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Acides aminés, peptides et protéines
Protéines
Protéines inhibitrices de la fraction C1 du complément
C1 Inhibiteur
C1 Inhibiteur : Questions médicales fréquentes
Diagnostic
5
Déficience en C1 inhibiteur
Tests de laboratoire
Protéines du complément
Tests de laboratoire
Antécédents médicaux
Hérédité
Symptômes
5
Maladies auto-immunes
Allergies
Prévention
5
Allergies alimentaires
Réactions
Stress
Facteurs déclenchants
Traitements
5
Traitement
Médicaments anti-inflammatoires
Corticostéroïdes
Inflammation
Thérapie génique
Traitements expérimentaux
Crise aiguë
Administration de médicaments
Complications
5
Œdème laryngé
Difficultés respiratoires
Maladies auto-immunes
Risque accru
Qualité de vie
Symptômes chroniques
Surveillance médicale
Complications
Facteurs de risque
5
Facteurs de risque
Hérédité
Facteurs environnementaux
Crises
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"@type": "Question",
"name": "Quels tests sont utilisés pour évaluer le C1 inhibiteur ?",
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"@type": "Question",
"name": "Quels symptômes indiquent un problème de C1 inhibiteur ?",
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"text": "Les symptômes incluent des œdèmes, des douleurs abdominales et des éruptions cutanées."
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"@type": "Question",
"name": "Le diagnostic nécessite-t-il une consultation spécialisée ?",
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"@type": "Question",
"name": "Peut-on diagnostiquer par des antécédents familiaux ?",
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"@type": "Question",
"name": "Quels sont les symptômes d'une carence en C1 inhibiteur ?",
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"position": 8,
"acceptedAnswer": {
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"name": "Y a-t-il des symptômes associés à des infections ?",
"position": 9,
"acceptedAnswer": {
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"text": "Oui, des infections peuvent exacerber les symptômes liés à la carence en C1 inhibiteur."
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"name": "Les symptômes peuvent-ils être confondus avec d'autres maladies ?",
"position": 10,
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"text": "Oui, ils peuvent être similaires à ceux d'autres troubles allergiques ou auto-immuns."
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"name": "Comment prévenir les crises d'œdème ?",
"position": 11,
"acceptedAnswer": {
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"text": "Éviter les déclencheurs connus et suivre un traitement préventif régulier."
}
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{
"@type": "Question",
"name": "Les patients doivent-ils éviter certains aliments ?",
"position": 12,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, certains aliments peuvent déclencher des réactions, il est conseillé de les identifier."
}
},
{
"@type": "Question",
"name": "Le stress influence-t-il les crises ?",
"position": 13,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, le stress peut être un facteur déclenchant pour certaines personnes."
}
},
{
"@type": "Question",
"name": "Des vaccinations sont-elles recommandées ?",
"position": 14,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les vaccinations peuvent aider à prévenir les infections qui aggravent les symptômes."
}
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{
"@type": "Question",
"name": "Les patients doivent-ils porter un bracelet médical ?",
"position": 15,
"acceptedAnswer": {
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"text": "Oui, un bracelet médical peut alerter en cas d'urgence liée à la carence en C1 inhibiteur."
}
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{
"@type": "Question",
"name": "Quels traitements sont disponibles pour la carence en C1 inhibiteur ?",
"position": 16,
"acceptedAnswer": {
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"text": "Les traitements incluent des concentrés de C1 inhibiteur et des médicaments anti-inflammatoires."
}
},
{
"@type": "Question",
"name": "Le traitement préventif est-il nécessaire ?",
"position": 17,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, un traitement préventif peut être recommandé pour éviter les crises d'œdème."
}
},
{
"@type": "Question",
"name": "Les corticostéroïdes sont-ils efficaces ?",
"position": 18,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les corticostéroïdes peuvent réduire l'inflammation, mais ne traitent pas la cause sous-jacente."
}
},
{
"@type": "Question",
"name": "Y a-t-il des traitements expérimentaux ?",
"position": 19,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des thérapies géniques et des traitements ciblés sont en cours d'évaluation."
}
},
{
"@type": "Question",
"name": "Comment gérer une crise aiguë ?",
"position": 20,
"acceptedAnswer": {
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"text": "L'administration rapide de C1 inhibiteur est essentielle lors d'une crise aiguë."
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"@type": "Question",
"name": "Quelles sont les complications possibles de la carence en C1 inhibiteur ?",
"position": 21,
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"text": "Les complications incluent des œdèmes sévères, des infections et des douleurs chroniques."
}
},
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"@type": "Question",
"name": "Les œdèmes peuvent-ils entraîner des complications graves ?",
"position": 22,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des œdèmes laryngés peuvent provoquer des difficultés respiratoires potentiellement mortelles."
}
},
{
"@type": "Question",
"name": "Y a-t-il un risque accru de maladies auto-immunes ?",
"position": 23,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les patients peuvent avoir un risque accru de développer des maladies auto-immunes."
}
},
{
"@type": "Question",
"name": "Les complications peuvent-elles affecter la qualité de vie ?",
"position": 24,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les symptômes chroniques et les crises peuvent significativement altérer la qualité de vie."
}
},
{
"@type": "Question",
"name": "Les complications nécessitent-elles une surveillance régulière ?",
"position": 25,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, une surveillance régulière est essentielle pour gérer les complications potentielles."
}
},
{
"@type": "Question",
"name": "Quels sont les facteurs de risque de carence en C1 inhibiteur ?",
"position": 26,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les antécédents familiaux et certaines mutations génétiques augmentent le risque."
}
},
{
"@type": "Question",
"name": "Les infections fréquentes sont-elles un facteur de risque ?",
"position": 27,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les infections récurrentes peuvent exacerber les symptômes chez les patients."
}
},
{
"@type": "Question",
"name": "Le sexe influence-t-il le risque de carence ?",
"position": 28,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, la carence en C1 inhibiteur est plus fréquente chez les femmes que chez les hommes."
}
},
{
"@type": "Question",
"name": "L'âge joue-t-il un rôle dans le risque ?",
"position": 29,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, le risque peut augmenter avec l'âge, bien que les symptômes apparaissent souvent plus tôt."
}
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{
"@type": "Question",
"name": "Les facteurs environnementaux influencent-ils le risque ?",
"position": 30,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, certains facteurs environnementaux peuvent déclencher des crises chez les individus sensibles."
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Expert en Médecine, Optimisation des Parcours de Soins et Révision Médicale
Validation scientifique effectuée le 31/01/2025
Contenu vérifié selon les dernières recommandations médicales
8 publications dans cette catégorie
Affiliations :
Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary. farkas.henriette@med.semmelweis-univ.hu.
4 publications dans cette catégorie
Affiliations :
Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary.
Publications dans "C1 Inhibiteur" :
4 publications dans cette catégorie
Affiliations :
Department of Immunology and Histocompatibility, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece.
CeMIA SA, Larissa, Greece.
Publications dans "C1 Inhibiteur" :
4 publications dans cette catégorie
Affiliations :
Department of Biomedical and Clinical Sciences Luigi Sacco, University of Milan, Via G.B. Grassi 74, 20157 Milan, Italy.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
ASST-Fatebenefratelli-Sacco, Milan, Italy. chiarasuffritti@gmail.com.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
Department of Immunology and Histocompatibility, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa, Greece.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
CeMIA SA, Larissa, Greece.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
Department of Allergology, Navy Hospital, Athens, Greece.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
Department of Otorhinolaryngology, Head and Neck Surgery, Ulm University Medical Center, Ulm, Germany.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
Department of Otorhinolaryngology, Head and Neck Surgery, Ulm University Medical Center, Ulm, Germany.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
Department of Otorhinolaryngology, Head and Neck Surgery, Ulm University Medical Center, Ulm, Germany.
Publications dans "C1 Inhibiteur" :
3 publications dans cette catégorie
Affiliations :
Department of Translational Medicine, University of Naples, Via S. Pansini 5, 80131 Naples, Italy.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Department of Translational Medicine, Division of Medical Protein Chemistry, Lund University, Malmö, Sweden.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Sanquin Diagnostic Services, Amsterdam, The Netherlands.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Department of Immunopathology, Sanquin Research and Landsteiner Laboratory, Amsterdam University Medical Centre, Amsterdam Infection and Immunity Institute, Amsterdam, The Netherlands.
Department of Pediatric Immunology, Rheumatology, and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Center, Amsterdam, The Netherlands.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Department of Internal Medicine and Haematology, Hungarian Angioedema Center of Reference and Excellence, Semmelweis University, Szentkiralyi u. 46, Budapest, 1088, Hungary.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
Publications dans "C1 Inhibiteur" :
2 publications dans cette catégorie
Affiliations :
Department of Pediatrics, Eastern Virginia Medical School, 700 West Olney Road, Norfolk, VA 23507, USA.
Children's Specialty Group, 811 Redgate Avenue, Norfolk, VA 23507, USA.
Children's Hospital of the King's Daughters, 601 Children's Lane, Norfolk, VA 23507, USA.
Department of Microbiology and Molecular Cell Biology, Eastern Virginia Medical School, 700 West Olney Road, Norfolk, VA 23507-1696, USA.
Publications dans "C1 Inhibiteur" :
Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can...
19 patients were diagnosed with C1-INH-AAE in the Hungarian Angioedema Center of Reference and Excellence; 79% of them had an underlying disease. Samples were examined with a newly developed in-house ...
Free circulating and complex antibodies are in a dynamically changing equilibrium. CAC measurements can help to predict the development of an underlying disease. The efficiency of the treatment for un...
ACE inhibitor (ACEi) induced angioedema predominantly affects the upper aerodigestive tract. As ACEi induced angioedema is mediated by bradykinin, therapeutic response to antihistamines and glucocorti...
We performed a double-blind, parallel-group, multicentre randomised placebo-controlled trial between December 2013 and September 2018. Eligible were adults with ACEi induced angioedema with airway obs...
30 patients (16 C1INH, 14 placebo) were randomised and dosed. 25 (9 C1INH, 12 placebo) completed the study. TCER was 29.63 h ± 15.56 h in the C1INH and 17.29 h ± 10.40 h in the placebo arm (p = 0.0457...
In the context of baseline application of steroids and antihistamines C1INH was inferior in the treatment of ACEi induced angioedema when compared to placebo with respect to time to complete resolutio...
High levels of human group IIA secreted phospholipase A...
Detailed demographic data on people with hereditary angioedema (HAE) and acquired C1 inhibitor deficiency in the United Kingdom are relatively limited. Better demographic data would be beneficial in p...
To obtain more accurate data on the demographics of HAE and acquired C1 inhibitor deficiency in the United Kingdom, including treatment modalities and services available to patients....
A survey was distributed to all centers in the United Kingdom that look after patients with HAE and acquired C1 inhibitor deficiency to collect these data....
The survey identified 1152 patients with HAE-1/2 (58% female and 92% type 1), 22 patients with HAE with normal C1 inhibitor, and 91 patients with acquired C1 inhibitor deficiency. Data were provided b...
Data obtained from the survey provide useful information about the demographics and treatment modalities used in HAE and acquired C1 inhibitor deficiency in the United Kingdom. These data are useful f...
Hereditary angioedema (HAE) is a rare disorder characterized by cutaneous and submucosal swelling caused mostly by excessive local bradykinin production. Bradykinin is a vasoactive peptide generated b...
Hereditary angioedema (HAE) with normal C1-INH (HAE-nl-C1INH) is phenotypically similar to HAE resulting from C1-INH deficiency (HAE-C1INH). Confirmatory diagnostic tests for HAE-nl-C1INH are limited ...
To estimate the prevalence and describe current management patterns for HAE-nl-C1INH in the United States (US)....
We conducted an Internet-based survey of US physicians to estimate the prevalence of the HAE-nl-C1INH population in the United States. Potential participating physicians were identified from the US He...
A total of 113 physicians provided data for the estimation of HAE-nl-C1INH prevalence and 81 physicians treating HAE-nl-C1INH patients provided data about treatment patterns. In bias-corrected analysi...
These survey data provide estimates of HAE-nl-C1INH prevalence in the United States as well as current diagnosis and management strategies. Results may be useful for developing studies to assess treat...
Chronic recurrent angioedema without wheals (CRA) with normal C1 inhibitor (C1-INH) that is unresponsive to antihistamines may involve patients with recurrent angioedema of unknown cause (ie, so-calle...
To identify patients with one of type of HAEnCI in a group of patients with CRA with normal C1-INH that was unresponsive to antihistamines....
A total of 132 patients with CRA and normal C1-INH that was unresponsive to antihistamines underwent mutational and clinical analysis. The presence of hereditary angioedema-specific mutations in Facto...
In 116 of 132 solitary patients with CRA (87.9%), none of the six HAEnCI-linked mutations could be found. Ten patients (7.6%) had the Factor XII mutation c.983C>A (p.T328K) and six (4.5%) the plasmino...
A search for HAEnCI-linked mutations in patients with solitary CRA may lead to the detection of patients and families with HAEnCI. This is important because family members can be identified who are at...
Coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2 is associated with a clinical spectrum ranging from asymptomatic carriers to critically ill patients with complications including thromboemboli...
Patients with hereditary angioedema experience recurrent, sometimes life-threatening, attacks of edema. It is a rare genetic disorder characterized by genetic and clinical heterogenicity. Most cases a...
The aim was to describe trans-inhibitory effects of full-length or near full-length C1INH encoded by 28 disease-associated SERPING1 variants....
HeLa cells were transfected with expression constructs encoding the studied SERPING1 variants. Extensive and comparative studies of C1INH expression, secretion, functionality, and intracellular locali...
Our findings characterized functional properties of a subset of SERPING1 variants allowing the examined variants to be subdivided into 5 different clusters, each containing variants sharing specific m...
We provide a functional classification of SERPING1 gene variants suggesting that different SERPING1 variants drive the pathogenicity through different and in some cases overlapping molecular disease m...
Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare hereditary disease characterized by recurrent subcutaneous or submucosal angioedema due to uncontrolled bradykinin product...