المؤلفون: Tim Niehues, Catherine Waruiru, Conleth Feighery, Uwe Schauer, Virginie Courteille, Kai Lehmberg, Ingo Müller, I. Esteves, Henner Morbach, Michael Borte, Patrick Hundsdoerfer, Klaus Schwarz, Ewelina Gowin, Alessandro Aiuti, Andreas Holbro, Federica Barzaghi, João Farela Neves, Dagmar Graf, Hannah Tamary, Veneta Milenova, Benedikt Boetticher, Eleonora Gambineri, Vera Goda, Alia Eldash, Jan-Christian Wasmuth, Fabio Candotti, Svetlana O. Sharapova, Markus Metzler, Juergen Brunner, Anna Hilfanova, Brindusa Ruxandra Capilna, Pere Soler-Palacín, Arnau Antolí, Horst von Bernuth, Vassilios Lougaris, Maria Carrabba, Bernd H. Belohradsky, Julian Thalhammer, Nathalie de Vergnes, Peter Olbrich, Peter Kopač, Leif G. Hanitsch, Alexandra Nieters, Filomeen Haerynck, Juliana Gabzdilova, Sezin Aydemir, Rabab El Hawary, Patrick F.K. Yong, Maria Giovanna Danieli, Alberto Tommasini, Sandra Steinmann, Ulrich Baumann, Figen Dogu, Elisabeth Förster-Waldl, Carolina Marasco, Donato Amodio, Lorenzo Lodi, Xavier Solanich, Caterina Cancrini, Brigita Sitkauskiene, Torsten Witte, Clementina Vanessa, Nima Rezaei, Jean-Christophe Goffard, Kirsten Wittke, Emmanouil Liatsis, Helen Baxendale, Susana L. Silva, Bodo Grimbacher, Henrike Ritterbusch, Evangelia Farmaki, Safa Meshaal, Sujal Ghosh, Larysa Kostyuchenko, David Edgar, Simone Cesaro, R Zeuner, Nerea Salmón Rodríguez, Isabella Quinti, Stephan Ehl, Pauline Brosselin, Joerg C. Henes, Pilar Llobet Agulló, Rosa Maria Dellepiane, Andrea Meinhardt, Marina Kojić, Georgios Sogkas, Stephan Borte, Catharina Schuetz, Suheyla Ocak, Karin Marschall, Lukas M. Gasteiger, Stefan Raffac, Sofia Tantou, Sadia Noorani, Matthaios Speletas, Philippe Randrianomenjanahary, Ursula Holzer, Ayca Kiykim, Johannes G. Liese, Angelo Vacca, Gisela Fecker, Ekrem Unal, Koen J. van Aerde, Alba Parra-Martínez, Kaan Boztug, Sophie Stiehler, Sybille Landwehr-Kenzel, Claudio Pignata, Jennifer Neubert, Janine Reichenbach, Shahnaz Parvin, Sarah Goddard, Andrea Schroll, Dirk Holzinger, Asghar Aghamohammadi, Hassan Abolhassani, Johannes Trück, Estela Paz-Artal, Shereen M. Reda, Anna Shcherbina, Maria Raptaki, Jaroslava Orosova, Beata Wolska-Kuśnierz, Tessa Kerre, Gerrit Ahrenstorf, Ben Zion Garty, Dirk Foell, Benjamin Becker, Ulrike F. Demel, Androniki Kapousouzi, Abraham Rutgers, Klaus Warnatz, Gemma Rocamora Blanch, Stephan Rusch, Luis M. Allende, Dalia Abd Elaziz, Safa Baris, Jorisvan Montfrans, Dominik T. Schneider, Raphael Scheible, Juana Gil-Herrera, Gerhard Kindle, Annarosa Soresina, Giovanna Fabio, Uwe Wintergerst, Emilia Faria, Maria Fasshauer, Silvia Ricci, Aisha Elmarsafy, Barbara Pietrucha, Carsten Speckmann, Nizar Mahlaoui, Ulrich Heininger, Isabelle Meyts, Matthew Buckland, Efimia Papadopoulou-Alataki, Robin Kobbe, A Herwadkar, Sebastian F. N. Bode, Ali Sobh, László Maródi, Baldassarre Martire, Chiara Azzari, Maximilian Heeg, Katja Masjosthusmann, Michael H. Albert, Matteo Chinello, Juan Luis Santos-Pérez, Aarnoud Huissoon, Tanya I. Coulter, Hendrik Schulze-Koops, Norbert Graf, Radwa Alkady, Jolanta Bernatoniene, Seraina Prader, Alenka Gagro, Joachim Roesler, Taco W. Kuijpers, Ewa Więsik-Szewczyk, Maria Elena Maccari, Conrad Ferdinand Lippert, Miriam González-Amores, Johannes Dirks, Daniel E Pleguezuelo, Christof M. Kramm, Anders Fasth, Volker Schuster, Olov Ekwall, Nikolaus Rieber, Javier Carbone, Petra Kaiser-Labusch, Diana Ernst, Lucia Augusta Baselli, Luis Ignacio Gonzalez-Granado, Maria Kanariou, Stefanie S. V. Henriet, Sigune Goldacker, Kerstin Felgentreff, Oana Joean, Fine Roosens, Fabian Hauck, Eva C. Schwaneck, Milos Jesenak, Manfred Hoenig, Lenka Kapustova, Christoph Boesecke, Alain Fischer, Sara Pereira da Silva, Julia Körholz, Ansgar Schulz, Carolynne Schwarze-Zander, Mikko Seppänen, Nermeen Galal, Nora Naumann-Bartsch, Tomaz Garcez, Peter Ciznar, Klara M. Posfay-Barbe, Zelimir Pavle Eric, Reinhold E. Schmidt, Hermann J. Girschick, Sabine Heine, Anika-Kerstin Biegner, Annick A. J. M. van de Ven, Stefan Schreiber, J. Merlijn van den Berg, Nurit Assia Batzir, Alexandra Jablonka, Kim Stol, Gregor Dückers, Antonios G.A. Kolios, Ioannis Kakkas, Christian Klemann, Marina N. Guseva, Sofia Grigoriadou, Elif Karakoc-Aydiner, Antonio Marzollo, Peter D. Arkwright, Urs C. Steiner, Sara Sebnem Kilic, Romina Dieli-Crimi, Gergely Kriván, Monika Sparber-Sauer, Marco Cazzaniga, Fulvio Porta, Paraskevi Maggina, Tomas Milota, Robbert G. M. Bredius, Martine Pergent, Klaus Tenbrock, Jana Pachlopnik Schmid, Florentia Dimitriou, Cathal Laurence Steele, Helen Bourne, Anna Bobcakova, Gerd Horneff, Judith Potjewijd, Marc Schmalzing, Tobias Ankermann, Paul Ryan, Oksana Boyarchuk, Necil Kutukculer, Carl Friedrich Classen, Zita Chovancová, Moira Thomas, Cinzia Milito, Michaela Bitzenhofer-Grüber, Faranaz Atschekzei, Eva Hlaváčková, Viviana Moschese, Julie Smet, Hans-Hartmut Peter, Carla Teixeira, Sabine M El-Helou, Suzanne de Kruijf Bazen, Helmut Wittkowski, Donate Jakoby, Marina Garcia-Prat, Esther de Vries, Richard Herriot, Sven Kracker, Alessandro Plebani, Lisa Göschl, Laura Hora Marques, Anna Sediva, Jiri Litzman, Mark M. Gompels, Renate Krüger, Şefika İlknur Kökçü Karadağ, Nadine Binder, Anna Szaflarska, Peter Jandus, Lisa Ibberson, Johann Greil, Ulf Schulze-Sturm, Mehtap Sirin, Aydan Ikinciogullari, Edyta Heropolitańska-Pliszka, Michael E. Weiss, Alla Skapenko, Lukas Wisgrill, Hana Alachkar, Uta Behrends, Silvia Sánchez-Ramón, Maria N. Hatzistilianou, Otilia Petrovicova, Darko Richter, Zoreh Nademi, Jürgen K. Rockstroh, Sohilla Lotfy, Markus G. Seidel, Timothy Ronan Leahy, Audra Blažienė

المساهمون: Translational Immunology Groningen (TRIGR), Paediatric Infectious Diseases / Rheumatology / Immunology, AII - Inflammatory diseases, ARD - Amsterdam Reproduction and Development, University of Zurich, Ehl, Stephan, Thalhammer, J., Kindle, G., Nieters, A., Rusch, S., Seppanen, M. R. J., Fischer, A., Grimbacher, B., Edgar, D., Buckland, M., Mahlaoui, N., Ehl, S., Boztug, K., Brunner, J., Demel, U. F., Forster-Waldl, E., Gasteiger, L. M., Goschl, L., Kojic, M., Schroll, A., Seidel, M. G., Wintergerst, U., Wisgrill, L., Sharapova, S. O., Goffard, J. -C., Kerre, T., Meyts, I., Roosens, F., Smet, J., Haerynck, F., Eric, Z. P., Milenova, V., Gagro, A., Richter, D., Chovancova, Z., Hlavackova, E., Litzman, J., Milota, T., Sediva, A., Elaziz, D. A., Alkady, R. S., El Sayed El Hawary, R., Eldash, A. S., Galal, N., Lotfy, S., Meshaal, S. S., Reda, S. M., Sobh, A., Elmarsafy, A., Brosselin, P., Courteille, V., De Vergnes, N., Kracker, S., Pergent, M., Randrianomenjanahary, P., Ahrenstorf, G., Albert, M. H., Ankermann, T., Atschekzei, F., Baumann, U., Becker, B. C., Behrends, U., Belohradsky, B. H., Biegner, A. -K., Binder, N., Bode, S. F. N., Boesecke, C., Boetticher, B., Borte, M., Borte, S., Classen, C. F., Dirks, J., Duckers, G., El-Helou, S., Ernst, D., Fasshauer, M., Fecker, G., Felgentreff, K., Foell, D., Ghosh, S., Girschick, H. J., Goldacker, S., Graf, N., Graf, D., Greil, J., Hanitsch, L. G., Hauck, F., Heeg, M., Heine, S. I., Henes, J. C., Hoenig, M., Holzer, U., Holzinger, D., Horneff, G., Hundsdoerfer, P., Jablonka, A., Jakoby, D., Joean, O., Kaiser-Labusch, P., Klemann, C., Kobbe, R., Korholz, J., Kramm, C. M., Kruger, R., Landwehr-Kenzel, S., Lehmberg, K., Liese, J. G., Lippert, C. F., Maccari, M. E., Masjosthusmann, K., Meinhardt, A., Metzler, M., Morbach, H., Muller, I., Naumann-Bartsch, N., Neubert, J., Niehues, T., Peter, H. -H., Rieber, N., Ritterbusch, H., Rockstroh, J. K., Roesler, J., Schauer, U., Scheible, R., Schmalzing, M., Schmidt, R. E., Schneider, D. T., Schreiber, S., Schuetz, C., Schulz, A., Schulze-Koops, H., Schulze-Sturm, U., Schuster, V., Schwaneck, E. C., Schwarz, K., Schwarze-Zander, C., Sirin, M., Skapenko, A., Sogkas, G., Sparber-Sauer, M., Speckmann, C., Steinmann, S., Stiehler, S., Tenbrock, K., von Bernuth, H., Warnatz, K., Wasmuth, J. -C., Weiss, M., Witte, T., Wittke, K., Wittkowski, H., Zeuner, R. A., Farmaki, E., Hatzistilianou, M. N., Kakkas, I., Kanariou, M. G., Kapousouzi, A., Liatsis, E., Maggina, P., Papadopoulou-Alataki, E., Raptaki, M., Speletas, M., Tantou, S., Goda, V., Krivan, G., Marodi, L., Abolhassani, H., Aghamohammadi, A., Rezaei, N., Feighery, C., Leahy, T. R., Ryan, P., Batzir, N. A., Garty, B. Z., Tamary, H., Aiuti, A., Amodio, D., Azzari, C., Barzaghi, F., Baselli, L. A., Cancrini, C., Carrabba, M., Cazzaniga, M., Cesaro, S., Chinello, M., Danieli, M. G., Dellepiane, R. M., Fabio, G., Gambineri, E., Lodi, L., Lougaris, V., Marasco, C., Martire, B., Marzollo, A., Milito, C., Moschese, V., Pignata, C., Plebani, A., Porta, F., Quinti, I., Ricci, S., Soresina, A., Tommasini, A., Vacca, A., Vanessa, C., Blaziene, A., Sitkauskiene, B., Gowin, E., Heropolitanska-Pliszka, E., Pietrucha, B., Szaflarska, A., Wiesik-Szewczyk, E., Wolska-Kusnierz, B., Esteves, I., Faria, E., Marques, L. H., Neves, J. F., Silva, S. L., Teixeira, C., Pereira da Silva, S., Capilna, B. R., Guseva, M. N., Shcherbina, A., Bobcakova, A., Ciznar, P., Gabzdilova, J., Jesenak, M., Kapustova, L., Orosova, J., Petrovicova, O., Raffac, S., Kopac, P., Allende, L. M., Antoli, A., Blanch, G. R., Carbone, J., Dieli-Crimi, R., Garcia-Prat, M., Gil-Herrera, J., Gonzalez-Granado, L. I., Agullo, P. L., Olbrich, P., Parra-Martinez, A., Paz-Artal, E., Pleguezuelo, D. E., Rodriguez, N. S., Sanchez-Ramon, S., Santos-Perez, J. L., Solanich, X., Soler-Palacin, P., Gonzalez-Amores, M., Ekwall, O., Fasth, A., Bitzenhofer-Gruber, M., Candotti, F., Dimitriou, F., Heininger, U., Holbro, A., Jandus, P., Kolios, A. G. A., Marschall, K., Schmid, J. P., Posfay-Barbe, K. M., Prader, S., Reichenbach, J., Steiner, U. C., Truck, J., Bredius, R. G., de Kruijf- Bazen, S., de Vries, E., Henriet, S. S. V., Kuijpers, T. W., Potjewijd, J., Rutgers, A., Stol, K., van Aerde, K. J., Van den Berg, J. M., van de Ven, A. A. J. M., Montfrans, J., Aydemir, S., Baris, S., Dogu, F., Ikinciogullari, A., Karakoc-Aydiner, E., Kilic, S. S., Kiykim, A., Kokcu Karadag, S. I., Kutukculer, N., Ocak, S., Unal, E., Boyarchuk, O., Hilfanova, A., Kostyuchenko, L. V., Alachkar, H., Arkwright, P. D., Baxendale, H. E., Bernatoniene, J., Coulter, T. I., Garcez, T., Goddard, S., Gompels, M. M., Grigoriadou, S., Herriot, R., Herwadkar, A., Huissoon, A., Ibberson, L., Nademi, Z., Noorani, S., Parvin, S., Steele, C. L., Thomas, M., Waruiru, C., Yong, P. F. K., Bourne, H.

المصدر: Journal of Allergy and Clinical Immunology, 148(5), 1332-1341.e5. MOSBY-ELSEVIER
Journal of allergy and clinical immunology, 148(5), 1332-1341.e5. Mosby Inc.

مصطلحات موضوعية: 0301 basic medicine, Male, Pediatrics, syndromic, Sex Factor, Disease, registry, medicine.disease_cause, Cohort Studies, 0302 clinical medicine, Primary Immunodeficiency Disease, inborn error of immunity, Immunology and Allergy, warning signs, Age Factor, Registries, Family history, presenting symptom, Child, Primary immunodeficiency, Granuloma, autoimmune, immune dysregulation, inflammatory, Adult, Autoimmune Diseases, Female, Humans, Infections, Lymphoproliferative Disorders, Middle Aged, Primary Immunodeficiency Diseases, Sex Factors, Age Factors, 10177 Dermatology Clinic, Infections/epidemiology, 3. Good health, Settore MED/02, Warning signs, Lymphoproliferative Disorder, 2723 Immunology and Allergy, Infection, Human, medicine.medical_specialty, Immunology, 610 Medicine & health, Malignancy, primary immunodeficiency, Autoimmune Disease, 03 medical and health sciences, Immunity, Autoimmune Diseases/epidemiology, medicine, 2403 Immunology, business.industry, warning sign, Common variable immunodeficiency, Granuloma/epidemiology, Immune dysregulation, medicine.disease, Primary Immunodeficiency Diseases/epidemiology, 030104 developmental biology, Lymphoproliferative Disorders/epidemiology, Cohort Studie, business, 030215 immunology

الوصف: BACKGROUND: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.OBJECTIVE: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts.METHODS: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered.RESULTS: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years.CONCLUSIONS: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.

وصف الملف: Thalhammer_JACI_2021_(PID_16486).pdf - application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::002c11f0424fbe10dfdb30679532b4b5Test
https://research.rug.nl/en/publications/5db77f56-3077-43ca-b935-ffa87bb6cbfbTest

المؤلفون: Linlin Yang, Claire Booth, Carsten Speckmann, Markus G. Seidel, Austen J.J. Worth, Gerhard Kindle, Arjan C. Lankester, Bodo Grimbacher, Andrew R. Gennery, Mikko R.J. Seppanen, Emma C. Morris, Siobhan O. Burns, Anna Sediva, Benedicte Neven, Fabian Hauck, Klaus Warnatz, Malgorzata Pac, Maria Carrabba, Pere Palacin, Peter Jandus, Ann Gardulf, Nizar Mahlaoui, Martine Pergent, Catharina Schutz, Svetlana Sharapova, Lougaris Vassilios, Fabio Candotti, Stephano Volpi

المصدر: Journal of Allergy and Clinical Immunology: In Practice, 150(2), 456-466. ELSEVIER

مصطلحات موضوعية: therapy, Genotype, phenotype, survival outcomes, adult, Immunology, IBD, Hematopoietic Stem Cell Transplantation, Apoptosis, Genetic Diseases, X-Linked, X-Linked Inhibitor of Apoptosis Protein, conservative treatment, primary immunodeficiency, Lymphohistiocytosis, Hemophagocytic, Lymphoproliferative Disorders, HSCT, XIAP deficiency, Humans, Immunology and Allergy, X-linked inhibitor of apoptosis, Retrospective Studies, HLH

الوصف: Background: X-linked inhibitor of apoptosis (XIAP) deficiency is a rare primary immunodeficiency disease caused by XIAP gene mutations. A broad range of phenotype, severity, and age at onset present challenges for patient management.Objective: We sought to characterize the phenotype, treatment, and survival outcomes of XIAP deficiency and to assess parameters influencing prognosis.Methods: Data published from 2006 to 2020 were retrospectively analyzed.Results: A total of 167 patients from 117 families with XIAP deficiency were reported with 90 different mutations. A wide spectrum of clinical features were seen, of which hemophagocytic lymphohistiocytosis (HLH) and inflammatory bowel disease were the most common. Patients frequently developed multiple features with no clear genotype-phenotype correlation. A total of 117 patients were managed conservatively and 50 underwent hematopoietic stem-cell transplantation (HSCT), with respective overall survival probabilities of 90% and 53% at age 16 years. The predominant indication for HSCT was early-onset HLH. Active HLH and myeloablative conditioning regimens increased HSCT-related mortality, although HSCT outcome was much better after 2015 than before. For conservatively managed patients reaching adulthood, survival probabilities were 86% at age 30 years and 37% by age 52 years, with worse outcomes for patients developing the disease before the age of 5 years or with new disease features in adulthood. Nine asymptomatic mutation carriers with a median age of 13.5 years were identified.Conclusions: Our study demonstrates the variable nature of XIAP deficiency, which evolves over life for individual patients. Better therapeutic strategies and prospective studies are required to reduce morbidity and mortality and improve decision making and long-term outcomes for patients with XIAP deficiency. (J Allergy Clin Immunol 2022;150:456-66.)

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::00befebbe6490b40844af71754643614Test
https://hdl.handle.net/1887/3575913Test

المؤلفون: Omar, Zgheib, Véronique, Trombert, Peter, Jandus, Christine, Serratrice

المصدر: BMJ case reports, Vol. 15, No 2 (2022) P. e248229

مصطلحات موضوعية: ddc:616, Unwanted effects / adverse reactions, Male, Anti-Inflammatory Agents, Non-Steroidal / adverse effects, Colitis, Ulcerative / drug therapy, Drug Hypersensitivity Syndrome / drug therapy, Anti-Inflammatory Agents, Non-Steroidal, Immunology, General Medicine, Gastrointestinal system, Ulcerative colitis, Pharmaceutical Preparations, Mesalamine / adverse effects, Drug Hypersensitivity Syndrome, ddc:618.97, Drug Hypersensitivity Syndrome / diagnosis, Drug Hypersensitivity Syndrome / etiology, Humans, Colitis, Ulcerative, Mesalamine, Aged

الوصف: Mesalazine is often used as first-line therapy for ulcerative colitis. Several reports have pointed to systemic adverse reactions associated with this drug. Most have evoked a drug-induced hypersensitivity syndrome, while some have described lupus syndromes but with limited clinical and varied biological features. A 75-year-old man presented with fever, dyspnoea, chest pain, polyarthralgia, and myalgia, following mesalazine introduction. Clinical symptoms and low-titre positive antihistone antibodies disappeared after mesalazine withdrawal without recourse to steroids. Pericardial effusion and 8F-fluorodeoxyglucose uptake on positron emission tomography/CT scan, and glomerular haematuria and proteinuria also disappeared. Cytokine-lymphocyte transformation tests showed a strong sensitisation pattern with interleukin-5 production. This case advances our knowledge of the mechanism of mesalazine-induced adverse effects, namely via drug-induced hypersensitivity with lupus manifestations, which we are the first to report.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7f11cdc245e571e8bdd7a1208028f24dTest
https://archive-ouverte.unige.ch/unige:159741Test

المؤلفون: Yann Coattrenec, L Ibrahim Yasmine, David Spoerl, Peter Jandus, Thomas Harr

المصدر: Journal of Investigational Allergology & Clinical Immunology, Vol. 30, No 1 (2020) pp. 58-59

مصطلحات موضوعية: Male, Pediatrics, medicine.medical_specialty, Eosinophilia/drug therapy, Omalizumab/therapeutic use, Immunology, Treatment outcome, MEDLINE, Omalizumab, ddc:616.07, Remission induction, Anti-Allergic Agents, Eosinophilia, Immunology and Allergy, Medicine, Humans, Cellulitis/drug therapy, Anti-Allergic Agents/therapeutic use, Skin pathology, Skin, Aged, ddc:616, business.industry, Skin/drug effects/pathology, Remission Induction, Cellulitis, Eosinophils/drug effects/immunology, medicine.disease, Eosinophils, Treatment Outcome, Wells syndrome, Eosinophilic cellulitis, Long term remission, business, medicine.drug

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::06d995be5d84bea32fa6a5a8271dba78Test
https://archive-ouverte.unige.ch/unige:145575Test

المؤلفون: Richard D. Cummings, Mai M. Abdelhafez, Bodo Grimbacher, Robert Rieben, Peter Jandus, Camilla Jandus, Stephan von Gunten, Hans-Uwe Simon, Dagmar Simon, Kayluz Frias Boligan, David F. Smith, Elisabeth de Graauw, Nicolai V. Bovin, Alain Despont

المصدر: Blood, Vol. 134, No 22 (2019) pp. 1941-1950
Jandus, Peter; Frias Boligan, Kayluz; Smith, David F; de Graauw, Elisabeth; Grimbacher, Bodo; Jandus, Camilla; Abd El Hafez, Mai Moustafa Ahmed; Despont, Alain; Bovin, Nicolai; Simon, Dagmar; Rieben, Robert; Simon, Hans-Uwe; Cummings, Richard D; von Gunten, Stephan (2019). The architecture of the IgG anti-carbohydrate repertoire in primary antibody deficiencies (PADs). Blood, 134(22), pp. 1941-1950. American Society of Hematology 10.1182/blood.2019001705 <http://dx.doi.org/10.1182/blood.2019001705Test>

مصطلحات موضوعية: Male, Glycan, Immunobiology and Immunotherapy, Primary Immunodeficiency Diseases, Immunology, Carbohydrates, 610 Medicine & health, medicine.disease_cause, Autoantigens, Biochemistry, Immunoglobulin G, Epitope, Autoimmunity, Epitopes, Immune system, Antibody Repertoire, Antigen, medicine, Humans, ddc:616, biology, Repertoire, Cell Biology, Hematology, biology.protein, Female

الوصف: Immune system failure in primary antibody deficiencies (PADs) has been linked to recurrent infections, autoimmunity, and cancer, yet clinical judgment is often based on the reactivity to a restricted panel of antigens. Previously, we demonstrated that the human repertoire of carbohydrate-specific immunoglobulin G (IgG) exhibits modular organization related to glycan epitope structure. The current study compares the glycan-specific IgG repertoires between different PAD entities. Distinct repertoire profiles with extensive qualitative glycan-recognition defects were observed, which are characterized by the common loss of Galα and GalNAc reactivity and disease-specific recognition of microbial antigens, self-antigens, and tumor-associated carbohydrate antigens. Antibody repertoire analysis may provide a useful tool to elucidate the degree and the clinical implications of immune system failure in individual patients.

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f2dc17170cd97727c4cbe1bf4339086dTest
https://archive-ouverte.unige.ch/unige:132415Test

المؤلفون: Thomas Harr, Peter Jandus

المصدر: Journal of Investigational Allergology and Clinical Immunology, Vol. 28, No 3 (2018) pp. 203-205

مصطلحات موضوعية: Adult, medicine.medical_specialty, Delayed Diagnosis, Gadolinium, media_common.quotation_subject, Immunology, Contrast Media, chemistry.chemical_element, Drug Hypersensitivity, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Immunology and Allergy, Contrast (vision), 030212 general & internal medicine, Anaphylaxis, Skin Tests, media_common, ddc:616, medicine.diagnostic_test, business.industry, Anaphylactic reaction, Magnetic resonance imaging, Magnetic Resonance Imaging, 030228 respiratory system, Late diagnosis, chemistry, Intradermal test, Female, Radiology, business

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4631a45aa7ab06336bae92a5c5719debTest
https://archive-ouverte.unige.ch/unige:115271Test

المؤلفون: Pedro Romero, Peter Jandus, Camilla Jandus, Bérengère Salomé, Arthur Helbling, Selena Vigano, Alexandre Harari, Sara Trabanelli, F Indulsi

المصدر: Journal of Investigational Allergology and Clinical Immunology, Vol. 27, No 6 (2017) pp. 394-396

مصطلحات موضوعية: Immunology, 03 medical and health sciences, 0302 clinical medicine, Agammaglobulinemia, Immunology and Allergy, Medicine, Humans, In patient, Lymphocyte Count, 610 Medicine & health, ddc:616, biology, business.industry, Immunoglobulins, Intravenous, Primary and secondary antibodies, Immunity, Innate, Lymphocyte Subsets, Institutional repository, 030220 oncology & carcinogenesis, biology.protein, Cytokines, Antibody, business, Biomarkers, 030215 immunology, Lymphocyte subsets

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::399f0dc83e9ae7f7f2feac25449de680Test
https://archive-ouverte.unige.ch/unige:112553Test

المؤلفون: Thomas Harr, David Spoerl, Peter Jandus

المصدر: The Journal of Allergy and Clinical Immunology: In Practice, Vol. 4, No 5 (2016) pp. 991-2

مصطلحات موضوعية: ddc:616, medicine.medical_specialty, Allergy, business.industry, medicine.drug_class, Biguanide, Chlorhexidine, Chlorhexidine allergy, medicine.disease, Dermatology, 03 medical and health sciences, 0302 clinical medicine, 030228 respiratory system, Antiseptic, Immunology, medicine, Immunology and Allergy, 030212 general & internal medicine, business, Allergic contact dermatitis, Anaphylaxis, medicine.drug, Asthma

الوصف: Chlorhexidine (CHX) is a synthetic biguanide available in different forms (diacetate, dihydrochloride, and digluconate) and used as an antiseptic. In the past few years, there has been a substantial increase in the number of CHX-containing products used in health care. Hypersensitivity reactions to CHX are increasingly reported and include allergic contact dermatitis, photosensitivity, fixed drug eruptions, asthma, and lifethreatening anaphylaxis. Here, we report 3 cases of anaphylaxis due to CHX with a particular course, highlighting possible pitfalls and peculiarities in CHX allergy.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2245b04920fe6db53fa94df978fa92e1Test