lectin (MBL) and MBL-Associated Serine Protease-2 and the Risk of Adverse Events in Pediatric Patients With Cancer and Fever in Neutropenia.
Brain Concussion
Effect of complement C1-esterase inhibitor on brain edema and inflammation after mild traumatic brain injury in an animal model.
Brain Edema
Effect of complement C1-esterase inhibitor on brain edema and inflammation after mild traumatic brain injury in an animal model.
Brain Injuries
Changes of C1 esterase inhibitor, alpha2 globulin and sedimentation rate after severe brain injury.
Mannan binding lectin-associated serine protease-2 (MASP-2) critically contributes to post-ischemic brain injury independent of MASP-1.
Brain Injuries, Traumatic
Targeted deletions of complement lectin pathway genes improve outcome in traumatic brain injury, with MASP-2 playing a major role.
Brain Ischemia
Combination Therapy with Low-Dose IVIG and a C1-esterase Inhibitor Ameliorates Brain Damage and Functional Deficits in Experimental Ischemic Stroke.
Mannan binding lectin-associated serine protease-2 (MASP-2) critically contributes to post-ischemic brain injury independent of MASP-1.
Capillary Leak Syndrome
Validation of C1-esterase inhibitor therapy in severe capillary leak syndrome by monitoring of extravascular lung water.
[Idiopathic anaphylaxis]
[Successful use of C1 esterase inhibitor in capillary leak syndrome].
Carcinogenesis
Clinical significance of mannose-binding lectin-associated serine protease-2 expression in esophageal squamous cell carcinoma.
Carcinoid Tumor
Chapter 25: Idiopathic anaphylaxis.
Idiopathic anaphylaxis.
[Idiopathic anaphylaxis]
Carcinoma
Clinical significance of mannose-binding lectin-associated serine protease-2 expression in esophageal squamous cell carcinoma.
Complement Component 1, s Subcomponent Overexpression is an Independent Poor Prognostic Indicator in Patients with Urothelial Carcinomas of the Upper Urinary Tract and Urinary Bladder.
Noncanonical Functions of C1s Complement Its Canonical Functions in Renal Cancer.
Serum C1-esterase inhibitor, an essential and independent prognosticator of gastric carcinoma.
Up-regulated expression of the MAT-8 gene in prostate cancer and its siRNA-mediated inhibition of expression induces a decrease in proliferation of human prostate carcinoma cells.
Carcinoma, Hepatocellular
Effect of conditioned media of acute myeloid leukemia blast cells on complement synthesis by cultured human cells of monocyte and hepatocyte origin.
Hereditary haemorrhagic telangiectasia: to transplant or not to transplant - is there a right time for liver transplantation?
Carcinoma, Lobular
Elastosis in breast carcinoma: II. Association of protease inhibitors with immature elastic fibres.
Carcinoma, Non-Small-Cell Lung
The Clinical Significance of Serum MASP-2 and IDH1 in the Early Diagnosis of Non-Small Cell Lung Cancer.
Carcinoma, Renal Cell
Noncanonical Functions of C1s Complement Its Canonical Functions in Renal Cancer.
Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis.
Cardiomyopathies
MASP2 haplotypes are associated with high risk of cardiomyopathy in chronic Chagas disease.
Cardiovascular Diseases
MASPs at the crossroad between the complement and the coagulation cascades - the case for COVID-19.
Caroli Disease
Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor.
Cerebrovascular Disorders
MASPs at the crossroad between the complement and the coagulation cascades - the case for COVID-19.
Cervical Intraepithelial Neoplasia
MASP-1 and MASP-2 Serum Levels Are Associated With Worse Prognostic in Cervical Cancer Progression.
Chondrosarcoma
Coordinated change between complement C1s production and chondrocyte differentiation in vitro.
Chronic Urticaria
An 82-year-old man with recurrent angioedema.
Protease inhibitor profiles in urticaria and angio-oedema.
[Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia].
Coinfection
A dual role for Mannan-binding lectin-associated serine protease 2 (MASP-2) in HIV infection.
Colic
Analysis of coagulation proteins as acute-phase reactants in horses with colic.
Colitis, Ulcerative
High normal serum levels of C3 and C1 inhibitor, two acute-phase proteins belonging to the complement system, occur more frequently in patients with Crohn's disease than ulcerative colitis.
Increased titers of anti-Saccharomyces cerevisiae antibodies in Crohn's disease patients with reduced H-ficolin levels but normal MASP-2 activity.
Colorectal Neoplasms
High levels of acute phase proteins and soluble 70 kDa heat shock proteins are independent and additive risk factors for mortality in colorectal cancer.
Mannan-binding lectin (MBL) and MBL-associated serine protease 2 (MASP-2) genotypes in colorectal cancer.
Pre- and postoperative levels in serum of mannan-binding lectin associated serine protease-2 -a prognostic marker in colorectal cancer.
Serum mannan-binding lectin-associated serine protease 2 levels in colorectal cancer: relation to recurrence and mortality.
WITHDRAWN: Diagnostic performance of MASP-2 in the diagnosis of colorectal carcinoma.
Common Variable Immunodeficiency
A new subset of common variable immune deficiency characterized by reduced C1 esterase inhibitor levels.
Anaesthetic management of an emergency caesarean section in a complex immunologically compromised patient with pre-eclampsia and multifactorial thrombocytopaenia.
Recombinant Human C1 Esterase Inhibitor for the Management of Adverse Events Related to Intravenous Immunoglobulin Infusion in Patients With Common Variable Immunodeficiency or Polyneuropathy: A Pilot Open-Label Study.
complement subcomponent c1s deficiency
A Case of Angioedema Due to Acquired C1 Esterase Inhibitor Deficiency Masquerading as Suspected Peritonitis: A Case Report.
Acquired C1 esterase deficiency with cold agglutinin disease associated with monoclonal IgM antibodies.
Acquired C1-esterase deficiency and optic neuropathy in non-Hodgkin's lymphoma.
An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female.
Circulating levels of mannan-binding lectin (MBL) and MBL-associated serine protease 2 in endemic pemphigus foliaceus.
Components of the lectin pathway of complement activation in paediatric patients of intensive care units.
Deficiency of mannose-binding lectin-associated serine protease-2 associated with increased risk of fever and neutropenia in pediatric cancer patients.
Emergency management of upper airway angio-oedema after routine dental extraction in a patient with C1 esterase deficiency.
Extraintestinal manifestations of Edwardsiella tarda infection: a 10-year retrospective review.
Falsely normal C4 in a case of acquired C1 esterase inhibitor deficiency.
Functional MASP2 gene polymorphism in patients with history of rheumatic fever.
Functional MASP2 single nucleotide polymorphism plays no role in psoriasis.
Genetic analysis of complement C1s deficiency associated with systemic lupus erythematosus highlights alternative splicing of normal C1s gene.
Hereditary angio-oedema as a rare cause of small-bowel obstruction.
Hereditary C1 esterase deficiency in a Zulu kindred.
Herpes simplex encephalitis in adult patients with MASP-2 deficiency.
L-ficolin (ficolin-2) insufficiency is associated with combined allergic and infectious respiratory disease in children.
Lectin pathway of complement activation in a Polish woman with MASP-2 deficiency.
Lectin-complement pathway molecules are decreased in patients with cirrhosis and constitute the risk of bacterial infections.
Mannan-binding lectin pathway deficiencies and invasive fungal infections following allogeneic stem cell transplantation.
Mannan-binding lectin-associated serine protease 2 is critical for the development of renal ischemia reperfusion injury and mediates tissue injury in the absence of complement C4.
Mannan-binding-lectin-associated serine proteases, characteristics and disease associations.
Mannose-binding lectin and mannose-binding lectin-associated serine protease 2 in susceptibility, severity, and outcome of pneumonia in adults.
Mannose-Binding Lectin and MBL-Associated Serine Protease-2 Genotypes and Serum Levels in Patients with Sporotrichosis.
Molecular basis of human complement C1s deficiency.
Selective complement C1s deficiency caused by homozygous four-base deletion in the C1s gene.
Should MASP-2 Deficiency Be Considered a Primary Immunodeficiency? Relevance of the Lectin Pathway.
Substitution therapy for C1 esterase deficiency.
Targeting of mannan-binding lectin-associated serine protease-2 confers protection from myocardial and gastrointestinal ischemia/reperfusion injury.
The late onset form of C1 esterase-inhibitor deficiency presenting as food allergy.
The mannan-binding lectin pathway and lung disease in cystic fibrosis--disfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major modifier.
Tuberculosis, onychomycosis and immune deficiency in complicated Crohn's disease.
[Anaesthesic management of vaginal delivery in a parturient with C1 esterase deficiency]
[Angioedema of the oropharynx]
[Hypocomplementemic urticarial vasculitis with glomerulopathy and renal venulitis]
Coronavirus Infections
Targeting the Complement Serine Protease MASP-2 as a Therapeutic Strategy for Coronavirus Infections.
COVID-19
C1 Esterase Inhibition: Targeting Multiple Systems in COVID-19.
C1 esterase inhibitor and the contact system in COVID-19.
Clinically distinct COVID-19 cases share notably similar immune response progression: A follow-up analysis.
Complement Activation in Kidneys of Patients With COVID-19.
Lectin Pathway Mediates Complement Activation by SARS-CoV-2 Proteins.
MASPs at the crossroad between the complement and the coagulation cascades - the case for COVID-19.
Multi-organ complement deposition in COVID-19 patients.
Multiple-Organ Complement Deposition on Vascular Endothelium in COVID-19 Patients.
Protease inhibitor plasma concentrations associate with COVID-19 infection.
Recombinant human C1 esterase inhibitor (conestat alfa) in the prevention of severe SARS-CoV-2 infection in hospitalized patients with COVID-19: A structured summary of a study protocol for a randomized, parallel-group, open-label, multi-center pilot trial (PROTECT-COVID-19).
Treatment of COVID-19 With Conestat Alfa, a Regulator of the Complement, Contact Activation and Kallikrein-Kinin System.
Crohn Disease
Dissociation between the functional activity and immunoreactive concentration of C1 esterase inhibitor in active and quiescent Crohn's disease.
High normal serum levels of C3 and C1 inhibitor, two acute-phase proteins belonging to the complement system, occur more frequently in patients with Crohn's disease than ulcerative colitis.
Increased titers of anti-Saccharomyces cerevisiae antibodies in Crohn's disease patients with reduced H-ficolin levels but normal MASP-2 activity.
[Simultaneous occurrence of hereditary angioneurotic edema and Crohn disease]
Croup
[Croup syndrome]
Cryoglobulinemia
Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease.
Monoclonal gammopathy with significance: case series and literature review.
Cystitis, Interstitial
Serum immunoglobulin and complement alterations in interstitial cystitis.
Dermatitis, Atopic
Helicobacter pylori infection and skin diseases.
Diabetes Mellitus
Extraintestinal manifestations of Edwardsiella tarda infection: a 10-year retrospective review.
Plasma Levels of MASP-1 and MASP-2 are Elevated in Type 1 Diabetes and Correlate with Glycaemic Control.
Diabetes Mellitus, Type 1
Incident microalbuminuria and complement factor mannan-binding lectin-associated protein 19 in people with newly diagnosed type 1 diabetes.
Plasma Levels of MASP-1 and MASP-2 are Elevated in Type 1 Diabetes and Correlate with Glycaemic Control.
Diabetes Mellitus, Type 2
Complement Activation and Prognosis in Patients With Type 2 Diabetes and Myocardial Infarction: A report from the DIGAMI 2 trial.
Embolism
C1 Esterase Inhibitor Activity in Amniotic Fluid Embolism.
Practical use of C1 esterase inhibitor concentrate for clinical amniotic fluid embolism.
Therapeutic application of C1 esterase inhibitor concentrate for clinical amniotic fluid embolism: a case report.
Embolism, Amniotic Fluid
C1 Esterase Inhibitor Activity in Amniotic Fluid Embolism.
Practical use of C1 esterase inhibitor concentrate for clinical amniotic fluid embolism.
Therapeutic application of C1 esterase inhibitor concentrate for clinical amniotic fluid embolism: a case report.
Encephalitis
Herpes simplex encephalitis in adult patients with MASP-2 deficiency.
Encephalitis, Herpes Simplex
Herpes simplex encephalitis in adult patients with MASP-2 deficiency.
Encephalomyelitis
Targeting the complement system in neuromyelitis optica spectrum disorder.
Encephalomyelitis, Autoimmune, Experimental
Targeting the complement system in neuromyelitis optica spectrum disorder.
Endotoxemia
C1-esterase inhibitor attenuates the inflammatory response during human endotoxemia.
Effects of C1 esterase inhibitor administration on intestinal functional capillary density, leukocyte adherence and mesenteric plasma extravasation during experimental endotoxemia.
Influence of C1-esterase inhibitor on tissue oxygenation of jejunal mucosa during endotoxemia.
The combinations C1 esterase inhibitor with coagulation factor XIII and N-acetylcysteine with tirilazad mesylate reduce the leukocyte adherence in an experimental endotoxemia in rats.
Erythema Nodosum
Complement determinations in the synovial fluid and serum of a patient with Erythema nodosum leprosum.
Esophageal Squamous Cell Carcinoma
Clinical significance of mannose-binding lectin-associated serine protease-2 expression in esophageal squamous cell carcinoma.
Food Hypersensitivity
Angioedema: frightening and frustrating.
Exogenous oestrogen as an alternative to food allergy in the aetiology of angioneurotic oedema.
The late onset form of C1 esterase-inhibitor deficiency presenting as food allergy.
Genetic Diseases, Inborn
A novel functional C1 inhibitor activity assay in dried blood spot for diagnosis of Hereditary angioedema.
A robust multiplexed assay to quantify C1-inhibitor, C1q, and C4 proteins for in vitro diagnosis of hereditary angioedema from dried blood spot.
Administration of conestat alfa, human C1 esterase inhibitor and icatibant in the treatment of acute angioedema attacks in adults with hereditary angioedema due to C1 esterase inhibitor deficiency. Treatment comparison based on systematic review results.
An investigational RNAi therapeutic targeting Factor XII (ALN-F12) for the treatment of hereditary angioedema.
An update on the diagnosis and management of hereditary angioedema with abnormal c1 inhibitor.
HAE update: epidemiology and burden of disease.
Hereditary angioedema: a clinical review for the otolaryngologist.
Hereditary angioedema: an update on causes, manifestations and treatment.
Hereditary angioedema: imaging manifestations and clinical management.
Hereditary angioedema: not an allergy.
Hereditary angioedema: quality of life in Brazilian patients.
Icatibant. Attacks of hereditary angioedema: continue to use C1 esterase inhibitor.
Long-Term Prophylaxis of Hereditary Angioedema with a Pasteurized C1 Inhibitor Concentrate.
Management of patients with hereditary angio-oedema in dental, oral, and maxillofacial surgery: a review.
Peri-operative management of a patient with hereditary angioedema undergoing laparoscopic cholecystectomy.
Perioperative management of tooth extractions for a patient with hereditary angioedema.
Recognition, Evaluation, and Management of Pediatric Hereditary Angioedema.
Recombinant human C1 esterase inhibitor in the management of hereditary angioedema.
Successful anesthetic management during abdominal wall-lifting laparoscopic cholecystectomy in a patient with hereditary angioedema.
Successful management of hereditary angioedema during tonsillectomy: a case report.
Successful resolution of bowel obstruction in a patient with hereditary angioedema.
Treatment with C1-esterase inhibitor concentrate in type I or II hereditary angioedema: a systematic literature review.
Glioblastoma
Construction of a competitive endogenous RNA network and analysis of potential regulatory axis targets in glioblastoma.
The Role of C1-Esterase Inhibitors in the Management of Vasogenic Edema in Glioblastoma.
Glioma
Increased expression and secretion of r-Gsp protein, rat counterpart of complement C1s precursor, during cyclic AMP-induced differentiation in rat C6 glioma cells.
Glomerulonephritis
Hereditary angioneurotic edema: immunochemical 'activity' without clinical expression.
Monoclonal gammopathy with significance: case series and literature review.
[Hereditary deficiency of C1 esterase inhibitor. Lupus and glomerulonephritis]
Glomerulonephritis, IGA
Safety, Tolerability and Efficacy of Narsoplimab, a Novel MASP-2 Inhibitor for the Treatment of IgA Nephropathy.
Glycogen Storage Disease
Budgetary Impact of Medicinal Therapies for Rare Diseases in Bulgaria.
Granuloma
Construction of human MASP-2-CCP1/2SP, CCP2SP, SP plasmid DNA nanolipoplexes and the effects on tuberculosis in BCG-infected mice.
Effects of hMASP-2 on the formation of BCG infection-induced granuloma in the lungs of BALB/c mice.
Guillain-Barre Syndrome
Guillain-Barré syndrome following danazol and corticosteroid therapy for hereditary angioedema.
Hearing Loss, Noise-Induced
Transcriptome characterization by RNA-Seq reveals the involvement of the complement components in noise-traumatized rat cochleae.
Heart Arrest
The complement lectin pathway after cardiac arrest.
Hematologic Neoplasms
Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2).
Hemoglobinuria
Biphenylsulfonyl-thiophene-carboxamidine inhibitors of the complement component C1s.
Complement blockade with a C1 esterase inhibitor in paroxysmal nocturnal hemoglobinuria.
Targeting the complement system in neuromyelitis optica spectrum disorder.
The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies.
Hemoglobinuria, Paroxysmal
Biphenylsulfonyl-thiophene-carboxamidine inhibitors of the complement component C1s.
Complement blockade with a C1 esterase inhibitor in paroxysmal nocturnal hemoglobinuria.
Targeting the complement system in neuromyelitis optica spectrum disorder.
The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies.
Hemolytic-Uremic Syndrome
Activation of both coagulation and fibrinolysis in childhood hemolytic uremic syndrome.
Hepatic Veno-Occlusive Disease
Hepatic veno-occlusive disease with severe capillary leakage after peripheral stem cell transplantation: treatment with recombinant plasminogen activator and C1-esterase inhibitor concentrate.
Hepatitis
Effects of MASP2 haplotypes and MASP-2 levels in hepatitis C-infected patients.
Hepatitis B
Contributions to the optimal use of human blood. IX. Elimination of hepatitis B transmission by (potentially) infectious plasma derivatives.
Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease.
Hepatitis C
Effects of MASP2 haplotypes and MASP-2 levels in hepatitis C-infected patients.
MASP2 gene polymorphism is associated with susceptibility to hepatitis C virus infection.
Hereditary Angioedema Types I and II
A case of hereditary angioedema associated with idiopathic hypoparathyroidism.
An Unusual Case of Acquired Angioedema and Monoclonal Gammopathy of Renal Significance in a Middle-Aged Caucasian Female.
Anaesthesia Management of a Patient with Hereditary Angioedema with Prophylactic Administration of C1 Esterase Inhibitor: Case report and literature review.
C1 esterase inhibitor activity is reduced in the acute phase following burn injury: a prospective observational study.
Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report.
Cinryze as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety.
Clinical presentation and diagnosis of hereditary angio-oedema in five families.
Complement abnormalities in diffuse plane xanthomatosis with paraproteinaemia.
Cutaneous manifestations of complement deficiencies.
Diagnosis and screening of patients with hereditary angioedema in primary care.
Diminished capacity of opsonization and immune complex solubilization, and detection of anti-C1q antibodies in sera from patients with hereditary angioedema.
DX-88 and HAE: a developmental perspective.
Ecallantide for the treatment of hereditary angiodema in adults.
Efficacy Assessments in Randomized Controlled Studies of Acute Therapy for Hereditary Angioedema.
Exacerbation of a lupus-erythematosus-like syndrome during treatment of non-C1-esterase-inhibitor-dependent angioedema with danazol.
General anaesthetic for a pilonidal sinus excision in a patient with hereditary angio-oedema.
Glomerulonephritis and hereditary angioedema: report of 2 cases.
Hereditary angioedema and pregnancy: successful management of recurrent and frequent attacks of angioedema with C1-inhibitor concentrate, danazol and tranexamic acid - a case report.
Hereditary angioedema type II--a study of two families.
Hereditary angioedema: a Taiwanese family with a novel gene mutation.
Hereditary angioedema: an update on causes, manifestations and treatment.
Hereditary angioedema: therapeutic effect of danazol on C4 and C1 esterase inhibitors.
Inherited disorders of complement.
Is there a need for clinical guidelines in the United States for the diagnosis of hereditary angioedema and the screening of family members of affected patients?
Long-Term Prophylaxis of Hereditary Angioedema with a Pasteurized C1 Inhibitor Concentrate.
Modern preoperative and intraoperative management of hereditary angioedema.
Modification of peripheral blood T-lymphocyte surface receptors and Langerhans cell numbers in hereditary angioedema.
Participation of Hageman factor dependent pathways in human disease states.
Prospective study of C1 esterase inhibitor in the treatment of successive acute abdominal and facial hereditary angioedema attacks.
Recognition, Evaluation, and Management of Pediatric Hereditary Angioedema.
Successful treatment of acute hereditary angioedema attacks with self-administered icatibant in patients with venous access problems.
The complex alteration in the network of IL-17-type cytokines in patients with hereditary angioedema.
The late onset form of C1 esterase-inhibitor deficiency presenting as food allergy.
treatment of hereditary angio-oedema by low dose attenuated androgens: disassociation of clinical response from levels of C1 esterase inhibitor and C4.
Type I hereditary angioedema in Taiwan -- clinical, biological features and genetic study.
[A study of coagulation contact factors in hereditary angioneurotic oedema (author's transl)]
[Acquired angioneurotic edema caused by acquired deficiency of C1 esterase inhibitor disclosing lymphoproliferative syndrome. Apropos of a case, review of the literature]
[Acquired deficiency of C1 esterase inhibitor and malignant lymphoproliferative syndromes]
[Acquired deficiency of C1 esterase inhibitor, persistent eyelid edema and subcutaneous lupus erythematosus]
[Clinical symptoms and therapy of the hereditary angioneurotic edema (author's transl)]
[Correction of deficiency of C1 esterase inhibitor in angioneurotic edema with oxymetholone]
[Hereditary angioedema caused by a deficiency of C1 esterase inhibitor. A sporadic case of late appearance]
[Hereditary angioedema, a rare cause of recurrent abdominal pains. A report of 2 clinical cases and comments of a general nature]
[Hereditary angioedema]
[Hereditary deficiency of C1 esterase inhibitor. Lupus and glomerulonephritis]
[Idiopathic anaphylaxis]
[Immune complex nephropathy and hereditary deficiency of C1 esterase inhibitor (author's transl)]
[Recurrent attacks of angioedema ascribed to the use of estrogen preparations and a pregnancy (hereditary angioedema type 3)]
[Respiratory manifestations in hereditary angioneurotic edema]
[Therapeutic approach of hereditary angioedema]
Hereditary Complement Deficiency Diseases
Glomerulonephritis and hereditary angioedema: report of 2 cases.
[Acquired angioneurotic edema caused by acquired deficiency of C1 esterase inhibitor disclosing lymphoproliferative syndrome. Apropos of a case, review of the literature]
[Immune complex nephropathy and hereditary deficiency of C1 esterase inhibitor (author's transl)]
HIV Infections
A dual role for Mannan-binding lectin-associated serine protease 2 (MASP-2) in HIV infection.
Hypersensitivity
Allergenicity and safety of recombinant human C1 esterase inhibitor in patients with allergy to rabbit or cow's milk.
Allergic urticaria and hereditary angioedema. Independent association in the same patient.
Serum concentrations of lectin-pathway components in healthy neonates, children and adults: mannan-binding lectin (MBL), M-, L-, and H-ficolin, and MBL-associated serine protease-2 (MASP-2).
Hypertension
MASPs at the crossroad between the complement and the coagulation cascades - the case for COVID-19.
Hypotension
Inhibition of the hypotensive effect of plasma protein solutions by C1 esterase inhibitor.
IgA Deficiency
Urticarial vasculitis, IgA deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report.
Infarction, Middle Cerebral Artery
Protective effect of C1 esterase inhibitor on reperfusion injury in the rat middle cerebral artery occlusion model.
Infections
A dual role for Mannan-binding lectin-associated serine protease 2 (MASP-2) in HIV infection.
Acquired C1-esterase inhibitor deficiency and recurrent herpes infection in a patient with chronic lymphocytic leukemia.
Angioedema due to acquired C1-esterase inhibitor deficiency in a patient with Helicobacter pylori infection.
Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
Association between MASP-2 gene polymorphism and risk of infection diseases: A meta-analysis.
Association of MASP-2 Levels and MASP2 Gene Polymorphisms with Rheumatoid Arthritis in Patients and Their Relatives.
C1 esterase inhibitor and the contact system in COVID-19.
C1 esterase inhibitor: new preparation. A major advance in emergency treatment of hereditary angioneurotic oedema.
Cleavage of kininogen and subsequent bradykinin release by the complement component: mannose-binding lectin-associated serine protease (MASP)-1.
Complement C1 esterase inhibitor levels linked to infections and contaminated heparin-associated adverse events.
Construction of human MASP-2-CCP1/2SP, CCP2SP, SP plasmid DNA nanolipoplexes and the effects on tuberculosis in BCG-infected mice.
Deficiency of mannose-binding lectin-associated serine protease-2 associated with increased risk of fever and neutropenia in pediatric cancer patients.
Effects of MASP2 haplotypes and MASP-2 levels in hepatitis C-infected patients.
Extraintestinal manifestations of Edwardsiella tarda infection: a 10-year retrospective review.
Impact of the complement lectin pathway on cytomegalovirus disease early after kidney transplantation.
Inherited disorders of complement.
L-ficolin (ficolin-2) insufficiency is associated with combined allergic and infectious respiratory disease in children.
Lack of association between polymorphisms of MASP2 and susceptibility to SARS coronavirus infection.
Low MBL-associated serine protease 2 (MASP-2) levels correlate with urogenital schistosomiasis in Nigerian children.
M-ficolin levels are associated with the occurrence of severe infections in patients with haematological cancer undergoing chemotherapy.
Mannan-binding lectin-associated serine protease-2 (MASP-2) in a large cohort of neonates and its clinical associations.
Pharmaceutical approval update.
Serum mannan-binding lectin-associated serine protease 2 levels in colorectal cancer: relation to recurrence and mortality.
Tuberculosis, onychomycosis and immune deficiency in complicated Crohn's disease.
Intestinal Obstruction
Recurrent intestinal obstruction with acquired angio-oedema, due to C1-esterase inhibitor deficiency.
Intussusception
Colonic Intussusception Secondary to Hereditary Angioedema.
Hereditary Angioedema and Gastrointestinal Complications: An Extensive Review of the Literature.
Ischemic Stroke
Combination Therapy with Low-Dose IVIG and a C1-esterase Inhibitor Ameliorates Brain Damage and Functional Deficits in Experimental Ischemic Stroke.
Serine proteases of the complement lectin pathway and their genetic variations in ischaemic stroke.
Tissue Plasminogen Activator-Induced Angioedema Involving a Posterior Cerebral Artery Infarct: A Case Presentation.
Kidney Neoplasms
Complement C1s and C4d as Prognostic Biomarkers in Renal Cancer: Emergence of Noncanonical Functions of C1s.
Laryngeal Edema
Asphyxiation by laryngeal edema in patients with hereditary angioedema.
Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency.
Hereditary angioedema presenting as epiglottitis.
Nanofiltered C1 esterase inhibitor for treatment of laryngeal attacks in patients with hereditary angioedema.
Treatment with C1-esterase inhibitor concentrate in type I or II hereditary angioedema: a systematic literature review.
Laryngismus
[Croup syndrome]
Leprosy
Leprosy association with low MASP-2 levels generated by MASP2 haplotypes and polymorphisms flanking MAp19 exon 5.
Leukemia
Association between serum levels of MASP-2 and neutropenic febrile attacks in children with leukemia.
Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis.
Leukemia, Lymphocytic, Chronic, B-Cell
Acquired C1-esterase inhibitor deficiency and recurrent herpes infection in a patient with chronic lymphocytic leukemia.
Lymphocytic lymphoma and acquired C1 esterase inhibitor deficiency.
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency.
Leukemia, Myeloid, Acute
Effect of conditioned media of acute myeloid leukemia blast cells on complement synthesis by cultured human cells of monocyte and hepatocyte origin.
Leukopenia
Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases.
Lung Diseases
The Clinical Significance of Serum MASP-2 and IDH1 in the Early Diagnosis of Non-Small Cell Lung Cancer.
The mannan-binding lectin pathway and lung disease in cystic fibrosis--disfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major modifier.
Lung Injury
Antihistone Properties of C1 Esterase Inhibitor Protect against Lung Injury.
Effect of complement fragment 1 esterase inhibition on survival of human decay-accelerating factor pig lungs perfused with human blood.
Nebulized C1-Esterase Inhibitor does not Reduce Pulmonary Complement Activation in Rats with Severe Streptococcus Pneumoniae Pneumonia.
Plasma-Derived Human C1-Esterase Inhibitor Does Not Prevent Mechanical Ventilation-Induced Pulmonary Complement Activation in a Rat Model of Streptococcus pneumoniae Pneumonia.
Targeting the Complement Serine Protease MASP-2 as a Therapeutic Strategy for Coronavirus Infections.
Lung Neoplasms
Limited proteolysis of beta 2-microglobulin at Lys-58 by complement component C1s.
The Clinical Significance of Serum MASP-2 and IDH1 in the Early Diagnosis of Non-Small Cell Lung Cancer.
[A case of lung cancer with hereditary angioedema treated effectively by chemo-radiotherapy with C1 esterase inhibitor concentrate and danazol]
Lupus Erythematosus, Systemic
CT Findings of Acute Small-Bowel Entities.
Facial angioedema and systemic lupus erythematosus: case report.
Genetic analysis of complement C1s deficiency associated with systemic lupus erythematosus highlights alternative splicing of normal C1s gene.
Human parvovirus infection: rheumatic manifestations, angioedema, C1 esterase inhibitor deficiency, ANA positivity, and possible onset of systemic lupus erythematosus.
Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease.
Intractable acquired autoimmune angioedema in a patient with systemic lupus erythematosus.
The lectin complement pathway serine proteases (MASPs) represent a possible crossroad between the coagulation and complement systems in thromboinflammation.
Lupus Nephritis
Gene Expression Analysis Detected a Low Expression Level of C1s Gene in ICR-Derived Glomerulonephritis (ICGN) Mice.
Lymphoma
Acquired C1 esterase inhibitor deficiency in a marginal zone lymphoma patient treated with rituximab.
Acquired C1 esterase inhibitor deficiency in lymphomas: prevalence, symptoms, and response to treatment.
Angioedema in the emergency department: a presentation of lymphoma.
C1 esterase inhibitor as a possible tumour marker in malignant lymphoma.
C1-esterase inhibitor autoantibodies in a patient with acute tongue swelling.
Intestinal angioedema caused by an acquired C1 esterase inhibitor deficiency associated with underlying splenic marginal zone lymphoma.
Long-standing angioedema with C1 esterase inhibitor deficiency associated with occult lymphoma.
Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma.
Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2).
Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema.
Urticarial vasculitis and asymptomatic acquired C1 esterase inhibitor deficiency revealing an angioimmunoblastic T cell lymphoma.
[Acquired C1 esterase inhibitor deficiency and lymphoproliferative syndromes]
[Acquired C1 esterase inhibitor deficiency, Gougerot-Sjögren syndrome and B lymphoma]
[Acquired C1-esterase inhibitor deficiency with angioedema in lymphoplasmocytic lymphoma]
[Apropos of the case reported by M. Leporrier et al., on mixed nodular lymphoma associated with a deficiency of C1-esterase inhibitor]
Lymphoma, B-Cell
Acquired C1-esterase inhibitor deficiency and positive lupus anticoagulant accompanied by splenic marginal zone B-cell lymphoma.
Lymphoma, B-Cell, Marginal Zone
Acquired C1-esterase inhibitor deficiency and positive lupus anticoagulant accompanied by splenic marginal zone B-cell lymphoma.
Lymphoma, Follicular
[Apropos of the case reported by M. Leporrier et al., on mixed nodular lymphoma associated with a deficiency of C1-esterase inhibitor]
[Mixed nodular lymphoma in association with a C1 esterase inhibitor deficiency (author's transl)]
Lymphoma, Non-Hodgkin
Acquired C1-esterase deficiency and optic neuropathy in non-Hodgkin's lymphoma.
Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma.
Lymphoma, T-Cell
Urticarial vasculitis and asymptomatic acquired C1 esterase inhibitor deficiency revealing an angioimmunoblastic T cell lymphoma.
Lymphoma, T-Cell, Peripheral
Pharmaceutical approval update.
Lymphoproliferative Disorders
Acquired angioedema.
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool.
Acquired C1 esterase inhibitor deficiency as manifestation of T-cell lymphoproliferative disorder.
Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration.
Acquired C1 esterase inhibitor deficiency.
Angioedema as the first presentation of B-cell non-Hodgkin lymphoma--an unusual case with normal C1 esterase inhibitor level: a case report.
Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
Antiepileptic drug-induced lymphoproliferative disorder associated with acquired C1 esterase inhibitor deficiency and angioedema.
Macroglossia
Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency.
Mania
Are Mannan-binding Lectine Serin Protease-2 and Alpha-1-microglobulin and Bukinin Precursor the Potential Biomarkers of Manic Episode? A Study via Urinary Proetomic Analysis.
Mastocytosis
[Idiopathic anaphylaxis]
Mastocytosis, Systemic
Chapter 25: Idiopathic anaphylaxis.
Idiopathic anaphylaxis.
Memory Disorders
Early treatment with C1 esterase inhibitor improves weight but not memory deficits in a rat model of status epilepticus.
Meningitis, Bacterial
Mannose-binding lectin-associated serine protease 2 (MASP-2) contributes to poor disease outcome in humans and mice with pneumococcal meningitis.
Meningitis, Pneumococcal
Mannose-binding lectin-associated serine protease 2 (MASP-2) contributes to poor disease outcome in humans and mice with pneumococcal meningitis.
Multiple Sclerosis
Gene-environment interactions in multiple sclerosis: innate and adaptive immune responses to human endogenous retrovirus and herpesvirus antigens and the lectin complement activation pathway.
Mannose binding lectin mediated complement pathway in multiple sclerosis.
Mycoses
Mannose-Binding Lectin and MBL-Associated Serine Protease-2 Genotypes and Serum Levels in Patients with Sporotrichosis.
Myocardial Infarction
Administration of C1-esterase inhibitor during emergency coronary artery bypass surgery in acute ST-elevation myocardial infarction.
Beneficial effects of C1 esterase inhibitor in ST-elevation myocardial infarction in patients who underwent surgical reperfusion: a randomised double-blind study.
Myocardial infarction induces atrial inflammation that can be prevented by C1-esterase inhibitor.
TFPI inhibits lectin pathway of complement activation by direct interaction with MASP-2.
Myocardial Ischemia
Cardioprotective effects of a C1 esterase inhibitor in myocardial ischemia and reperfusion.
MASP-2 activation is involved in ischemia-related necrotic myocardial injury in humans.
Rescue therapy with C1-esterase inhibitor concentrate after emergency coronary surgery for failed PTCA.
Myocardial Reperfusion Injury
Beneficial effects of C1 esterase inhibitor in ST-elevation myocardial infarction in patients who underwent surgical reperfusion: a randomised double-blind study.
Nasal Obstruction
[Hereditary angioedema due to C1-esterase inhibitor deficiency]
Neoplasm Metastasis
Clinical significance of mannose-binding lectin-associated serine protease-2 expression in esophageal squamous cell carcinoma.
[Proteinase inhibitors and fibrinogen split products in patients with malignant diseases (author's transl)]
Neoplasms
Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration.
Acquired C1 esterase inhibitor deficiency: An atypical first presentation intra-operatively.
Acquired C1 inhibitor deficiency: postmortem diagnosis.
Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency.
Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
Antithrombin III, but not C1 esterase inhibitor reduces inflammatory response in lipopolysaccharide-stimulated human monocytes in an ex-vivo whole blood setting.
C1 esterase inhibitor as a possible tumour marker in malignant lymphoma.
C1 esterase inhibitor protein associated with human cancer: a tumour marker.
C1-esterase inactivator from cancer cells is C-reactive protein.
Clinical significance of mannose-binding lectin-associated serine protease-2 expression in esophageal squamous cell carcinoma.
Deficiency of mannose-binding lectin-associated serine protease-2 associated with increased risk of fever and neutropenia in pediatric cancer patients.
Effect of tumour necrosis factor-alpha on proliferation, activation and protein synthesis of rat hepatic stellate cells.
Human cytokine responses to coronary artery bypass grafting with and without cardiopulmonary bypass.
Mannan-binding lectin (MBL) and MBL-associated serine protease-2 in children with cancer.
Mannose-Binding Lectin (MBL) and MBL-associated serine protease-2 (MASP-2) in women with malignant and benign ovarian tumours.
MASP-1 and MASP-2 Serum Levels Are Associated With Worse Prognostic in Cervical Cancer Progression.
Microheterogeneous changes of the C1-esterase inhibitor in plasma cannot predict operability, postoperative complications, or recurrence in cancer surgery.
Pre- and postoperative levels in serum of mannan-binding lectin associated serine protease-2 -a prognostic marker in colorectal cancer.
Pre-operative plasma levels of C-reactive protein, albumin and various plasma protease inhibitors for the pre-operative assessment of operability and recurrence in cancer surgery.
Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2).
Serum Concentrations of Mannan-Binding Lectin (MBL) and MBL-Associated Serine Protease-2 and the Risk of Adverse Events in Pediatric Patients With Cancer and Fever in Neutropenia.
Serum mannan-binding lectin-associated serine protease 2 levels in colorectal cancer: relation to recurrence and mortality.
The Clinical Significance of Serum MASP-2 and IDH1 in the Early Diagnosis of Non-Small Cell Lung Cancer.
Up-regulated expression of the MAT-8 gene in prostate cancer and its siRNA-mediated inhibition of expression induces a decrease in proliferation of human prostate carcinoma cells.
Useful laboratory tests for studying thrombogenesis in acute cardiac syndromes.
[Croup syndrome]
[Proteinase inhibitors and fibrinogen split products in patients with malignant diseases (author's transl)]
Nephritis
Guillain-Barré syndrome following danazol and corticosteroid therapy for hereditary angioedema.
Nephrotic Syndrome
Monoclonal gammopathy with significance: case series and literature review.
Neuromyelitis Optica
Potential therapeutic benefit of C1-esterase inhibitor in neuromyelitis optica evaluated in vitro and in an experimental rat model.
Purified human C1-esterase inhibitor is safe in acute relapses of neuromyelitis optica.
Targeting the complement system in neuromyelitis optica spectrum disorder.
Neutropenia
Deficiency of mannose-binding lectin-associated serine protease-2 associated with increased risk of fever and neutropenia in pediatric cancer patients.
Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases.
Serum Concentrations of Mannan-Binding Lectin (MBL) and MBL-Associated Serine Protease-2 and the Risk of Adverse Events in Pediatric Patients With Cancer and Fever in Neutropenia.
Obesity
Contact activated kallikrein generation is reduced six months after gastric bypass.
Onychomycosis
Tuberculosis, onychomycosis and immune deficiency in complicated Crohn's disease.
Optic Nerve Diseases
Acquired C1-esterase deficiency and optic neuropathy in non-Hodgkin's lymphoma.
Ovarian Neoplasms
Mannose-Binding Lectin (MBL) and MBL-associated serine protease-2 (MASP-2) in women with malignant and benign ovarian tumours.
Pancreatitis
Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation.
Combined treatment with C1 esterase inhibitor and antithrombin III improves survival in severe acute experimental pancreatitis.
Effects of C1-esterase inhibitor in three models of acute pancreatitis.
Pancreatitis, Acute Hemorrhagic
Effects of C1-esterase inhibitor in three models of acute pancreatitis.
Paralysis
[Croup syndrome]
Paraproteinemias
Monoclonal gammopathy with significance: case series and literature review.
Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis.
Urticarial vasculitis in a connective tissue disease clinic: patterns, presentations, and treatment.
[Idiopathic anaphylaxis]
Parvoviridae Infections
Human parvovirus infection: rheumatic manifestations, angioedema, C1 esterase inhibitor deficiency, ANA positivity, and possible onset of systemic lupus erythematosus.
Peritonitis
A Case of Angioedema Due to Acquired C1 Esterase Inhibitor Deficiency Masquerading as Suspected Peritonitis: A Case Report.
Persistent Infection
The mannan-binding lectin pathway and lung disease in cystic fibrosis--disfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major modifier.
Persistent Vegetative State
Association of Lectin Pathway Protein Levels and Genetic Variants Early after Injury with Outcomes after Severe Traumatic Brain Injury: A Prospective Cohort Study.
Pheochromocytoma
Chapter 25: Idiopathic anaphylaxis.
Idiopathic anaphylaxis.
Pneumococcal Infections
The lectin pathway of complement activation is a critical component of the innate immune response to pneumococcal infection.
Pneumonia
Human plasma-derived C1 esterase inhibitor concentrate has limited effect on house dust mite-induced allergic lung inflammation in mice.
Mannan-binding lectin (MBL) and MBL-associated serine protease 2 (MASP-2) genotypes in colorectal cancer.
Nebulized C1-Esterase Inhibitor does not Reduce Pulmonary Complement Activation in Rats with Severe Streptococcus Pneumoniae Pneumonia.
Plasma-Derived Human C1-Esterase Inhibitor Does Not Prevent Mechanical Ventilation-Induced Pulmonary Complement Activation in a Rat Model of Streptococcus pneumoniae Pneumonia.
Serum mannan-binding lectin-associated serine protease 2 levels in colorectal cancer: relation to recurrence and mortality.
Polyneuropathies
Recombinant Human C1 Esterase Inhibitor for the Management of Adverse Events Related to Intravenous Immunoglobulin Infusion in Patients With Common Variable Immunodeficiency or Polyneuropathy: A Pilot Open-Label Study.
Pre-Eclampsia
C1-esterase inhibitor in uncomplicated pregnancy and mild and moderate preeclampsia.
Primary Myelofibrosis
Correction of recurrent angioedema related to C1 esterase inhibitor deficiency as a secondary event following nonmyeloablative allogeneic stem cell transplantation in a patient with myelofibrosis.
Proteinuria
Absence of the Lectin Activation Pathway of Complement Ameliorates Proteinuria-Induced Renal Injury.
Pruritus
Acquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma.
Hereditary angioedema as a cause of transient abdominal pain.
Psoriasis
Functional MASP2 single nucleotide polymorphism plays no role in psoriasis.
Pulmonary Disease, Chronic Obstructive
MASPs at the crossroad between the complement and the coagulation cascades - the case for COVID-19.
Purpura
Helicobacter pylori infection and skin diseases.
Purpura, Thrombocytopenic, Idiopathic
Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma.
Renal Insufficiency, Chronic
MASPs at the crossroad between the complement and the coagulation cascades - the case for COVID-19.
The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies.
Reperfusion Injury
A novel series of arylsulfonylthiophene-2-carboxamidine inhibitors of the complement component C1s.
Administration of C1-esterase inhibitor during emergency coronary artery bypass surgery in acute ST-elevation myocardial infarction.
Apotransferrin, C1-esterase inhibitor, and alpha 1-acid glycoprotein for cerebral protection during experimental hypothermic circulatory arrest.
C1 esterase inhibitor ameliorates ischemia reperfusion injury in a swine musculocutaneous flap model.
C1 esterase inhibitor reduces lower extremity ischemia/reperfusion injury and associated lung damage.
C1-esterase inhibitor reduces reperfusion injury after lung transplantation.
C1-esterase inhibitor reverses functional consequences of superior mesenteric artery ischemia/reperfusion by limiting reperfusion injury and restoring microcirculatory perfusion.
Design and synthesis of polyethylene glycol-modified biphenylsulfonyl-thiophene-carboxamidine inhibitors of the complement component C1s.
Effects of Ischemic Preconditioning and C1 Esterase Inhibitor Administration following Ischemia-Reperfusion Injury in a Rat Skin Flap Model.
Intracoronary application of C1 esterase inhibitor improves cardiac function and reduces myocardial necrosis in an experimental model of ischemia and reperfusion.
Mannan binding lectin-associated serine protease-2 (MASP-2) critically contributes to post-ischemic brain injury independent of MASP-1.
Protective effect of C1 esterase inhibitor on reperfusion injury in the rat middle cerebral artery occlusion model.
Single-lung transplantation in a patient with cystic fibrosis and an asymmetric thorax.
Targeting of mannan-binding lectin-associated serine protease-2 confers protection from myocardial and gastrointestinal ischemia/reperfusion injury.
Three-Year Outcomes of a Randomized, Double-Blind, Placebo-Controlled Study Assessing Safety and Efficacy of C1 Esterase Inhibitor for Prevention of Delayed Graft Function in Deceased Donor Kidney Transplant Recipients.
Respiratory Distress Syndrome
Design and synthesis of polyethylene glycol-modified biphenylsulfonyl-thiophene-carboxamidine inhibitors of the complement component C1s.
Respiratory Insufficiency
Successful use of daily intravenous infusion of C1 esterase inhibitor concentrate in the treatment of a hereditary angioedema patient with ascites, hypovolemic shock, sepsis, renal and respiratory failure.
Rheumatic Diseases
Trimer and tetramer complexes containing C1 esterase inhibitor, C1r and C1s, in serum and synovial fluid of patients with rheumatic disease.
Rheumatic Fever
Association of MASP2 polymorphisms and protein levels with rheumatic fever and rheumatic heart disease.
Functional MASP2 gene polymorphism in patients with history of rheumatic fever.
Rheumatic Heart Disease
Association of MASP2 polymorphisms and protein levels with rheumatic fever and rheumatic heart disease.
Functional MASP2 gene polymorphism in patients with history of rheumatic fever.
Rosacea
Helicobacter pylori infection and skin diseases.
Schistosomiasis haematobia
Low MBL-associated serine protease 2 (MASP-2) levels correlate with urogenital schistosomiasis in Nigerian children.
Scleroderma, Systemic
Helicobacter pylori infection and skin diseases.
Sepsis
Bench-to-bedside review: The role of C1-esterase inhibitor in sepsis and other critical illnesses.
C1-esterase inhibitor and its effects on endotoxin-induced leukocyte adherence and plasma extravasation in postcapillary venules.
C1-esterase inhibitor attenuates the inflammatory response during human endotoxemia.
C1-esterase inhibitor in early septicemia.
C1-esterase inhibitor infusion increases survival rates for patients with sepsis*
C1-esterase inhibitor substitution in sepsis.
Components of the lectin pathway of complement activation in paediatric patients of intensive care units.
Plasma kallikrein-kinin system in patients with uncomplicated sepsis and septic shock--comparison with cardiogenic shock.
Successful use of daily intravenous infusion of C1 esterase inhibitor concentrate in the treatment of a hereditary angioedema patient with ascites, hypovolemic shock, sepsis, renal and respiratory failure.
[Safety and efficacy of purified human C1-esterase inhibitor in the treatment of patients with sepsis]
Severe Acute Respiratory Syndrome
C1 esterase inhibitor and the contact system in COVID-19.
Shock, Septic
Combined antithrombin III and C1-esterase inhibitor treatment decreases intravascular fibrin deposition and attenuates cardiorespiratory impairment in rabbits exposed to Escherichia coli endotoxin.
Cysteine proteinase from Streptococcus pyogenes enables evasion of innate immunity via degradation of complement factors.
Initial studies on the administration of C1-esterase inhibitor to patients with septic shock or with a vascular leak syndrome induced by interleukin-2 therapy.
Plasma kallikrein-kinin system in patients with uncomplicated sepsis and septic shock--comparison with cardiogenic shock.
Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor.
Studies on components of the plasma kallikrein-kinin system in plasma samples from normal individuals and patients with septic shock.
The effect of C1-esterase inhibitor in definite and suspected streptococcal toxic shock syndrome. Report of seven patients.
The role of complement system in septic shock.
Skin Diseases
Helicobacter pylori infection and skin diseases.
Spinal Cord Injuries
Protective effect of C1 esterase inhibitor on acute traumatic spinal cord injury in the rat.
Sporotrichosis
Mannose-Binding Lectin and MBL-Associated Serine Protease-2 Genotypes and Serum Levels in Patients with Sporotrichosis.
Status Epilepticus
Early treatment with C1 esterase inhibitor improves weight but not memory deficits in a rat model of status epilepticus.
Stroke
Genetically-defined deficiency of mannose-binding lectin is associated with protection after experimental stroke in mice and outcome in human stroke.
Mannan binding lectin-associated serine protease-2 (MASP-2) critically contributes to post-ischemic brain injury independent of MASP-1.
Outcomes of long term treatments of type I hereditary angioedema in a Turkish family.
Plasma levels of mannan-binding lectin (MBL)-associated serine proteases (MASPs) and MBL-associated protein in cardio- and cerebrovascular diseases.
TFPI inhibits lectin pathway of complement activation by direct interaction with MASP-2.
Subarachnoid Hemorrhage
Human brain trauma severity is associated with lectin complement pathway activation.
Sweet Syndrome
Helicobacter pylori infection and skin diseases.
Thrombophilia
Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction.
The influence of C1-esterase inhibitor substitution on coagulation and cardiorespiratory parameters in an endotoxin-induced rabbit model of hypercoagulability.
Thrombosis
Assessment of hereditary angioedema treatment risks.
C1-esterase inhibitor enhances thrombin generation and spatial fibrin clot propagation in the presence of thrombomodulin.
Hypersensitive mousetraps, alpha1-antitrypsin deficiency and dementia.
Plasma levels of mannan-binding lectin (MBL)-associated serine proteases (MASPs) and MBL-associated protein in cardio- and cerebrovascular diseases.
Thrombotic Microangiopathies
The Inhibition of Complement System in Formal and Emerging Indications: Results from Parallel One-Stage Pairwise and Network Meta-Analyses of Clinical Trials and Real-Life Data Studies.
Tuberculosis
Construction of human MASP-2-CCP1/2SP, CCP2SP, SP plasmid DNA nanolipoplexes and the effects on tuberculosis in BCG-infected mice.
Effects of hMASP-2 on the formation of BCG infection-induced granuloma in the lungs of BALB/c mice.
Effects of MBL-associated serine protease-2 (MASP-2) on liquefaction and ulceration in rabbit skin model of tuberculosis.
Impact of MBL and MASP-2 gene polymorphism and its interaction on susceptibility to tuberculosis.
Tuberculosis, onychomycosis and immune deficiency in complicated Crohn's disease.
Tuberculosis, Pulmonary
No Strong Relationship Between Components of the Lectin Pathway of Complement and Susceptibility to Pulmonary Tuberculosis.
Urticaria
Allergic urticaria and hereditary angioedema. Independent association in the same patient.
An update on the diagnosis and management of hereditary angioedema with abnormal c1 inhibitor.
Angioedema due to Systemic Isotretinoin Therapy.
Angioedema suppressed by a combination of anti-histamine and leukotriene modifier.
Exogenous oestrogen as an alternative to food allergy in the aetiology of angioneurotic oedema.
Hereditary angioedema as a cause of transient abdominal pain.
Intractable acquired autoimmune angioedema in a patient with systemic lupus erythematosus.
Monoclonal gammopathy in association with allergic disorders of the skin and respiratory tract.
On the role of the C1-esterase inhibitor in cold urticaria.
Protease inhibitors in plasma of patients with chronic urticaria.
The value of C1 esterase inhibitor in patients with aspirin-sensitive urticaria.
[Behavior of esterase inhibitor C1 in patients with urticaria due to aspirin hypersensitivity]
Uterine Cervical Neoplasms
MASP-1 and MASP-2 Serum Levels Are Associated With Worse Prognostic in Cervical Cancer Progression.
Uterine Inertia
Reference range for C1-esterase inhibitor (C1 INH) in the third trimester of pregnancy.
Vasculitis
Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
Experimentally-induced anti-myeloperoxidase vasculitis does not require properdin, MASP-2 or bone marrow-derived C5.
Monoclonal gammopathy with significance: case series and literature review.
Urticarial vasculitis and asymptomatic acquired C1 esterase inhibitor deficiency revealing an angioimmunoblastic T cell lymphoma.
Urticarial vasculitis in a connective tissue disease clinic: patterns, presentations, and treatment.
Urticarial vasculitis, IgA deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report.
Vocal Cord Paralysis
[Croup syndrome]
Whooping Cough
Bordetella pertussis autotransporter Vag8 binds human C1 esterase inhibitor and confers serum resistance.
Bordetella pertussis binds human C1 esterase inhibitor during the virulent phase, to evade complement-mediated killing.
Xanthomatosis
Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis.