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1. Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency

Author

Taghizade, Nigar, Babayeva, Royala, Kara, Altan, Karakus, Ibrahim Serhat, Catak, Mehmet Cihangir, Bulutoglu, Alper, Haskologlu, Zehra Sule, Akay Haci, Idil, Tunakan Dalgic, Ceyda, Karabiber, Esra, Bilgic Eltan, Sevgi, Yorgun Altunbas, Melek, Sefer, Asena Pinar, Sezer, Ahmet, Kokcu Karadag, Sefika Ilknur, Arik, Elif, Karali, Zuhal, Ozhan Kont, Aylin, Tuzer, Can, Karaman, Sait, Mersin, Selver Seda, Kasap, Nurhan, Celik, Enes, Kocacik Uygun, Dilara Fatma, Aydemir, Sezin, Kiykim, Ayca, Aydogmus, Cigdem, Ozek Yucel, Esra, Celmeli, Fatih, Karatay, Emrah, Bozkurtlar, Emine, Demir, Semra, Metin, Ayse, Karaca, Neslihan Edeer, Kutukculer, Necil, Aksu, Guzide, Guner, Sukru Nail, Keles, Sevgi, Reisli, Ismail, Kendir Demirkol, Yasemin, Arikoglu, Tugba, Gulez, Nesrin, Genel, Ferah, Kilic, Sara Sebnem, Aytekin, Caner, Keskin, Ozlem, Yildiran, Alisan, Ozcan, Dilek, Altintas, Derya Ufuk, Ardeniz, Fatma Omur, Dogu, Esin Figen, Ikinciogullari, Kamile Aydan, Karakoc-Aydiner, Elif, Ozen, Ahmet, and Baris, Safa

Published
2023
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2. Activated phosphoinositide 3-kinase δ syndrome: Update from the ESID Registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity

Author

Seidel, Markus G., Seppänen, Mikko R.J., Gennery, Andrew, Kanariou, Maria G., Tantou, Sofia, Grigoriadou, Sofia, Cericola, Gabriella, Hanitsch, Leif G., Scheibenbogen, Carmen, Hlaváčková, Eva O., Krivan, Gergely, McGuire, Frances K., Leahy, Timothy Ronan, Edgar, John David M., Bakhtiar, Shahrzad, Bader, Peter, Rohner, Geraldine Blanchard, Haerynck, Filomeen, Claes, Karlien, Lehmberg, Kai, Müller, Ingo, Farmand, Susan, Fasshauer, Maria, Graf, Dagmar, Neves, Joao Farela, Kostyuchenko, Larysa, Gonzalez-Granado, Luis Ignacio, Jeseňák, Miloš, Carrabba, Maria, Fabio, Giovanna, Pignata, Claudio, Giardino, Giuliana, Karadağ, Ilknur Kökçü, Yıldıran, Alişan, Hancioglu, Gonca, Králíčková, Pavlína, Steinmann, Sandra, Pietrucha, Barbara Maria, Gernert, Michael, Soomann, Maarja, Witte, Torsten, Markocsy, Adam, Wolska-Kusnierz, Beata, Randrianomenjanahary, Philippe, Rouger, Jérémie, Kostaridou, Stavroula, Zabara, Dariia V., Rodina, Yulia A., Shvets, Oksana A., Maccari, Maria Elena, Wolkewitz, Martin, Schwab, Charlotte, Lorenzini, Tiziana, Leiding, Jennifer W., Aladjdi, Nathalie, Abolhassani, Hassan, Abou-Chahla, Wadih, Aiuti, Alessandro, Azarnoush, Saba, Baris, Safa, Barlogis, Vincent, Barzaghi, Federica, Baumann, Ulrich, Bloomfield, Marketa, Bohynikova, Nadezda, Bodet, Damien, Boutboul, David, Bucciol, Giorgia, Buckland, Matthew S., Burns, Siobhan O., Cancrini, Caterina, Cathébras, Pascal, Cavazzana, Marina, Cheminant, Morgane, Chinello, Matteo, Ciznar, Peter, Coulter, Tanya I., D’Aveni, Maud, Ekwall, Olov, Eric, Zelimir, Eren, Efrem, Fasth, Anders, Frange, Pierre, Fournier, Benjamin, Garcia-Prat, Marina, Gardembas, Martine, Geier, Christoph, Ghosh, Sujal, Goda, Vera, Hammarström, Lennart, Hauck, Fabian, Heeg, Maximilian, Heropolitanska-Pliszka, Edyta, Hilfanova, Anna, Jolles, Stephen, Karakoc-Aydiner, Elif, Kindle, Gerhard R., Kiykim, Ayca, Klemann, Christian, Koletsi, Patra, Koltan, Sylwia, Kondratenko, Irina, Körholz, Julia, Krüger, Renate, Jeziorski, Eric, Levy, Romain, Le Guenno, Guillaume, Lefevre, Guillaume, Lougaris, Vassilios, Marzollo, Antonio, Mahlaoui, Nizar, Malphettes, Marion, Meinhardt, Andrea, Merlin, Etienne, Meyts, Isabelle, Milota, Tomas, Moreira, Fernando, Moshous, Despina, Mukhina, Anna, Neth, Olaf, Neubert, Jennifer, Neven, Benedicte, Nieters, Alexandra, Nove-Josserand, Raphaele, Oksenhendler, Eric, Ozen, Ahmet, Olbrich, Peter, Perlat, Antoinette, Pac, Malgorzata, Schmid, Jana Pachlopnik, Pacillo, Lucia, Parra-Martinez, Alba, Paschenko, Olga, Pellier, Isabelle, Sefer, Asena Pinar, Plebani, Alessandro, Plantaz, Dominique, Prader, Seraina, Raffray, Loic, Ritterbusch, Henrike, Riviere, Jacques G., Rivalta, Beatrice, Rusch, Stephan, Sakovich, Inga, Savic, Sinisa, Scheible, Raphael, Schleinitz, Nicolas, Schuetz, Catharina, Schulz, Ansgar, Sediva, Anna, Semeraro, Michaela, Sharapova, Svetlana O., Shcherbina, Anna, Slatter, Mary A., Sogkas, Georgios, Soler-Palacin, Pere, Speckmann, Carsten, Stephan, Jean-Louis, Suarez, Felipe, Tommasini, Alberto, Trück, Johannes, Uhlmann, Annette, van Aerde, Koen J., van Montfrans, Joris, von Bernuth, Horst, Warnatz, Klaus, Williams, Tony, Worth, Austen J.J., Ip, Winnie, Picard, Capucine, Catherinot, Emilie, Nademi, Zohreh, Grimbacher, Bodo, Forbes Satter, Lisa R., Kracker, Sven, Chandra, Anita, Condliffe, Alison M., and Ehl, Stephan

Published
2023
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3. PFAPA Syndrome: Evaluation of Clinical Findings, Immunological Alterations, and Treatment Approaches.

Author

Kökçü Karadağ, Şefika İlknur, Çepni, Esra Bekar, Gündüz, Zeynep Gizem, and Yıldıran, Alişan

Subjects
PHYSICAL diagnosis, PHARYNGITIS, TONSILLECTOMY, FEVER, CANKER sores, TREATMENT effectiveness, IMMUNE system, PREDNISOLONE, COLCHICINE, ROUTINE diagnostic tests, LYMPHADENITIS, SYMPTOMS
Abstract

Copyright of Journal of Current Pediatrics / Guncel Pediatri is the property of Galenos Yayinevi Tic. LTD. STI 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
2024
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4. Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency

Author

Kiykim, Ayca, Ogulur, Ismail, Dursun, Esra, Charbonnier, Louis Marie, Nain, Ercan, Cekic, Sukru, Dogruel, Dilek, Karaca, Neslihan Edeer, Cogurlu, Mujde Tuba, Bilir, Ozlem Arman, Cansever, Murat, Kapakli, Hasan, Baser, Dilek, Kasap, Nurhan, Kutlug, Seyhan, Altintas, Derya Ufuk, Al-Shaibi, Ahmad, Agrebi, Nourhen, Kara, Manolya, Guven, Ayla, Somer, Ayper, Aydogmus, Cigdem, Ayaz, Nuray Aktay, Metin, Ayse, Aydogan, Metin, Uncuoglu, Aysen, Patiroglu, Turkan, Yildiran, Alisan, Guner, Sukru Nail, Keles, Sevgi, Reisli, Ismail, Aksu, Guzide, Kutukculer, Necil, Kilic, Sara S., Yilmaz, Mustafa, Karakoc-Aydiner, Elif, Lo, Bernice, Ozen, Ahmet, Chatila, Talal A., and Baris, Safa

Published
2019
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6. BCG vaccination in patients with severe combined immunodeficiency: Complications, risks, and vaccination policies

Author

Marciano, Beatriz E., Huang, Chiung-Yu, Joshi, Gyan, Rezaei, Nima, Carvalho, Beatriz Costa, Allwood, Zoe, Ikinciogullari, Aydan, Reda, Shereen M., Gennery, Andrew, Thon, Vojtech, Espinosa-Rosales, Francisco, Al-Herz, Waleed, Porras, Oscar, Shcherbina, Anna, Szaflarska, Anna, Kiliç, Şebnem, Franco, Jose L., Gómez Raccio, Andrea C., Roxo, Persio, Jr., Esteves, Isabel, Galal, Nermeen, Grumach, Anete Sevciovic, Al-Tamemi, Salem, Yildiran, Alisan, Orellana, Julio C., Yamada, Masafumi, Morio, Tomohiro, Liberatore, Diana, Ohtsuka, Yoshitoshi, Lau, Yu-Lung, Nishikomori, Ryuta, Torres-Lozano, Carlos, Mazzucchelli, Juliana T.L., Vilela, Maria M.S., Tavares, Fabiola S., Cunha, Luciana, Pinto, Jorge A., Espinosa-Padilla, Sara E., Hernandez-Nieto, Leticia, Elfeky, Reem A., Ariga, Tadashi, Toshio, Heike, Dogu, Figen, Cipe, Funda, Formankova, Renata, Nuñez-Nuñez, M. Enriqueta, Bezrodnik, Liliana, Marques, Jose Gonçalo, Pereira, María I., Listello, Viviana, Slatter, Mary A., Nademi, Zohreh, Kowalczyk, Danuta, Fleisher, Thomas A., Davies, Graham, Neven, Bénédicte, and Rosenzweig, Sergio D.

Published
2014
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8. Çocuklarda COVID-19 ile İlişkili Multisistem Enflamatuvar Sendromunda Tanı Esnasında ve Tedavi Sonrasında Lenfosit Alt Gruplarının Değerlendirildiği Vaka Serisi.

Author

Kökçü Karadağ, Şefika İlknur, Erdeniz, Emine Hafize, Özkan, Esra, and Yıldıran, Alişan

Abstract

Copyright of Journal of Pediatric Infection / Çocuk Enfeksiyon Dergisi is the property of Journal of Pediatric Infection / Cocuk Enfeksiyon Dergisi 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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9. Case Series Evaluating Lymphocyte Subgroups at Diagnosis and After Treatment in Multisystem Inflammatory Syndrome Associated with COVID-19 in Children.

Author

Kökçü Karadağ, Şefika İlknur, Erdeniz, Emine Hafize, Özkan, Esra, and Yıldıran, Alişan

Abstract

Copyright of Journal of Pediatric Infection / Çocuk Enfeksiyon Dergisi is the property of Journal of Pediatric Infection / Cocuk Enfeksiyon Dergisi 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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10. Tekrarlayan Tüberküloz ile Kronik Granülomatöz Tanısı Konulan Adölesan.

Author

Kökcü Karadağ, Şefika İlknur, Kutluğ, Şeyhan, Uzun, Oğuz, Köker, Mustafa Yavuz, and Yıldıran, Alişan

Abstract

Copyright of Journal of Pediatric Infection / Çocuk Enfeksiyon Dergisi is the property of Journal of Pediatric Infection / Cocuk Enfeksiyon Dergisi 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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12. The Efficacy of Long-Term Voriconazole Prophylaxis on Seven Patients with Chronic Granulomatous Disease and Review of the Literature.

Author

Kutluğ, Şeyhan, Kökçü Karadağ, Şefika İlknur, Şensoy, S. Gülnar, Birinci, Asuman, Köker, Mustafa Yavuz, and Yıldıran, Alişan

Abstract

Copyright of Journal of Pediatric Infection / Çocuk Enfeksiyon Dergisi is the property of Journal of Pediatric Infection / Cocuk Enfeksiyon Dergisi 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
2021
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15. Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey.

Author

Yıldıran, Alişan, Çeliksoy, Mehmet Halil, Borte, Stephan, Güner, Şükrü Nail, Elli, Murat, Fışgın, Tunç, Özyürek, Emel, Sancak, Recep, and Oğur, Gönül

Subjects
*HEMATOPOIETIC stem cell transplantation, *IMMUNOLOGICAL deficiency syndromes, *HEMOPHAGOCYTIC lymphohistiocytosis, *SEVERE combined immunodeficiency, *TREATMENT effectiveness, *RETROSPECTIVE studies, *WISKOTT-Aldrich syndrome, *CHEDIAK-Higashi syndrome, *IMMUNOCOMPROMISED patients
Abstract

Hematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. We retrospectively reviewed pediatric cases of patients diagnosed with primary immunodeficiencies and scheduled for hematopoietic stem cell transplantation. We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received hematopoietic stem cell transplantation. The patient diagnoses included severe combined immunodeficiency (n=11), Chediak-Higashi syndrome (n=2), leukocyte adhesion deficiency (n=2), MHC class 2 deficiency (n=2), chronic granulomatous syndrome (n=2), hemophagocytic lymphohistiocytosis (n=1), Wiskott-Aldrich syndrome (n=1), and Omenn syndrome (n=1). Of the 22 patients, 7 received human leukocyte antigen-matched related hematopoietic stem cell transplantation, 12 received haploidentical hematopoietic stem cell transplantation, and 2 received matched unrelated hematopoietic stem cell transplantation. The results showed that 5 patients had graft failure. Fourteen patients survived, yielding an overall survival rate of 67%. Screening newborn infants for primary immunodeficiency diseases may result in timely administration of hematopoietic stem cell transplantation. [ABSTRACT FROM AUTHOR]

Published
2017
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16. An evaluation of vitamin D levels in children with seasonal allergic rhinitis during pollen season.

Author

Kutluğ, Seyhan, Kılıç, Mehtap, Bilgici, Birşen, Paksu, Şule, Yıldıran, Alişan, and Sancak, Recep

Subjects
VITAMIN D, ALLERGIC rhinitis, POLLEN, FAT-soluble vitamins, ALLERGENS
Abstract

Background Serum vitamin D levels have not been studied in children with seasonal allergic rhinitis ( SAR). The aim of this study was to evaluate the vitamin D levels of children with SAR and to compare them to levels in healthy children during pollen season. Methods This study was conducted in 100 children with SAR and 100 healthy controls. Clinical and laboratory evaluations and vitamin D analyses of all the participants were performed between the months of April and July. Pollen sensitization was detected in the patient group using a skin prick test. 25( OH)D3 levels were compared between the patient and control groups. Associations among the patient 25( OH)D3 levels and their demographic, clinical, and laboratory characteristics were analyzed. Results Overall, 72% of the patients were male, the median age was 12.35 years (range: 6-17.8 years), and the median body mass index value was 19.15 (range: 13.6-27.8). There were no differences between the patients and healthy controls in terms of gender, age, or body mass index. The mean levels of 25( OH)D3 (20.78±6) in patients were higher than those of the controls (17.92±4). In the patient group, no associations were found among 25( OH)D3 levels, demographic characteristics, atopy test results, atopy history, severity of rhinitis, and the total four symptoms score (all P>.05). Conclusions During pollen season, children with SAR may have higher vitamin D levels than healthy controls. The presence of asthma and/or atopic dermatitis in addition to SAR did not change this result. [ABSTRACT FROM AUTHOR]

Published
2017
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17. Pulmonary hypoplasia presenting with recurrent wheezing in an infant.

Author

Çeliksoy, Mehmet Halil, Tander, Burak, Aşılıoğlu, Nazik, Barış, Yakup Sancar, and Yıldıran, Alişan

Subjects
PULMONARY hypoplasia, NEWBORN infants, AUTOPSY, COMPUTED tomography, MAGNETIC resonance, NEUROMUSCULAR diseases
Abstract

Pulmonary hypoplasia is characterized by decrease in the number and size of pulmonary airways, alveoli and vessels. In autopsy, pulmonary hypoplasia is a major cause of death in neonates and infants. The disease is usually diagnosed in childhood period. Although it mimics lung parenchymal disease and other vascular abnormalities radiologically, it is easily recognized with computed tomography angiography and magnetic resonance angiography examinations. In 50% of patients, concomitant cardiovascular, neuromuscular, gastrointestinal tract, and urogenital anomalies are also available. There are two types of pulmonary hypoplasia: primary and secondary. Primary unilateral pulmonary hypoplasia may be asymptomatic and the tendency for bronchopulmonary infections is often increased in children. In this case report, a 22-month-old male patient characterized by recurrent infections and recurrent wheezes in infantile period, whose episodes of wheezing regressed after the pulmonectomy, was presented. [ABSTRACT FROM AUTHOR]

Published
2015
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20. c.761C>T Mutation Linked Hyper IgM Syndrome Presenting with Hypertransaminasemia and Arthritis.

Author

Celiksoy, Mehmet Halil, Borte, Stephan, İkincioğulları, Aydan, Bilgici, Meltem Ceyhan, Karagöz, Filiz, Kalaycı, Ayhan Gazi, and Yıldıran, Alişan

Subjects
ARTHRITIS diagnosis, BILE duct diseases, IMMUNOLOGICAL deficiency syndrome complications, LIVER disease diagnosis, ABDOMINAL radiography, CHOLANGIOGRAPHY, BIOPSY, BLOOD testing, EDEMA, ENZYMES, IMMUNOGLOBULINS, LIVER, MAGNETIC resonance imaging, GENETIC mutation, PHYSICAL diagnosis, KNEE pain, CHILDREN, DIAGNOSIS
Abstract

The article presents a case study of an eight-year-old male patient with two-year history of knee swelling and pain. The patient was found negative for surface antigen of the hepatitis B virus (HBsAg), anti-HBs, anti-HCV (hepatitis-C), and Cytomegalovirus by polymerase chain reaction. Hyperimmunoglobulin M syndrome (HIGM). The patient was diagnosed with Hyperimmunoglobulin M syndrome (HIGM) syndrome.

Published
2014
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