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2. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations

Author

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

Published
2021
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3. Long-term outcomes of 176 patients with X-linked hyper-IgM syndrome treated with or without hematopoietic cell transplantation.

Author

de la Morena, M Teresa, Leonard, David, Torgerson, Troy R, Cabral-Marques, Otavio, Slatter, Mary, Aghamohammadi, Asghar, Chandra, Sharat, Murguia-Favela, Luis, Bonilla, Francisco A, Kanariou, Maria, Damrongwatanasuk, Rongras, Kuo, Caroline Y, Dvorak, Christopher C, Meyts, Isabelle, Chen, Karin, Kobrynski, Lisa, Kapoor, Neena, Richter, Darko, DiGiovanni, Daniela, Dhalla, Fatima, Farmaki, Evangelia, Speckmann, Carsten, Español, Teresa, Shcherbina, Anna, Hanson, Imelda Celine, Litzman, Jiri, Routes, John M, Wong, Melanie, Fuleihan, Ramsay, Seneviratne, Suranjith L, Small, Trudy N, Janda, Ales, Bezrodnik, Liliana, Seger, Reinhard, Raccio, Andrea Gomez, Edgar, J David M, Chou, Janet, Abbott, Jordan K, van Montfrans, Joris, González-Granado, Luis Ignacio, Bunin, Nancy, Kutukculer, Necil, Gray, Paul, Seminario, Gisela, Pasic, Srdjan, Aquino, Victor, Wysocki, Christian, Abolhassani, Hassan, Dorsey, Morna, Cunningham-Rundles, Charlotte, Knutsen, Alan P, Sleasman, John, Costa Carvalho, Beatriz Tavares, Condino-Neto, Antonio, Grunebaum, Eyal, Chapel, Helen, Ochs, Hans D, Filipovich, Alexandra, Cowan, Mort, Gennery, Andrew, Cant, Andrew, Notarangelo, Luigi D, and Roifman, Chaim M

Subjects
Humans, Hematopoietic Stem Cell Transplantation, Proportional Hazards Models, Retrospective Studies, Cohort Studies, Follow-Up Studies, Time, Adolescent, Adult, Middle Aged, Child, Child, Preschool, Infant, Female, Male, Hyper-IgM Immunodeficiency Syndrome, Young Adult, Kaplan-Meier Estimate, CD40 ligand, Karnofsky/Lansky scores, X-linked hyper-IgM syndrome, defects in class-switch recombination, hematopoietic cell transplantation, long-term outcomes, primary immunodeficiency, Pediatric, Clinical Research, Transplantation, Inflammatory and immune system, Good Health and Well Being, Immunology, Allergy
Abstract

BackgroundX-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency with high morbidity and mortality compared with those seen in healthy subjects. Hematopoietic cell transplantation (HCT) has been considered a curative therapy, but the procedure has inherent complications and might not be available for all patients.ObjectivesWe sought to collect data on the clinical presentation, treatment, and follow-up of a large sample of patients with XHIGM to (1) compare long-term overall survival and general well-being of patients treated with or without HCT along with clinical factors associated with mortality and (2) summarize clinical practice and risk factors in the subgroup of patients treated with HCT.MethodsPhysicians caring for patients with primary immunodeficiency diseases were identified through the Jeffrey Modell Foundation, United States Immunodeficiency Network, Latin American Society for Immunodeficiency, and Primary Immune Deficiency Treatment Consortium. Data were collected with a Research Electronic Data Capture Web application. Survival from time of diagnosis or transplantation was estimated by using the Kaplan-Meier method compared with log-rank tests and modeled by using proportional hazards regression.ResultsTwenty-eight clinical sites provided data on 189 patients given a diagnosis of XHIGM between 1964 and 2013; 176 had valid follow-up and vital status information. Sixty-seven (38%) patients received HCT. The average follow-up time was 8.5 ± 7.2 years (range, 0.1-36.2 years). No difference in overall survival was observed between patients treated with or without HCT (P = .671). However, risk associated with HCT decreased for diagnosis years 1987-1995; the hazard ratio was significantly less than 1 for diagnosis years 1995-1999. Liver disease was a significant predictor of overall survival (hazard ratio, 4.9; 95% confidence limits, 2.2-10.8; P

Published
2017

11. Alergenska imunoterapija u alergijskim bolestima.

Author

Navratil, Marta, Bandić, Davor, Čavčić, Anamarija, Bukvić, Blaženka Kljaić, Jureković, Irena Ivković, Pelčić, Gordana, Rogulj, Marijana, Šegulja, Silvije, Turkalj, Mirjana, and Richter, Darko

Subjects
REGULATORY B cells, REGULATORY T cells, TRANSFORMING growth factors-beta, T helper cells, TOLERATION, FOOD allergy, TERMINATION of treatment, ACHIEVEMENT
Abstract

Copyright of Lijecnicki Vjesnik is the property of Croatian Medical Association and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2023
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13. The approach to a child with severe asthma

Author

Kljaić Bukvić, Blaženka, primary, Ivković Jureković, Irena, additional, Navratil, Marta, additional, Rogulj, Marijana, additional, Bandić, Davor, additional, Kelečić, Jadranka, additional, Šegulja, Silvije, additional, and Richter, Darko, additional

Published
2022
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14. From phenotype to biological treatment of severe asthma

Author

Kljaić Bukvić, Blaženka, primary, Ivković Jureković, Irena, additional, Navratil, Marta, additional, Rogulj, Marijana, additional, Bandić, Davor, additional, Kelečić, Jadranka, additional, Šegulja, Silvije, additional, and Richter, Darko, additional

Published
2022
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15. Pristup djetetu s teškom astmom

Author

Kljaić Bukvić, Blaženka, Navratil, Marta, Rogulj, Marijana, Bandić, Davor, Kelečić, Jadranka, Šegulja, Silvije, Richter, Darko, Kljaić Bukvić, Blaženka, Navratil, Marta, Rogulj, Marijana, Bandić, Davor, Kelečić, Jadranka, Šegulja, Silvije, and Richter, Darko

Abstract

Teška astma je složena i heterogena bolest obilježena neprimjerenom kontrolom unatoč visokom stupnju liječenja. Procjenjuje se kako je prisutna u 2 - 5% djece s astmom, češće u djece starije od 10 godina i u dječaka. Iako je teška astma rijetka, ova skupina djece ima povećani rizik nuspojava liječenja, kao i teških i po život opasnih egzacerbacija. Povećani su izravni i neizravni troškovi u vidu potrošnje lijekova, češćih redovitih i hitnih posjeta liječniku, hospitalizacija, izostanaka s nastave i roditeljskih odsustava s posla. Definicije teške astme nisu ujednačene, ali im je svima zajednička potreba za kombinacijskim liječenjem inhalacijskim kortikosteroi- dima i bronhodilatatorima dugog djelovanja u visokim dozama neophodnima za kontrolu simptoma, ili, čak nedostatnima za punu kontrolu bolesti. Nekontrolirana astma zahtijeva iscrpno preispitivanje koje uključuje diferencijalno dijagnostičku reevaluaciju i nedvojbenu potvrdu dijagnoze astme, otkrivanje otegotnih čimbenika poput neredovitog uzimanja i/ili loše tehnike primjene inha- lacijskih lijekova, utjecaja okoliša (izloženost alergenima i iritansima) te komorbiditeta (bolesti gornjeg dišnog puta, gastroezofagu- sni refluks, pretilost i anksioznost). Ukoliko je dijagnoza astme potvrđena, a otegotni čimbenici ispravljeni, te je postignuta kontrola bolesti, radi se o teško lječivoj astmi (engl. difficult-to-treat asthma). Ako se ovim mjerama ne postigne kontrola, radi se o teškoj rezi- stentnoj astmi (engl. severe, therapy-resistant asthma). U pristupu djetetu s teško lječivom astmom slijedimo sistematičnu evaluaciju kojom u približno dvije trećine bolesnika djelovanjem na modificirajuće čimbenike ispunjavamo dugoročne ciljeve liječenja kroz postizanje kontrole simptoma te smanjenje rizika za po- goršanja i nepovratna oštećenja bronha/komplikacija liječenja. U preostale djece, u koje primjena ovih mjera ne dovodi do usposta- ve kontrole, radi se o teškoj, na terapiju rezistentnoj astmi. U njih slijedi, kroz odr, Severe asthma is considered a complex and heterogeneous disease, which includes different phenotypes, defined in terms of both clinical and molecular characteristics and underlining endotypes. It is estimated that severe asthma affects 2-5% of all children with asthma. It occurs more frequently in children older than ten years of age, with a slight prevalence among the male sex. Although severe asthma is uncommon, this group of children has an increased risk of drug side effects and life-threatening exacerbations that impair quality of life. Also, the financial burden from medication, scheduled and unscheduled doctor visits, hospitalizations and absence from school and work by parents have to be considered. There is no uniform definition of severe asthma, but the common characteristic is the need for maximal maintenance therapy, including high-dose inhaled steroids, long-acting beta-agonists, and/ or leukotriene receptor antagonists/theophylline. Despite the highest doses of maintenance therapy, patients with severe asthma fail to control the disease. Uncontrolled asthma has to be re-evaluated by confirming the diagnosis and modifying factors contribut- ing to symptoms and exacerbations like poor adherence, environmental risks (persistent allergen and pollutant exposure) and co- morbidities (upper airway disease, gastroesophageal reflux, obesity, anxiety). Children with poor asthma control due to misdiagnosed asthma, poor adherence or environmental risks have difficult-to-treat asthma, whereas children who still have poor control despite re-education to improve adherence and modification of environmen- tal risks have severe, therapy-resistant asthma. The approach to children with difficult-to-treat-asthma, which includes systematic evaluation and acting on modifying factors, enables achieving the long-term goals of asthma treatment in approximately two-thirds of patients. The remaining children, whose asthma is still uncontrolled despite optimized therapy, have seve

Published
2022

16. Od fenotipa do biološkog liječenja teške astme

Author

Kljaić Bukvić, Blaženka, Ivković Jureković, Irena, Navratil, Marta, Rogulj, Marijana, Bandić, Davor, Kelečić, Jadranka, Šegulja, Silvije, Richter, Darko, Kljaić Bukvić, Blaženka, Ivković Jureković, Irena, Navratil, Marta, Rogulj, Marijana, Bandić, Davor, Kelečić, Jadranka, Šegulja, Silvije, and Richter, Darko

Abstract

Teška astma je složena i heterogena bolest koja kliničara stavlja pred težak zadatak razlikovanja bolesnika prema rizičnim manife- stacijama bolesti (fenotipovi) i specifičnim patofiziološkim mehanizmima u podlozi bolesti (endotipovi), a sve u cilju odabira što učinkovitijeg liječenja prilagođenog potrebama pojedinog bolesnika. Dosadašnja istraživanja i pokušaji fenotipiziranja bolesnika s astmom, pa tako i teškom astmom, kod djece nisu dovela do jedno- značnog zaključka, osim već otprije poznatih činjenica da najveći dio djece ima atopiju s višestrukim preosjetljivostima (nerijetko kombinacija nutritivnih i aeroalergena, uloga plijesni), reverzibilnu bronhoopstrukciju i rane znakove remodelacije bronha. Samo mali broj djece ima trajnu bronhoopstrukciju (FEV1< 80%). Endotipovi teške astme ne razlikuju se od onih opisanih u astmi općenito: tip 2 (visoki Th2; eozinofili u serumu i sputumu, visoki IgE, FeNO; ključni citokini IL-4, IL-5, IL-13) i ne-tip 2 (niski Th2, neutrofilna, pau- cigranulocitna ili miješana upala; ključni citokini IL-8, IL-17, IL-22). Tip 2 je češći endotip kod djece i za njega postoji dostupna i odo- brena biološka terapija (anti-IgE i anti-IL-5). Ne-tip 2 je rjeđi endotip, obilježen općenito ograničenim terapijskim opcijama, među kojima se razmatra primjena azitromicina. Teška astma, iako kod djece rijetko zastupljena, predstavlja rizični fenotip koji ozbiljno narušava kvalitetu života. Pažljivo praćenje bolesnika i određivanje temeljnog endotipa omogućuje izbor i primjenu ciljanog i personaliziranog liječenja., Severe asthma is considered a complex and heterogeneous disease, which includes different phenotypes, defined in terms of both clinical and molecular characteristics and different underlining endotypes. According to different studies, there are several clinical phenotypes of severe asthma in children. Most children are allergic to multiple aeroallergen sensitization (house dust mites, pollen, molds) and have high levels of total and specific IgE, reversible airflow obstruc- tion and early signs of remodelation. A small subgroup of children has persistent airflow limitation (FEV1 <80% predicted). There are two major underlining functional or pathophysiologic mechanisms for different phenotypes of asthma and severe asthma accord- ing to the immune mechanism: Type 2 asthma (Th2-high asthma, eosinophils in serum and sputum, high IgE levels, high FeNO; key cytokines IL-4, IL-5, IL-13) and non-Type 2 asthma (Th2-low asthma, neutrophilic, paucigranulocytic and mixed granulocytic inflam- mation; key cytokines IL-8, IL-17, IL-22). The type 2 asthma endotype is more common in children, while biomarkers involved in the pathogenesis, such as IgE and IL-5 have become targets for biological therapy. The non-type 2 asthma endotype, less frequent in children with severe asthma, has fewer therapeutic options. The effect of azithromycin is still under investigation. Severe asthma, although uncommon, is a complex and high-risk phenotype of childhood asthma. Close monitoring of the patient and precise definition of underlying endotype during evaluation enables identification and use of personalized, endotype-targeted treatment.

Published
2022

17. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations

Author

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

Published
2021
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19. 155 Clinical, immunological and genetic findings in 22 patients with combined immunodeficiency treated in a specialized center in Croatia

Author

Cigrovski, Nevenka, primary, Richter, Darko, additional, Tješić-Drinković, Dorian, additional, Bambir, Ivan, additional, Batinić, Drago, additional, Babić, Antonija, additional, Kurić, Lejla, additional, Bojanić, Ines, additional, Grubić, Zorana, additional, Tešović, Goran, additional, Vrdoljak, Maja, additional, and Kelečić, Jadranka, additional

Published
2021
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20. Clinical, immunological and genetic findings in 22 patients with combined immunodeficiency treated in a specialized center in Croatia

Author

Cigrovski, Nevenka, Richter, Darko, Tješić- Drinković Dorian, Bambir, Ivan, Batinić, Drago, Babić, Antonija, Kurić, Lejla, Bojanić, Ines, Grubić, Zorana, Tešović, Goran, Vrdoljak, Maja, and Kelečić, Jadranka

Subjects
combined immunodeficiency
Abstract

To gain insight into the peculiarities of clinical presentation and time of diagnosis in the population of patients with combined immunodeficiency treated in our center. We retrospectively analyzed 22 patients with combined immunodeficiency treated in our center in the past 25 years. Clinical findings were collected, including age of presentation and age of diagnosis, genetic defects, consanguinity, family history, number and site of infections, presence of diarrhea, failure to thrive, skin rash, vaccine complications and type of treatment. Immunological findings included WBC with absolute counts, lymphocyte subsets and immunoglobulin levels. Patients (n=22) with severe combined immunodeficiency (n=17) and other combined immunodeficiencies (n=5) were included in the study. Sixteen patients (77%) were male. The most common clinical manifestations were opportunistic infections. Most of the patients were diagnosed after 3 months of age. Prenatal diagnosis was performed in one patient (4.5%), two patients (9%) did not undergo genetic analysis. The spectrum of genetic defect in our cohort show wide heterogeneity. Seventeen patients (77%) underwent HSCT, one patient (4.5%) is on enzyme replacement therapy. None of our patients have undergone gene therapy. Twelve patients (54.5%) died, of which four (18%) died before HSCT. Combined immunodeficiencies are fatal without definitive therapy (HSCT or gene therapy). The outcomes are better if infants are diagnosed and treated in pre-symptomatic stage, preferably before 3.5 months of age. More and more countries are implementing newborn screening for primary immunodeficiency diseases in their health care systems to diagnose patients as early as possible and to prevent infections. For the same reason, we advocate for expanding our national neonatal screening program

Published
2021

25. Revisiting human IL-12R(beta)1 deficiency: a survey of 141 patients from 30 countries

Author

de Beaucoudrey, Ludovic, Samarina, Arina, Bustamante, Jacinta, Cobat, Aurelie, Boisson-Dupuis, Stephanie, Feinberg, Jacqueline, Al-Muhsen, Saleh, Janniere, Lucile, Rose, Yoann, de Suremain, Maylis, Kong, Xiao-Fei, Filipe-Santos, Orchidee, Chapgier, Ariane, Picard, Capucine, Fischer, Alain, Dogu, Figen, Ikinciogullari, Aydan, Tanir, Gonul, Al-Hajjar, Sami, Al-Jumaah, Suliman, Frayha, Husn H., AlSum, Zobaida, Al-Ajaji, Sulaiman, Alangari, Abdullah, Al-Ghonaium, Abdulaziz, Adimi, Parisa, Mansouri, Davood, Ben-Mustapha, Imen, Yancoski, Judith, Garty, Ben-Zion, Rodriguez-Gallego, Carlos, Caragol, Isabel, Kutukculer, Necil, Kumararatne, Dinakantha S., Patel, Smita, Doffinger, Rainer, Exley, Andrew, Jeppsson, Olle, Reichenbach, Janine, Nadal, David, Boyko, Yaryna, Pietrucha, Barbara, Anderson, Suzanne, Levin, Michael, Schandene, Liliane, Schepers, Kinda, Efira, Andre, Mascart, Francoise, Matsuoka, Masao, Sakai, Tatsunori, Siegrist, Claire-Anne, Frecerova, Klara, Bluetters-Sawatzki, Renate, Bernhoft, Jutta, Freihorst, Joachim, Baumann, Ulrich, Richter, Darko, Haerynck, Filomeen, De Baets, Frans, Novelli, Vas, Lammas, David, Vermylen, Christiane, Tuerlinckx, David, Nieuwhof, Chris, Pac, Malgorzata, Haas, Walther H., Muller-Fleckenstein, Ingrid, Fleckenstein, Bernhard, Levy, Jacob, Raj, Revathi, Cohen, Aileen Cleary, Lewis, David B., Holland, Steven M., Yang, Kuender D., Wang, Xiaochuan, Wang, Xiaohong, Jiang, Liping, Yang, Xiqiang, Zhu, Chaomin, Xie, Yuanyuan, Lee, Pamela Pui Wah, Chan, Koon Wing, Chen, Tong-Xin, Castro, Gabriela, Natera, Ivelisse, Codoceo, Ana, King, Alejandra, Bezrodnik, Liliana, Di Giovani, Daniela, Gaillard, Maria Isabel, de Moraes-Vasconcelos, Dewton, Grumach, Anete Sevciovic, da Silva Duarte, Alberto Jose, Aldana, Ruth, Espinosa-Rosales, Francisco Javier, Bejaoui, Mohammed, Bousfiha, Ahmed Aziz, El Baghdadi, Jamila, Ozbek, Namik, Aksu, Guzide, Keser, Melike, Somer, Ayper, Hatipoglu, Nevin, Aydogmus, Cigdem, Asilsoy, Suna, Camcioglu, Yildiz, Gulle, Saniye, Ozgur, Tuba T., Ozen, Meteran, Oleastro, Matias, Bernasconi, Andrea, Mamishi, Setareh, Parvaneh, Nima, Rosenzweig, Sergio, Barbouche, Ridha, Pedraza, Sigifredo, Lau, Yu Lung, Ehlayel, Mohammad S., Fieschi, Claire, Abel, Laurent, Sanal, Ozden, and Casanova, Jean-Laurent

Subjects
Interleukin-12 -- Physiological aspects, Interleukin-12 -- Abnormalities, Mycobacterial infections -- Risk factors, Mycobacterial infections -- Distribution, Mycobacterial infections -- Surveys, Disease susceptibility -- Surveys, Mendel's law -- Analysis, Company distribution practices
Published
2010

26. Genetic and demographic features of X-linked agammaglobulinemia in Eastern and Central Europe: A cohort study

Author

Tóth, Beáta, Volokha, Alla, Mihas, Alexander, Pac, Malgorzata, Bernatowska, Ewa, Kondratenko, Irina, Polyakov, Alexander, Erdős, Melinda, Pasic, Srdjan, Bataneant, Michaela, Szaflarska, Anna, Mironska, Kristina, Richter, Darko, Stavrik, Katarina, Avcin, Tadej, Márton, Gabriella, Nagy, Kálmán, Dérfalvi, Beáta, Szolnoky, Miklós, Kalmár, Ágnes, Belevtsev, Michael, Guseva, Marina, Rugina, Aurica, Kriván, Gergely, Timár, László, Nyul, Zoltán, Mosdósi, Bernadett, Kareva, Lidija, Peova, Sonja, Chernyshova, Liudmyla, Gherghina, Ioan, Serban, Margit, Conley, Mary Ellen, Notarangelo, Luigi D., Smith, Edvard C.I., van Dongen, Jacques, van der Burg, Mirjam, and Maródi, László

Published
2009
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27. Od fenotipa do biološkog liječenja teške astme.

Author

Bukvić, Blaženka Kljaić, Ivković-Jureković, Irena, Navratil, Marta, Rogulj, Marijana, Bandić, Davor, Kelečić, Jadranka, Šegulja, Silvije, and Richter, Darko

Subjects
HOUSE dust mites, ASTHMA in children, BIOTHERAPY, PATIENT monitoring, ASTHMA, ATOPY, MILK allergy
Abstract

Copyright of Paediatria Croatica is the property of Paediatria Croatica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2022
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28. Pristup djetetu s teškom astmom.

Author

Bukvić, Blaženka Kljaić, Ivković-Jureković, Irena, Navratil, Marta, Rogulj, Marijana, Bandić, Davor, Kelečić, Jadranka, Šegulja, Silvije, and Richter, Darko

Subjects
DRUG side effects, SCHOOL attendance, SCHOOL absenteeism, JOB absenteeism, ASTHMA in children, COUGH
Abstract

Copyright of Paediatria Croatica is the property of Paediatria Croatica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2022
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29. Neobični oblik anafilaksije, desenzitizacija i provokacija lijekom

Author

Richter, Darko and Ivković-Jureković, Irena

Subjects
anafilaksija ovisna o hrani a potaknuta naporom, napor, provokacijski pokus, lijek, desenzitizacija
Abstract

Neobičnim oblikom anafilaksije nazivamo događaje koji klinički jesu anafilaksija, a onda i patogenetski, samo što je patogenetski put neuobičajen ili sastavljen od nekoliko faktora. To je npr. anafilaksija ovisna o hrani, a potaknuta naporom. Provokacijski pokus lijekom je kontrolirana primjena lijeka kako bi se uz najmanji mogući rizik otkrila ili isključila moguća reakcija preosjetljivosti. Prethodno se klinički istražuje izgled reakcije, vjerojatni lijek i vremenski razmak od početka uzimanja lijeka i od zadnje primljene doze. Desenzitizacija jest indukcija nereaktivnosti na lijek koji je prethodno bio uzrokom reakcije preosjetljivosti. Oboje su visoko rizične intervencije koje valja provoditi u jedinicama spremnim za intervenciju u slučaju anafilatičkog šoka. Razlike između provokacije i desenzitizacije u indikaciji su intencijske, a u provedbi kvantitativne. Provokacijski se pokus ne smije pretvoriti u desenzitizaciju. Zato se provokacija počinje većim dozama nego je to slučaj kod desenzitizacije. Ako se krene minimiziranim dozama može se napraviti učinak desenzitizacije, i neće biti vidljive reakcije na lijek, ali, nakon samo kraćeg vremena (svega nekoliko sati ili dana), ponovnom primjenom možemo prouzročiti burnu reakciju preosjetljivosti.

Published
2019

30. Anafilaksija

Author

Ivković-Jureković, Irena, Navratil, Marta, and Richter, Darko

Subjects
ANAFILAKSIJA, ADRENALIN, SISTEMSKA ALERGIJSKA REAKCIJA, ALERGIJA NA HRANU, ALERGIJA NA UBOD INSEKTA, ALERGIJA NA LIJEKOVE, IDIOPATSKA ANAFILAKSIJA, ANAFILAKSIJA POTAKNUTA NAPOROM
Abstract

Anafilaksija je hitno stanje koje može biti opasno po život i svaki liječnik koji skrbi o djetetu mora biti upoznat s njezinim zbrinjavanjem. Cilj ovog pregleda je podsjetiti na patofiziologiju anafilaktičke reakcije, prikazati kliničku sliku i kriterije za dijagnozu te posebne oblike anafilaksije. Brzo prepoznavanje simptoma anafilaksije i rana primjena adrenalina ključni su za ishod bolesti. Utvrđivanje uzročnog alergena obavezno je kod sve djece koja su doživjela anfilaksiju. Plan dugoročnog zbrinjavanja i praćenja ovih bolesnika uključuje upute o izbjegavanju uzročnog alergena i postupanju u slučaju recidiva simptoma te poduku o primjeni samoinjektora adrenalina.

Published
2019

35. Teška hipernatrijemijska dehidracija i cirkulacijski šok u dojenog novorođenčeta

Author

Braovac, Duje, Huljev Frković, Sanda, Šalamon Janečić, Mia, Bartoniček, Dorotea, Richter, Darko, Jotanović, Željana, Ružman, Lucija, Grizelj, Ruža, Benjak, Vesna, Dasović Buljević, Andrea, Filipović-Grčić, Boris, Ninković, Dorotea, Čupić, Ana, and Ćuk, Mario

Subjects
novorođenče, hipernatremijska dehidracija, dojenje
Abstract

Liječili smo zdravo rođeno dijete koje je u prvih deset dana života razvilo tešku hipernatremijsku dehidraciju s cirkulacijskim šokom i bilo životno ugroženo zbog nedostatnog dojenja. Ovakvo stanje obično je posljedica entero- kolitisa ili visokofebrilnih stanja u novorođenčadi na umjetnoj prehrani, ali u ovom je slučaju bila riječ o poteškoćama pri dojenju. Pomni nadzor vještine dojenja, praćenje napredovanja na TM-u u prvim danima života i edukacija trudnica prije porođaja postupci su koji mogu pridonijeti preveniranju ili pravodobnom prepoznavanju i liječenju neishranjenosti i HD-a novorođenčeta.

Published
2018

36. Influenza – Microbiology, Immunology and Epidemiology for the Physician Contemplating Immunization

Author

Richter, Darko

Subjects
Deskriptori: Gripa – epidemiologija, imunologija, prevencija, virologija, Virus gripe A – genetika, klasifikacija, imunologija, Virus gripe B - klasifikacija, imunologija, Cjepiva protiv gripe – imunologija, Cijepljenje – metode, Protuvirusni lijekovi – terapijska primjena, Virusni antigeni – genetika, imunologija, Antigenske varijacije, Virusna protutijela – imunologija, Influenza, human – epidemiology, immunology, prevention and control, virology, Influenza A virus – classsification, genetics, immunology, Influenza B virus – classsification, immunology, Influenza vaccines – immnunology, Vaccination – methods, Antiviral agents – therapeutic use, Antigens, viral – genetics, immunology, Antigenic variation, Antibodies, viral – immunology
Abstract

Sažetak. Ovaj pregledni članak o gripi (influenci) sadržava mikrobiološke, imunološke i epidemiološke podatke posljednjih 10 godina kako bi liječnicima omogućio lakše snalaženje u argumentima za izbor vlastitog cijepljenja. Ukratko se prelaze osnove mikrobiologije virusa influence, njegove patogeneze i širenja. Raspravlja se o razlozima antigenske nepodudarnosti cjepnih i divljih sojeva, uključujući i one tehnološko-proizvodne prirode. Tablično se izlaže usporedba epidemioloških i vakcinalnih parametara sezonske gripe u posljednjih 10 godina. Udio A-podtipova i B-tipova (loza) virusa influence, antigenska podudarnost i prosječna zaštitnost cjepiva izdvojeni su iz podataka Centers for Disease Control and Prevention, SAD i Hrvatskog zavoda za javno zdravstvo. Pokazalo se da od 2010. g. raste udio drugog B-virusa u sezonskoj gripi. Sve se više preporučuju četverovalentna cjepiva koja uključuju 2 A-podtipa i 2 B-loze. Neizravni podatci koji su izronili tijekom pandemije H1N1 2009. g. pokazuju da imunost, prema specifičnom podtipu influence, može biti znatno dugotrajnija od jedne sezone. Širu, ali slabije specifičnu zaštitu može pružiti imunost prema heterosuptipskim antigenima prisutnim u unutrašnjosti viriona koja se stječe prirodnom infekcijom. U cjepiva tekuće sezone, a prema predviđanju Svjetske zdravstvene organizacije, uvrštena su kao najvjerojatnije dominantna ova 4 soja: A/Michigan, A/Singapore, B/Colorado i B/Phuket. Od ovih je A/Michigan varijanta pandemijskog virusa iz 2009. g. (A/California/07/2009), a A/Singapore i B/Colorado novi su sojevi kojih nije bilo u cjepivima prethodnih sezona. B/Phuket nalazio se u trovalentnom cjepivu u sezoni 2015. – 2016. g. i u četverovalentnom cjepivu u sezonama 2016. – 2017. i 2017. – 2018. Osobe koje se nisu cijepile 2014. – 2015. g. trebale bi ove sezone posegnuti za četverovalentnim cjepivom, dok bi onima koji su se cijepili trovalentnim cjepivom 2015. – 2016. g. ili četverovalentnim cjepivom 2016. – 2017. g. i/ili 2017. – 2018. g. trovalentno cjepivo u ovoj sezoni trebalo biti dovoljno., Summary. This review of influenza microbiology, immunology and spread is garnished with epidemiological data of the past 10 seasons, in order to enable physicians an informed navigation through the arguments for their own vaccination. The reasons for antigenic mismatch, including the role of vaccine production technology are discussed. A tabulated comparison of circulating and vaccine type viruses over the past 10 seasons is layed out. The proportion of A subtypes and B types (lineages) in each season, antigenic match and average protectiveness of the influenza vaccine is extracted from the data available at The Centers of Disease Control and Prevention in the USA and the The Institute of Public Health of Croatia. A growing proportion of influenza cases are due to the second B virus. Tetravalent vaccines including 2 A subtypes and 2 B types are increasingly recommended. Indirect data from the 2009 H1N1 pandemic disclosed that immunity against a specific influenza A subtype can hold significantly longer than a single season. Broader but less specific long lasting immunity may be conferred by heterosubtypic antigens contained within the influenza virus acquired by natural infection. The current seasonal influenza is expected to be caused by 4 viruses: A/Michigan, A/Singapore, B/Colorado and B/Phuket (the latter is included only in the 4-valent vaccines). Of these, A/Michigan is a drifted 2009 pandemic virus (A/California/07/2009) and A/Singapore and B/Colorado are new strains. B/Phuket could be found in the trivalent 2015-16 vaccine, and in the tetravalent 2016-17 and 2017-18 vaccines. Individuals who did not get vaccinated since the 2014-15 season, should offer themselves a tetravalent vaccine, while those who received a trivalent vaccine for the season 2015-16 or a tetravalent one for the 2016-17 and 2017-18 seasons should be well off with a trivalent vaccine.

Published
2018

37. Alergija na hranu

Author

Ivković-Jureković, Irena, Navratil, Marta, Voskresensky-Baričić, Tamara, Kljaić-Bukvić, Blaženka, Gagro, Alenka, Richter, Darko, Čičak, Biserka, and Kelečić, Jadranka

Subjects
alergija na hranu, epigenetički čimbenici
Abstract

Svrha ovog preglednog rada je prikazati nove spoznaje u epidemiologiji, patogenezi, dijagnostici i liječenju alergije na hranu. Značajan postotak djece i odraslih ima neki oblik alergije na hranu uza sve više dokaza o porastu prevalencije širom svijeta. Mogući rizični čimbenici su, uz nasljeđe i način života, pojačana higijena, promjene u načinu ishrane, nedostatak vitamina D, pretilost, te vrijeme uvođenja dohrane. Prikazat ćemo utjecaj epigenetičkih čimbenika na razvoj alergije te ulogu parenteralne (perkutane, inhalacijske i intradermalne - ubodi kukaca) senzitizacije u patogenezi alergije na hranu. Komponentna dijagnostika na velika vrata ulazi u kliničku praksu i moćno je dijagnostičko sredstvo koje je velika pomoć u utvrđivanju težine alergije i predviđanju kliničkog tijeka. Preporuke o sprječavanju alergije na hranu i atopijskih bolesti korjenito se mijenjaju, pa tako proizlazi da odgađanje uvođenja potencijalnih alergena u prehranu pridonosi razvoju alergije, a ne podnošljivosti. Imunoterapija se pokazala korisnom u podizanju praga podnošljivosti kod IgE-posredovane alergije na hranu, ali još nije u upotrebi u rutinskoj kliničkoj praksi.

Published
2018

38. Burden of varicella in Central and Eastern Europe: findings from a systematic literature review

Author

Mészner, Zsófia, primary, Wysocki, Jacek, additional, Richter, Darko, additional, Zavadska, Dace, additional, Ivaskeviciene, Inga, additional, Usonis, Vytautas, additional, Pokorn, Marko, additional, Mangarov, Atanas, additional, Jancoriene, Ligita, additional, Man, Sorin C., additional, Kristufkova, Zuzana, additional, Jesenak, Milos, additional, Tešović, Goran, additional, Pluta, Justyna, additional, and Wolfson, Lara J., additional

Published
2019
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39. Gripa – mikrobiologija, imunologija i epidemiologija za liječnika koji razmišlja o cijepljenju

Author

Richter, Darko and Richter, Darko

Abstract

Sažetak. Ovaj pregledni članak o gripi (influenci) sadržava mikrobiološke, imunološke i epidemiološke podatke posljednjih 10 godina kako bi liječnicima omogućio lakše snalaženje u argumentima za izbor vlastitog cijepljenja. Ukratko se prelaze osnove mikrobiologije virusa influence, njegove patogeneze i širenja. Raspravlja se o razlozima antigenske nepodudarnosti cjepnih i divljih sojeva, uključujući i one tehnološko-proizvodne prirode. Tablično se izlaže usporedba epidemioloških i vakcinalnih parametara sezonske gripe u posljednjih 10 godina. Udio A-podtipova i B-tipova (loza) virusa influence, antigenska podudarnost i prosječna zaštitnost cjepiva izdvojeni su iz podataka Centers for Disease Control and Prevention, SAD i Hrvatskog zavoda za javno zdravstvo. Pokazalo se da od 2010. g. raste udio drugog B-virusa u sezonskoj gripi. Sve se više preporučuju četverovalentna cjepiva koja uključuju 2 A-podtipa i 2 B-loze. Neizravni podatci koji su izronili tijekom pandemije H1N1 2009. g. pokazuju da imunost, prema specifičnom podtipu influence, može biti znatno dugotrajnija od jedne sezone. Širu, ali slabije specifičnu zaštitu može pružiti imunost prema heterosuptipskim antigenima prisutnim u unutrašnjosti viriona koja se stječe prirodnom infekcijom. U cjepiva tekuće sezone, a prema predviđanju Svjetske zdravstvene organizacije, uvrštena su kao najvjerojatnije dominantna ova 4 soja: A/Michigan, A/Singapore, B/Colorado i B/Phuket. Od ovih je A/Michigan varijanta pandemijskog virusa iz 2009. g. (A/California/07/2009), a A/Singapore i B/Colorado novi su sojevi kojih nije bilo u cjepivima prethodnih sezona. B/Phuket nalazio se u trovalentnom cjepivu u sezoni 2015. – 2016. g. i u četverovalentnom cjepivu u sezonama 2016. – 2017. i 2017. – 2018. Osobe koje se nisu cijepile 2014. – 2015. g. trebale bi ove sezone posegnuti za četverovalentnim cjepivom, dok bi onima koji su se cijepili trovalentnim cjepivom 2015. – 2016. g. ili četverovalentnim cjepivom 2016. – 2017. g. i/ili 2017., Summary. This review of influenza microbiology, immunology and spread is garnished with epidemiological data of the past 10 seasons, in order to enable physicians an informed navigation through the arguments for their own vaccination. The reasons for antigenic mismatch, including the role of vaccine production technology are discussed. A tabulated comparison of circulating and vaccine type viruses over the past 10 seasons is layed out. The proportion of A subtypes and B types (lineages) in each season, antigenic match and average protectiveness of the influenza vaccine is extracted from the data available at The Centers of Disease Control and Prevention in the USA and the The Institute of Public Health of Croatia. A growing proportion of influenza cases are due to the second B virus. Tetravalent vaccines including 2 A subtypes and 2 B types are increasingly recommended. Indirect data from the 2009 H1N1 pandemic disclosed that immunity against a specific influenza A subtype can hold significantly longer than a single season. Broader but less specific long lasting immunity may be conferred by heterosubtypic antigens contained within the influenza virus acquired by natural infection. The current seasonal influenza is expected to be caused by 4 viruses: A/Michigan, A/Singapore, B/Colorado and B/Phuket (the latter is included only in the 4-valent vaccines). Of these, A/Michigan is a drifted 2009 pandemic virus (A/California/07/2009) and A/Singapore and B/Colorado are new strains. B/Phuket could be found in the trivalent 2015-16 vaccine, and in the tetravalent 2016-17 and 2017-18 vaccines. Individuals who did not get vaccinated since the 2014-15 season, should offer themselves a tetravalent vaccine, while those who received a trivalent vaccine for the season 2015-16 or a tetravalent one for the 2016-17 and 2017-18 seasons should be well off with a trivalent vaccine.

Published
2018

42. Long-term outcomes of 176 patients with X-linked hyper-IgM syndrome treated with or without hematopoietic cell transplantation

Author

Stoma/continentiezorg, Immuno/reuma patientenzorg, Infection & Immunity, Child Health, de la Morena, M Teresa, Leonard, David, Torgerson, Troy R, Cabral-Marques, Otavio, Slatter, Mary, Aghamohammadi, Asghar, Chandra, Sharat, Murguia-Favela, Luis, Bonilla, Francisco A, Kanariou, Maria, Damrongwatanasuk, Rongras, Kuo, Caroline Y, Dvorak, Christopher C., Meyts, Isabelle, Chen, Karin, Kobrynski, Lisa, Kapoor, Neena, Richter, Darko, DiGiovanni, Daniela, Dhalla, Fatima, Farmaki, Evangelia, Speckmann, Carsten, Español, Teresa, Shcherbina, Anna, Hanson, Imelda Celine, Litzman, Jiri, Routes, John M, Wong, Melanie, Fuleihan, Ramsay, Seneviratne, Suranjith L., Small, Trudy N, Janda, Ales, Bezrodnik, Liliana, Seger, Reinhard, Raccio, Andrea Gomez, Edgar, J David M, Chou, Janet, Abbott, Jordan K, van Montfrans, Joris, González-Granado, Luis Ignacio, Bunin, Nancy, Kutukculer, Necil, Gray, Paul, Seminario, Gisela, Pasic, Srdjan, Aquino, Victor, Wysocki, Christian, Abolhassani, Hassan, Dorsey, Morna, Cunningham-Rundles, Charlotte, Knutsen, Alan P, Sleasman, John, Costa Carvalho, Beatriz Tavares, Condino-Neto, Antonio, Grunebaum, Eyal, Chapel, Helen, Ochs, Hans D., Filipovich, Alexandra, Cowan, Mort, Gennery, Andrew, Cant, Andrew, Notarangelo, Luigi D, Roifman, Chaim M, Stoma/continentiezorg, Immuno/reuma patientenzorg, Infection & Immunity, Child Health, de la Morena, M Teresa, Leonard, David, Torgerson, Troy R, Cabral-Marques, Otavio, Slatter, Mary, Aghamohammadi, Asghar, Chandra, Sharat, Murguia-Favela, Luis, Bonilla, Francisco A, Kanariou, Maria, Damrongwatanasuk, Rongras, Kuo, Caroline Y, Dvorak, Christopher C., Meyts, Isabelle, Chen, Karin, Kobrynski, Lisa, Kapoor, Neena, Richter, Darko, DiGiovanni, Daniela, Dhalla, Fatima, Farmaki, Evangelia, Speckmann, Carsten, Español, Teresa, Shcherbina, Anna, Hanson, Imelda Celine, Litzman, Jiri, Routes, John M, Wong, Melanie, Fuleihan, Ramsay, Seneviratne, Suranjith L., Small, Trudy N, Janda, Ales, Bezrodnik, Liliana, Seger, Reinhard, Raccio, Andrea Gomez, Edgar, J David M, Chou, Janet, Abbott, Jordan K, van Montfrans, Joris, González-Granado, Luis Ignacio, Bunin, Nancy, Kutukculer, Necil, Gray, Paul, Seminario, Gisela, Pasic, Srdjan, Aquino, Victor, Wysocki, Christian, Abolhassani, Hassan, Dorsey, Morna, Cunningham-Rundles, Charlotte, Knutsen, Alan P, Sleasman, John, Costa Carvalho, Beatriz Tavares, Condino-Neto, Antonio, Grunebaum, Eyal, Chapel, Helen, Ochs, Hans D., Filipovich, Alexandra, Cowan, Mort, Gennery, Andrew, Cant, Andrew, Notarangelo, Luigi D, and Roifman, Chaim M

Published
2017

43. Medikamentozni osipi u djece

Author

Richter, Darko, Ivković-Jureković, Irena, Gagro, Alenka, Voskresensky-Baričić, Tamara, Radonić, Marija, Kelečić, Jadranka, and Čičak, Biserka

Subjects
osipna reakcija na lijek, alergija na lijek, IgE, p-i koncept, hipersenzitivni sindrom induciran lijekom
Abstract

Pojava osipa neposredno, za vrijeme ili nedugo nakon sistemne primjene lijeka smjesta se pripisuje alergijskoj reakciji, makar još nema nikakvog dokaza da su u pitanju humoralni ili stanični imunosni mehanizmi. Ovakvo preskakanje, kojim se likovni fenomen izjednačava s mehanizmom i dijagnozom, a koje ne razmatra raznolike imunopatološke mehanizme osipnih reakcija na lijekove, rezultira suviše jednostavnim i često netočnim savjetima da se lijek zauvijek isključi zbog straha od anafi laksije, ili obrnuto, da se propusti upozoriti na kasniju moguću tešku kožnu reakciju. U članku ćemo razmotriti kliničku sliku i imunosne mehanizme te na kraju dijagnostičke postupke i algoritam obrade osipnih reakcija na lijekove. Na kraju je dan osvrt na kožne reakcije na biološku terapiju.

Published
2016

46. KONTRAINDIKACIJE, NUSPOJAVE I MJERE OPREZA PRILIKOM CIJEPLJENJA DJECE.

Author

RICHTER, DARKO

Subjects
*ROTAVIRUS vaccines, *SEVERE combined immunodeficiency, *WHOOPING cough vaccines, *INTESTINAL intussusception, *VACCINES, *CONTRAINDICATIONS
Abstract

Contraindication is one or a set of known clinical or laboratory circumstances which render certain therapeutic or diagnostic interventions undesirable due to a predictable health risk. Contraindications can be absolute, relative, permanent or temporary. There are but 3 permanent absolute contraindications to vaccination: anaphylaxis to any vaccine, encephalopathy occurring within 7 days of receiving pertussis containing vaccine without other explanatory factors, and severe combined immunodeficiency, or intussusceptions or uncorrected intestinal malformation for the rotavirus vaccination. Preganancy is an absolute, albeit temporary contraindication to any live vaccine. All vaccines, inactivated, live or live and inactivated may be given simultaneously, at one visit. Otherwise it is mandatory to delay the next immunization with the same inactivated vaccine, or any live vaccine following a live vaccine, for at least 4 weeks, due to immune interference rendering the second (early) vaccine ineffective. There is no overall testig for vaccines in advance of immunization. [ABSTRACT FROM AUTHOR]

Published
2019

47. HIPER-IgE U PRIMARNIM IMUNODEFICIJENCIJAMA - DIJAGNOSTIČKI I TERAPIJSKI IZAZOV.

Author

RICHTER, DARKO

Subjects
*ALLERGIES, *LYMPHOPROLIFERATIVE disorders, *AUTOIMMUNE diseases, *WISKOTT-Aldrich syndrome, *PARASITIC diseases, *ATOPY, *AUTOIMMUNE hemolytic anemia
Abstract

Hyper-IgE is defined as IgE concentration >1000 kU/L. Although this is primarily present in atopic and parasitic diseases, nevertheless it is a significant hint toward a number of primary immunodeficiency diseases. The "red baby syndrome" accompanied by hyper IgE is virtually specific for Omenn and Comel-Netherton syndromes. The appearance of eczema within the first 6 weeks of life accompanied by hyper-IgE may point to the hyper-IgE syndrome (Job syndrome), DOCK8 immunodeficiency and Wiskott- Aldrich syndrome. About 50% of isolated IgA deficiency cases with IgE up to 3800 kU/L are discovered during regular work-up for standard allergic disease. Primary immunodeficiency with hyper-IgE is frequently complicated by autoimmune disease (usually autoimmune hemolytic anemia and other autoimmune citopenias, vasculitis, IgE nephropathy). In some primary immunodeficiency diseases such as Job syndrome and in a subset of autoimmune lymphoproliferative syndrome patients with eosinophilia and hyper- IgE there is no increase in atopic disease nor increase in specific IgE sensitization. In others, like DOCK8 deficiency and Wiskott- Aldrich syndrome atopy, allergic disease and risk of anaphylaxis are increased. [ABSTRACT FROM AUTHOR]

Published
2019

48. ANAFILAKSIJA.

Author

NAVRATIL, MARTA, IVKOVIĆ-JUREKOVIĆ, IRENA, and RICHTER, DARKO

Subjects
ANAPHYLAXIS, ALLERGIES, HISTORY of children, FOOD allergy, CHILD care, EMERGENCY physicians
Abstract

Copyright of Paediatria Croatica, Supplement is the property of Paediatria Croatica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2019

49. Antivakcinalni pokret

Author

Richter, Darko, Čičak, Biserka, Gagro, Alenka, Ivković-Jureković, Irena, Kelečić, Jadranka, Radonić, Marija, and Voskresensky-Baričić, Tamara

Subjects
antivakcinalni pokret, obvezni program cijepljenja, dopunsko osiguranje, odštetni fond
Abstract

Fenomen odbijanja cijepljenja nazivamo antivakcinacijskim stavom, a širenje antivakcinacijskim pokretom ili kampanjom. Pritom oni koji odbijaju cijepljenje zapravo ne razumiju motive onih koji ih od cijepljenja odgovaraju, ali se brane da to rade zato jer sumnjaju ili ne razumiju argumente onih koji ih na cijepljenje nagovaraju. Cijepljenje se odbija iz međusobno logički i stručno suprotstavljenih argumenata. Riječ je o stručnoj shizmi u kojoj nema oblikovane ni transparente osnove, već se homogenizacija protiv cijepljenja zasniva na mnogo različitih i često suprotstavljenih kritika/negacija cjepiva, cijepljenja, struke, establišmenta, zakonske regulative, biznisa i farmaceutske industrije. Antivakcinacijski pokret došao je i do Hrvatske. U nascentnoj situaciji trebalo bi zaštititi cjepitelje od materijalne ili kaznene odgovornosti za još i sad obveznu preventivnu mjeru, te hitnim i razgovijetnim mjerama edukacije i javne promidžbe osigurati održanje adekvatnog obuhvata za postojeća cjepiva iz obveznog programa. Uz to treba osnovati odštetni fond za nuspojave cijepljenja. Umjesto sankcije za odbijanje cijepljenja treba uvesti obvezu posebnog dopunskog osiguranja za rizik svake bolesti za koju roditelj odbije cijepiti svoje dijete. U današnjoj situaciji prikladni odgovor bio bi i uvođenje novog cjepiva u program univerzalnog cijepljenja (npr. cijepljenje protiv rotavirusa ili pneumokokne bolesti).

Published
2014

50. Genetic basis of primary immunodeficiencies in Croatian patients

Author

Merkler, Ana, Richter, Darko, Kelecic, Jadranka, Ljubic, Hana, Caban, Domagoj, and Sertic, Jadranka

Subjects
primary immunodeficiencies, BTK gene, SBDS gene, DNA sequencing
Abstract

Introduction: Primary immunodeficiency diseases (PID) are a heterogenic group of rare inherited conditions that occur in individuals born with malfunctioned immune system. Objective: The objective was to confirm clinical diagnosis at molecular genetic level in patients with PID and to define carrier status by analyzing DNA samples of individuals with PID in family history. Methods: We analyzed 24 samples of genomic DNA: 11 samples of patients with suspicion on one of the PID and 13 samples of their family members. For identification of mutations in the coding region of analyzed genes, we used the sequencing method on Applied Biosystems 3130xl Genetic analyzer and BigDye® Terminator v3.1 Cycle Sequencing Kit. Results: In 2 patients with X-linked agammaglobulinemia, mutations in the BTK gene were found. In the first patient, mutation occurred de novo in mother‘s egg cells and, in the other, mutation was inherited from the mother. 3 patients with XLA suspicion didn‘t have mutation in the BTK gene. In 4 patients with cyclic and severe congenital neutropenia, 4 mutations occurred de novo in mother‘s egg cells were found. In 2 patients with suspicion on Shwachmann-Diamond syndrome mutation in SBDS gene inherited from the parents were found. Conclusions: Understanding the genetic basis of PID is the final step in confirmation of the PID diagnosis as it provides comprehension of disease mechanisms at the molecular level and correlation between genotype and phenotype of PID.

Published
2013

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