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1. Immunization status in chronic obstructive pulmonary disease: A multicenter study from Turkey

Author

Ozlu, Tevfik, Bulbul, Yilmaz, Aydin, Derya, Tatar, Dursun, Kuyucu, Tulin, Erboy, Fatma, Koseoglu, Handan Inonu, Anar, Ceyda, Sunnetcioglu, Aysel, Gulhan, Pinar Yildiz, Sahin, Unal, Ekici, Aydanur, Duru, Serap, Ulasli, Sevinc Sarinc, Kurtipek, Ercan, Gunay, Sibel, Okutan, O., Yildiz, B. P., Cetinkaya, P. D., Arslan, S., Cakmak, G., Cirak, A. K., Sarioglu, N., Kocak, N. D., Akturk, U. A., Demir, M., Kilic, T., Dalli, A., Hezer, H., Altintas, N., Acat, M., Dagli, C. E., Kargi, A., Yakar, F., Kirkil, G., Baccioglu, A., Gedik, C., Intepe, Y. S., Karadeniz, G., Onyilmaz, T., Saylan, B., Baslilar, S., Sariman, N., Ozkurt, S., Arinc, S., Kanbay, A., Yazar, E. E., Yildirim, Z., Kadioglu, E. E., Gul, S., Sengul, A., Berk, S., Dikis, O. S., Kurt, O. K., Arslan, Y., Erol, S., Korkmaz, C., Balaban, A., Erbay, Toru U., Sogukpinar, O., Uzaslan, E. K., Babaoglu, E., Bahadir, A., Baris, S. A., Ugurlu, A. O., Ilgazli, A. H., Fidan, F., Kararmaz, E., Guzel, A., Alzafer, S., Cortuk, M., Hocanli, I., Ortakoylu, M. G., Erginel, M. S., Yaman, N., Erbaycu, A. E., Demir, A., Duman, D., Tanriverdi, H., Yavuz, M. Y., Sertogullarindan, B., Ozyurt, S., Bulcun, E., Yuce, G. D., Sariaydin, M., Ayten, O., Bayraktaroglu, M., Tekgul, S., Erel, F., Senyigit, A., Kaya, S. B., Ayik, S., Yazici, O., Akgedik, A., Yasar, Z. A., Hayat, E., Kalpaklioglu, F., Sever, F., Sarac, P., Ugurlu, E., Kasapoglu, U. S., Gunluoglu, G., Demirci, N. Y., Bora, M., Talay, F., Ozkara, B., Yilmaz, M. U., Yavsan, D. M., Cetinoglu, E. D., Balcan, M. B., Ciftci, T., Havan, A., Gok, A., Nizam, M., Investigators, R.I.M.P.A.C.T. Study, Acibadem University Dspace, Maltepe Üniversitesi, BAİBÜ, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Talay, Fahrettin, OMÜ, Tıp Fakültesi, Kırıkkale Üniversitesi, Zonguldak Bülent Ecevit Üniversitesi, [Ozlu, Tevfik -- Bulbul, Yilmaz] Karadeniz Tech Univ, Sch Med, Dept Chest Dis, TR-61080 Trabzon, Turkey -- [Aydin, Derya] Pulm Dis Hosp, Chest Dis Clin, Balikesir, Turkey -- [Tatar, Dursun -- Anar, Ceyda] Dr Suat Seren Pulm Dis & Thorac Surg Educ & Res H, Dept Pulm Dis, Izmir, Turkey -- [Kuyucu, Tulin] Sureyyapasa Pulm Dis & Thorac Surg Educ & Res Hos, Dept Pulm Dis, Istanbul, Turkey -- [Erboy, Fatma] Bulent Ecevit Univ, Sch Med, Dept Chest Dis, Zonguldak, Turkey -- [Koseoglu, Handan Inonu] Gaziosmanpasa Univ, Sch Med, Dept Chest Dis, Tokat, Turkey -- [Sunnetcioglu, Aysel] Yuzuncu Yil Univ, Sch Med, Dept Chest Dis, Van, Turkey -- [Gulhan, Pinar Yildiz] Tosya State Hosp, Chest Dis Clin, Kastamonu, Turkey -- [Sahin, Unal] Recep Tayyip Erdogan Univ, Sch Med, Dept Chest Dis, Rize, Turkey -- [Ekici, Aydanur] Kirikkale Univ, Sch Med, Dept Chest Dis, Kirikkale, Turkey -- [Duru, Serap] Diskapi Yildirim Beyazid Educ & Res Hosp, Dept Pulm Dis, Ankara, Turkey -- [Ulasli, Sevinc Sarinc] Hacettepe Univ, Sch Med, Dept Chest Dis, Ankara, Turkey -- [Kurtipek, Ercan] Konya Educ & Res Hosp, Dept Pulm Dis, Konya, Turkey -- [Gunay, Sibel] Afyon State Hosp, Chest Dis Clin, Afyon, Turkey -- [Okutan, O. -- Ayten, O.] Haydarpasa Hosp, Gulhane Mil Med Acad, Dept Chest Dis, Istanbul, Turkey -- [Yildiz, B. P. -- Yazar, E. E. -- Gul, S. -- Bahadir, A. -- Ortakoylu, M. G. -- Bayraktaroglu, M. -- Gunluoglu, G. -- Nizam, M.] Pulm Dis & Thorac Surg Educ & Res Hosp Yedikule, Istanbul, Turkey -- [Cirak, A. K. -- Karadeniz, G. -- Erol, S. -- Erbaycu, A. E. -- Demir, A. -- Yavuz, M. Y. -- Tekgul, S. -- Sever, F. -- Yilmaz, M. U.] Dr Suat Seren, Izmir, Turkey -- [Kocak, N. D. -- Akturk, U. A. -- Arinc, S. -- Sogukpinar, O. -- Duman, D. -- Kasapoglu, U. S.] Sureyyapasa, Istanbul, Turkey -- [Cetinkaya, P. D.] Educ & Res Hosp Numune, Adana, Turkey -- [Dikis, O. S. -- Bora, M.] Sevket Yilmaz, Bursa, Turkey -- [Balaban, A.] Evliya Celebi, Kutahya, Turkey -- [Yuce, G. D.] Diskapi Yildirim Beyazid, Ankara, Turkey -- [Arslan, S. -- Berk, S.] Cumhuriyet Univ, Sch Med, Dept Chest Dis, Sivas, Turkey -- [Sarioglu, N. -- Erel, F.] Balikesir Univ, Balikesir, Turkey -- [Demir, M. -- Senyigit, A.] Dicle Univ, Diyarbakir, Turkey -- [Kilic, T. -- Kaya, S. B.] Inonu Univ, Malatya, Turkey -- [Dalli, A. -- Ayik, S.] Katip Celebi Univ, Izmir, Turkey -- [Altintas, N.] Namik Kemal Univ, Tekirdag, Turkey -- [Acat, M. -- Cortuk, M. -- Yazici, O.] Karabuk Univ, Karabuk, Turkey -- [Dagli, C. E. -- Akgedik, A.] Ordu Univ, Ordu, Turkey -- [Kargi, A. -- Kurt, O. K. -- Yasar, Z. A. -- Talay, F.] Abant Izzet Baysal Univ, Bolu, Turkey -- [Yakar, F. -- Hayat, E.] Bezmialem Vakif Univ, Istanbul, Turkey -- [Kirkil, G.] Firat Univ, Elazig, Turkey -- [Baccioglu, A. -- Bulcun, E. -- Kalpaklioglu, F.] Kirikkale Univ, Kirikkale, Turkey -- [Gedik, C. -- Kanbay, A.] Medeniyet Univ, Istanbul, Turkey -- [Intepe, Y. S.] Bozok Univ, Yozgat, Turkey -- [Sariman, N.] Maltepe Univ, Istanbul, Turkey -- [Ozkurt, S. -- Ugurlu, E.] Pamukkale Univ, Denizli, Turkey -- [Yildirim, Z. -- Demirci, N. Y.] Gazi Univ, Ankara, Turkey -- [Korkmaz, C. -- Yavsan, D. M.] Necmettin Erbakan Univ, Konya, Turkey -- [Erbay, Toru U.] Dumlupinar Univ, Kutahya, Turkey -- [Uzaslan, E. K. -- Cetinoglu, E. D.] Uludag Univ, Bursa, Turkey -- [Baris, S. A. -- Ilgazli, A. H. -- Ciftci, T.] Kocaeli Univ, Kocaeli, Turkey -- [Fidan, F. -- Havan, A.] Fatih Univ, Istanbul, Turkey -- [Guzel, A. -- Gok, A.] Ondokuz Mayis Univ, Samsun, Turkey -- [Alzafer, S.] Acibadem Univ, Bakirkoy Hosp, Istanbul, Turkey -- [Erginel, M. S.] Osmangazi Univ, Eskisehir, Turkey -- [Tanriverdi, H.] Bulent Ecevit Univ, Zonguldak, Turkey -- [Sertogullarindan, B.] Yuzuncu Yil Univ, Van, Turkey -- [Ozyurt, S.] Recep Tayyip Erdogan Univ, Rize, Turkey -- [Sariaydin, M.] Afyon Kocatepe Univ, Afyon, Turkey -- [Onyilmaz, T. -- Sarac, P.] State Hosp Mardin, Mardin, Turkey -- [Arslan, Y. -- Ozkara, B.] Etimesgut Mil Hosp, Ankara, Turkey -- [Ugurlu, A. O. -- Balcan, M. B.] Baskent Univ, Istanbul Hosp, Istanbul, Turkey -- [Yaman, N.] Pulm Dis Hosp, Balikesir, Turkey, intepe, yavuz selim -- 0000-0002-5697-5291, Sertogullarindan, Bunyamin -- 0000-0002-1478-1990, and Bulbul, Yilmaz -- 0000-0002-8488-3650

Subjects
Acute Exacerbations, Pulmonary and Respiratory Medicine, lcsh:Diseases of the circulatory (Cardiovascular) system, medicine.medical_specialty, Coverage, pneumococcal vaccine, Exacerbation, disease exacerbation, influenza vaccine, Influenza vaccine, Copd Exacerbation, Self-Reported Influenza, Chronic Obstructive Pulmonary Disease Exacerbation, Latin-American Cities, Logistic regression, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Prevalence, medicine, chronic obstructive pulmonary disease exacerbation, Adults, Mass index, 030212 general & internal medicine, Determinants, lcsh:RC705-779, COPD, business.industry, Chronic obstructive pulmonary disease, chronic obstructive pulmonary, Retrospective cohort study, lcsh:Diseases of the respiratory system, Frequency, medicine.disease, Vaccination, 030228 respiratory system, Pneumococcal vaccine, lcsh:RC666-701, Pneumococcal Vaccination, Original Article, Surgery, Cardiology and Cardiovascular Medicine, business
Abstract

WOS: 000455908800007, PubMed ID: 30745939, OBJECTIVE: The purpose of this study is to detect the prevalence and the factors associated with influenza and pneumococcal vaccination and outcomes of vaccination during 2013-2014 season in patients with chronic obstructive pulmonary disease (COPD) in Turkey. METHODS: This was a multicenter retrospective cohort study performed in 53 different centers in Turkey. RESULTS: During the study period, 4968 patients were included. COPD was staged as GOLD 1-2-3-4 in 9.0%, 42.8%, 35.0%, and 13.2% of the patients, respectively. Influenza vaccination rate in the previous year was 37.9%; and pneumococcus vaccination rate, at least once during in a life time, was 13.3%. Patients with older age, higher level of education, more severe COPD, and comorbidities, ex-smokers, and patients residing in urban areas had higher rates of influenza vaccination. Multivariate logistic regression analysis showed that advanced age, higher education levels, presence of comorbidities, higher COPD stages, and exacerbation rates were associated with both influenza and pneumococcal vaccination. The number of annual physician/outpatient visits and hospitalizations due to COPD exacerbation was 2.73 +/- 2.85 and 0.92 +/- 1.58 per year, respectively. Patients with older age, lower education levels, more severe COPD, comorbid diseases, and lower body mass index and patients who are male and are residing in rural areas and vaccinated for influenza had significantly higher rates of COPD exacerbation. CONCLUSIONS: The rates of influenza and pneumococcal vaccination in COPD patients were quite low, and the number of annual physician/outpatient visits and hospitalizations due to COPD exacerbation was high in Turkey. Advanced age, higher education levels, comorbidities, and higher COPD stages were associated with both influenza and pneumococcal vaccination.

Published
2019

2. Does gestational age affect ultrasonographic findings of the hip in preterm newborns? A sonographic study of the early neonatal period

Author

Yilmaz, M. U., Erel, F., Senyigit, A., Sever, F., Sarac, P., Ugurlu, E., Kasapoglu, U. S., Gunluoglu, G., Yavsan, D. M., Cetinoglu, E. D., Balcan, M. B., Ciftci, T., Havan, A., Gok, A., Nizam, M., Ozkara, B., ÖZLÜ, TEVFİK, Aydin, Derya, Tatar, Dursun, Kuyucu, Tulin, ERBOY, FATMA, İNÖNÜ KÖSEOĞLU, HANDAN, Anar, Ceyda, Sünnetçioğlu, Aysel, Gulhan, Pinar Yildiz, BÜLBÜL, YILMAZ, Okutan, O., Yildiz, B. P., Cetinkaya, P. D., Arslan, S., Cakmak, G., Cirak, A. K., Yakar, F., Kirkil, G., Baccioglu, A., Gedik, C., Sahin, Unal, EKİCİ, AYDANUR, Duru, Serap, Ulasli, Sevinc Sarinc, Kurtipek, Ercan, Gunay, Sibel, Dalli, A., Sarioglu, N., Kocak, N. D., Akturk, U. A., Demir, M., Kilic, T., Hezer, H., Altintas, N., Acat, M., Dagli, C. E., Kargi, A., Karadeniz, G., Intepe, Y. S., Onyilmaz, T., Saylan, B., Baslilar, S., Sariman, N., Ozkurt, S., Arinc, S., Kanbay, A., Yazar, E. E., Yildirim, Z., Kadioglu, E. E., Gul, S., Sengul, A., Berk, S., Dikis, O. S., Bahadir, A., Baris, S. A., Ugurlu, A. O., Ilgazli, A. H., Ortakoylu, M. G., Erginel, M. S., Yaman, N., Arslan, Y., Kurt, O. K., Erol, S., Korkmaz, C., Balaban, A., Erbay, Toru U., Sogukpinar, O., Uzaslan, E. K., Babaoglu, E., Fidan, F., Kararmaz, E., Guzel, A., Alzafer, S., Cortuk, M., Hocanli, I, Tanriverdi, H., Yavuz, M. Y., Sertogullarindan, B., Ozyurt, S., Erbaycu, A. E., Demir, A., Duman, D., Kaya, S. B., Ayik, S., Yazici, O., Akgedik, A., Yasar, Z. A., Hayat, E., Kalpaklioglu, F., Demirci, N. Y., Bora, M., Talay, F., Bulcun, E., Yuce, G. D., Sariaydin, M., Ayten, O., Bayraktaroglu, M., Tekgul, S., and BURSAL DURAMAZ, BURCU

Subjects
musculoskeletal diseases, Male, medicine.medical_specialty, Birth weight, Gestational Age, 03 medical and health sciences, 0302 clinical medicine, Neonatal Screening, Medicine, Humans, Orthopedics and Sports Medicine, Hip Dislocation, Congenital, Retrospective Studies, Ultrasonography, 030222 orthopedics, Developmental dysplasia, business.industry, Obstetrics, Infant, Newborn, Gestational age, Retrospective cohort study, Early neonatal period, Duramaz A., BURSAL DURAMAZ B., Bilgili M. G. , -Does gestational age affect ultrasonographic findings of the hip in preterm newborns? A sonographic study of the early neonatal period-, JOURNAL OF PEDIATRIC ORTHOPAEDICS-PART B, cilt.28, ss.107-110, 2019, Pediatrics, Perinatology and Child Health, Gestation, Female, business, 030217 neurology & neurosurgery, Infant, Premature
Abstract

There are only a few studies in the literature investigating the effects of gestational age on developmental dysplasia of the hip. The aim of this study was to investigate the effects of gestational age on hip ultrasound findings in the early neonatal period in preterm newborns born between 30th and 36th weeks of gestational age. Between January 2008 and December 2013, a total of 788 hips of 394 premature newborns with a gestational age of up to 36th weeks who underwent hip ultrasonography in the first week of their life were retrospectively examined. The distribution of roof angles and hip types in terms of sexes was compared between groups. Birth weight, birth height, a, and beta angles were analyzed in terms of the gestational age. The mean gestational age was 33.07 weeks (SD 2.09; between 30th and 36th). Six hundred and seven hips were classified as type I, 154 as type IIa, 21 as type IIc, and 6 as type III. In the 30th week, type IIc hips in females and type III hips in males were statistically significantly higher (P=0.001). In the 34th week, type IIc hips in males were statistically significantly higher than the females (P=0.013). In the 35th week, type IIa hips in females hips were statistically significantly higher than the males (P=0.008). Among all preterm infants, type IIc hips were more common in the 30th, 31st, 32nd, and 34th weeks, whereas type III hips were statistically significantly more common in the 30th week (P=0.0001). The 30th, 31st, 32nd, and 34th weeks of age are gestational ages that should be considered in terms of dysplastic and subluxed hips in premature newborns. Copyright (C) 2018 Wolters Kluwer Health, Inc. All rights reserved.

Published
2018

3. Long-term follow-up of IPEX syndrome patients after different therapeutic strategies: An international multicenter retrospective study

Author

Barzaghi, F., Hernandez, L.C.A., Neven, B., Ricci, S., Kucuk, Z.Y., Bleesing, J.J., Nademi, Z., Slatter, M.A., Ulloa, E.R., Shcherbina, A., Roppelt, A., Worth, A., Silva, J., Aiuti, A., Murguia-Favela, L., Speckmann, C., Carneiro-Sampaio, M., Fernandes, J.F., Baris, S., Ozen, A., Karakoc-Aydiner, E., Kiykim, A., Schulz, A., Steinmann, S., Notarangelo, L.D., Gambineri, E., Lionetti, P., Shearer, W.T., Forbes, L.R., Martinez, C., Moshous, D., Blanche, S., Fisher, A., Ruemmele, F.M., Tissandier, C., Ouachee-Chardin, M., Rieux-Laucat, F., Cavazzana, M., Qasim, W., Lucarelli, B., Albert, M.H., Kobayashi, I., Alonso, L., Heredia, C.D. de, Kanegane, H., Lawitschka, A., Seo, J.J., Gonzalez-Vicent, M., Diaz, M.A., Goyal, R.K., Sauer, M.G., Yesilipek, A., Kim, M., Yilmaz-Demirdag, Y., Bhatia, M., Khlevner, J., Padilla, E.J.R., Martino, S., Montin, D., Neth, O., Molinos-Quintana, A., Valverde-Fernandez, J., Broides, A., Pinsk, V., Ballauf, A., Haerynck, F., Bordon, V., Dhooge, C., Garcia-Lloret, M.L., Bredius, R.G., Kawak, K., Haddad, E., Seidel, M.G., Duckers, G., Pai, S.Y., Dvorak, C.C., Ehl, S., Locatelli, F., Goldman, F., Gennery, A.R., Cowan, M.J., Roncarolo, M.G., Bacchetta, R., PIDTC, IEWP, European Soc Blood Marrow, Barzaghi, Federica, Hernandez, Laura Cristina Amaya, Neven, Benedicte, Ricci, Silvia, Kucuk, Zeynep Yesim, Bleesing, Jack J., Nademi, Zohreh, Slatter, Mary Anne, Ulloa, Erlinda Rose, Shcherbina, Anna, Roppelt, Anna, Worth, Austen, Silva, Juliana, Aiuti, Alessandro, Murguia-Favela, Luis, Speckmann, Carsten, Carneiro-Sampaio, Magda, Fernandes, Juliana Folloni, Baris, Safa, Ozen, Ahmet, Karakoc-Aydiner, Elif, Kiykim, Ayca, Schulz, Ansgar, Steinmann, Sandra, Notarangelo, Lucia Dora, Gambineri, Eleonora, Lionetti, Paolo, Shearer, William Thomas, Forbes, Lisa R., Martinez, Caridad, Moshous, Despina, Blanche, Stephane, Fisher, Alain, Ruemmele, Frank M., Tissandier, Come, Ouachee-Chardin, Marie, Rieux-Laucat, Frederic, Cavazzana, Marina, Qasim, Waseem, Lucarelli, Barbarella, Albert, Michael H., Kobayashi, Ichiro, Alonso, Laura, De Heredia, Cristina Diaz, Kanegane, Hirokazu, Lawitschka, Anita, Seo, Jong Jin, Gonzalez-Vicent, Marta, Diaz, Miguel Angel, Goyal, Rakesh Kumar, Sauer, Martin G., Yesilipek, Akif, Kim, Minsoo, Yilmaz-Demirdag, Yesim, Bhatia, Monica, Khlevner, Julie, Padilla, Erick J. Richmond, Martino, Silvana, Montin, Davide, Neth, Olaf, Molinos-Quintana, Agueda, Valverde-Fernandez, Justo, Broides, Arnon, Pinsk, Vered, Ballauf, Antje, Haerynck, Filomeen, Bordon, Victoria, Dhooge, Catharina, Garcia-Lloret, Maria Laura, Bredius, Robbert G., Kalwak, Krzysztof, Haddad, Elie, Seidel, Markus Gerhard, Duckers, Gregor, Pai, Sung-Yun, Dvorak, Christopher C., Ehl, Stephan, Locatelli, Franco, Goldman, Frederick, Gennery, Andrew Richard, Cowan, Mort J., Roncarolo, Maria-Grazia, Bacchetta, Rosa, Amaya Hernandez, Laura Cristina, Murguia-Favela, Lui, Shearer, William Thoma, Rieux-Laucat, Frédéric, Diaz De Heredia, Cristina, Richmond Padilla, Erick J., and Kałwak, Krzysztof

Subjects
0301 basic medicine, Male, Allergy, medicine.medical_treatment, Medizin, Disease, Hematopoietic stem cell transplantation, SIROLIMUS, Regenerative Medicine, primary immune deficiency, Medicine and Health Sciences, IPEX, Immunology and Allergy, 2.1 Biological and endogenous factors, Enteropathy, Aetiology, POLYENDOCRINOPATHY, Child, Pediatric, CÉLULAS-TRONCO, immunosuppression, Hematopoietic Stem Cell Transplantation, Genetic Diseases, X-Linked, Immunosuppression, Forkhead Transcription Factors, X-LINKED SYNDROME, Allografts, Survival Rate, surgical procedures, operative, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Immune System Diseases, Genetic Diseases, Child, Preschool, hematopoietic stem cell transplantation, Female, hematopoietic stem, neonatal diabetes, FOXP3, Primary Immune Deficiency, Treg cells, enteropathy, genetic autoimmunity, rapamycin, Type 1, Diarrhea, Adult, medicine.medical_specialty, Adolescent, Immunology, Neonatal onset, Article, Disease-Free Survival, 03 medical and health sciences, Neonatal diabete, Clinical Research, Internal medicine, IMMUNODYSREGULATION, medicine, Primary Immune Deficiency Treatment Consortium (PIDTC) and the Inborn Errors Working Party (IEWP) of the European Society for Blood and Marrow Transplantation, Diabetes Mellitus, Genetics, Humans, REGULATORY T-CELLS, cell transplantation, Preschool, Retrospective Studies, Immunosuppression Therapy, Transplantation, IMMUNE DYSREGULATION, business.industry, MUTATIONS, Infant, Retrospective cohort study, STEM-CELL TRANSPLANTATION, IPEX syndrome, X-Linked, medicine.disease, Stem Cell Research, BONE-MARROW-TRANSPLANTATION, Treg cell, FOXP3 MUTATIONS, Diabetes Mellitus, Type 1, 030104 developmental biology, ENGRAFTMENT, Mutation, business, Follow-Up Studies
Abstract

Background Immunodysregulation polyendocrinopathy enteropathy x-linked(IPEX) syndromeis a monogenic autoimmune disease caused by FOXP3 mutations. Because it is a rare disease, the natural history and response to treatments, including allogeneic hematopoietic stem cell transplantation (HSCT) and immunosuppression (IS), have not been thoroughly examined. Objective This analysis sought to evaluate disease onset, progression, and long-term outcome of the 2 main treatments in long-term IPEX survivors. Methods Clinical histories of 96 patients with a genetically proven IPEX syndrome were collected from 38 institutions worldwide and retrospectively analyzed. To investigate possible factors suitable to predict the outcome, an organ involvement (OI) scoring system was developed. Results We confirm neonatal onset with enteropathy, type 1 diabetes, and eczema. In addition, we found less common manifestations in delayed onset patients or during disease evolution. There is no correlation between the site of mutation and the disease course or outcome, and the same genotype can present with variable phenotypes. HSCT patients (n = 58) had a median follow-up of 2.7 years (range, 1 week-15 years). Patients receiving chronic IS (n = 34) had a median follow-up of 4 years (range, 2 months-25 years). The overall survival after HSCT was 73.2% (95% CI, 59.4-83.0) and after IS was 65.1% (95% CI, 62.8-95.8). The pretreatment OI score was the only significant predictor of overall survival after transplant (P = .035) but not under IS. Conclusions Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long-term disease-free survival. When performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life, independent of age, donor source, or conditioning regimen., GRAPHICAL ABSTRACT

Published
2018

10. Efficacy of Intravenous Immunoglobulin Treatment in Children with Common Variable Immunodeficiency

Author

Baris, S., Hulya Ercan, Hasret Cagan, H., Ozen, A., Karakoc-Aydiner, E., Ozdemir, C., Bahceciler, N. N., Baris, S., Ercan, H., Hasret Cagan, H., Ozen, A., Karakoc-Aydiner, E., Ozdemir, C., Bahceciler, N.N., and Yeditepe Üniversitesi

Subjects
Children, Intravenous immunoglobulin, Common variable immunodeficiency
Abstract

Background: Children with common variable immunodeficiency (CVID) have increased susceptibility to infections. Objective: We evaluated the role of intravenous immunoglobulin (IVIG) replacement therapy on the clinical outcome of patients with CVID. Methods: We studied children diagnosed with CVID and treated with IVIG (500 mg/kg every 3 weeks). Results: The study population comprised 29 children with CVID (mean [SD] age, 11.8 [6.1] years) with at least 1 year of follow-up before IVIG replacement therapy. Mean follow-up duration was 5.6 (3.5) years (range, 15 months-14 years). During therapy, median serum IgG levels increased from 410 to 900 mg/dL. The mean number of respiratory infections per patient per year decreased significantly from 10.2 to 2.5. The annual number and length of hospital stays decreased significantly from 1.36 to 0.21 and 16.35 to 6.33 days per patient, respectively. The mean annual number of antibiotics used decreased significantly from 8.27 to 2.50 per patient. Twelve patients had developed bronchiectasis before initiation of IVIG; 3 patients were cured of this condition. Age at diagnosis, diagnostic delay, number of respiratory tract infections, and number of antibiotics were found to be significantly higher in patients with bronchiectasis, as was lower B-cell percentage. However, gastrointestinal involvement due to noninfectious causes did not improve significantly after IVIG replacement therapy. Conclusion: CVID patients treated with IVIG (500 mg/kg every 3 weeks) had satisfactory serum IgG levels, fewer respiratory tract infections, fewer and shorter hospital stays, and reduced antibiotic usage. However, no effect on gastrointestinal involvement was observed. Early IVIG replacement therapy is important in preventing bronchiectasis. © 2011 Esmon Publicidad.

17. Atopic dermatitis: insight into the mechanism

Author

Baris, S., Akdeniz, F., Ng, Hatirnaz O., Karakoc-Aydiner, E., Ozen, A., Saricoban-Ercan, H., Ayca Kiykim, Cagan, H., Ozek, E., Cokugras, H., Cengizlier, R., Ozbek, U., Bahceciler, N., Yucelten, D., Demirel, Yanikkaya G., and Barlan, I.

23. The Middle East and North Africa Diagnosis and Management Guidelines for Inborn Errors of Immunity

Author

Safa Baris, Hassan Abolhassani, Michel J. Massaad, Maryam Al-Nesf, Zahra Chavoshzadeh, Sevgi Keles, Ismail Reisli, Azzeddine Tahiat, Hiba Mohammad Shendi, Dalia Abd Elaziz, Brahim Belaid, Fatima Al Dhaheri, Sule Haskologlu, Figen Dogu, Imen Ben-Mustapha, Ali Sobh, Nermeen Galal, Safa Meshaal, Rabab Elhawary, Aisha El-marsafy, Fayhan J. Alroqi, Bandar Al-Saud, Mona Al-Ahmad, Tariq Al Farsi, Nashat AL Sukaiti, Salem Al-Tamemi, Cybel Mehawej, Ghassan Dbaibo, Gehad ElGhazali, Sara Sebnem Kilic, Ferah Genel, Ayca Kiykim, Ugur Musabak, Hasibe Artac, Sukru Nail Guner, Rachida Boukari, Reda Djidjik, Nadia Kechout, Deniz Cagdas, Zeinab Awad El-Sayed, Elif Karakoc-Aydiner, Raed Alzyoud, Mohamed Ridha Barbouche, Mehdi Adeli, Rima Hanna Wakim, Shereen M. Reda, Aydan Ikinciogullari, Ahmet Ozen, Aziz Bousfiha, Hamoud Al-Mousa, Nima Rezaei, Waleed Al-Herz, Raif S. Geha, and Baris S., Abolhassani H., Massaad M. J. , Al-Nesf M., Chavoshzadeh Z., Keles S., Reisli I., Tahiat A., Shendi H. M. , Elaziz D. A. , et al.

Subjects
Treatment, MENA region, Primary immunodeficiency, Diagnosis, Immunology and Allergy, Inborn errors of immunity
Abstract

Human inborn errors of immunity (IEI) are a group of 485 distinct genetic disorders affecting children and adults. Signs and symptoms of IEI are heterogeneous, and accurate diagnosis can be challenging and depends on the available human expertise and laboratory resources. The Middle East and North Africa (MENA) region has an increased prevalence of IEI because of the high rate of consanguinity with a predominance of autosomal recessive disorders. This area also exhibits more severe disease phenotypes compared with other regions, probably due to the delay in diagnosis. The MENA-IEI registry network has designed protocols and guidelines for the diagnosis and treatment of IEI, taking into consideration the variable regional expertise and resources. These guidelines are primarily meant to improve the care of patients within the region, but can also be followed in other regions with similar patient populations.

Published
2023
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24. Outcome of hypogammaglobulinemia in children: Immunoglobulin levels as predictors

Author

Elif Karakoc-Aydiner, Ahmet Ozen, Safa Baris, Cevdet Ozdemir, Nerin N. Bahceciler, Isil Barlan, Ozen, A., Baris, S., Karakoc-Aydiner, E., Ozdemir, C., Bahceciler, N.N., Barlan, I.B., and Yeditepe Üniversitesi

Subjects
Male, IgM, Adolescent, Allergy, Unclassified Hypogammaglobulinemia, Immunology, Immunoglobulins, THI, Immunoglobulin E, Transient Hypogammaglobulinemia, Hypogammaglobulinemia, Agammaglobulinemia, Predictive Value of Tests, Immunopathology, medicine, Humans, Immunology and Allergy, Child, IgM deficiency, Retrospective Studies, Outcome, B cell, biology, Predictors, business.industry, Common variable immunodeficiency, CVID, Immunoglobulin level, Prognosis, medicine.disease, Child, Preschool, Predictive value of tests, Antibody response, biology.protein, Female, Antibody, business, Biomarkers
Abstract

We evaluated 131 children (M = 88, F = 43) with hypogammaglobulinemia. Data was analyzed mainly for delineating predictor factors for outcome. The distance from the lower limit of normal (-2SD) for any single measurement of immunoglobulins (Ig) was calculated and transformed into Ig scores. Mean age and duration of follow-up were 5.06 ± 4.05 and 3.7 ± 3.03. years, respectively. The diagnoses were: 22 CVID, 16 IgA deficiency, 33 transient hypogammaglobulinemia of childhood (THC), 3 selective IgM deficiency and 57 unclassified hypogammaglobulinemia (UCH). Low IgA scores (

Published
2010
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25. Predictors of atopic dermatitis phenotypes and severity: roles of serum immunoglobulins and filaggrin gene mutation R501X

Author

T. Ispir, Safa Baris, Mehmet Reha Cengizlier, H. Ercan, Deniz Kirac, S. Oztezcan, Ahmet Ozen, Ercan, H., Ispir, T., Kirac, D., Baris, S., Ozen, A., Oztezcan, S., Cengizlier, M.R., and Yeditepe Üniversitesi

Subjects
Pulmonary and Respiratory Medicine, Male, Genotype, Turkey, Immunology, DNA Mutational Analysis, Immunoglobulins, Filaggrin R501X mutation, Gene mutation, Filaggrin Proteins, medicine.disease_cause, Dermatitis, Atopic, Atopy, Intermediate Filament Proteins, medicine, Immunology and Allergy, Humans, Child, Children, Immunoglobulin levels, Gene, Genotyping, Atopic dermatitis, Asthma, Mutation, biology, business.industry, Infant, General Medicine, medicine.disease, Prognosis, Phenotype, Child, Preschool, biology.protein, Disease Progression, Female, Gene-Environment Interaction, Antibody, business
Abstract

Background Atopic dermatitis (AD), the most common chronic relapsing skin condition of infancy and childhood, is a complex multifactorial disease, which arises from the interaction between strong genetic and environmental factors. Objective To investigate the roles of several factors on the severity of AD including FLG R501X gene mutation, serum immunoglobulin (Ig) levels, atopy and accompanying allergic disorders. Method Children were genotyped for the mutation in FLG R501X gene. Serum levels of major Ig isotypes, atopy and accompanying allergic disorders were assessed. Results Study group consisted of 49 patients (M: 26, F: 23) with a mean age of 4.9 ± 3.6 years and control group consisted of 50 children (M: 30, F: 20) with a mean age of 3.8 ± 2.8 years. Genotyping of R501X mutation revealed risk alleles in none of the children in study group or control group. IgG z-scores were significantly higher in patients with AD compared to controls (−0.97 ± 1.13 vs 1.48 ± 1.02, p = 0.026). There was a positive trend in IgG z-scores and a negative trend in IgA z-scores across the severity of AD. History of recurrent infections was significantly associated with asthma and/or AR (47.8% in patients with asthma/AR vs 3.8% in those without). Children with low IgG or IgA levels presented at an earlier age with lower rates of atopy and mild type AD. Conclusion In a sample of Turkish children, FLG R501X genotyping revealed no risk alleles in variable severities of AD or healthy controls. Our data suggest that IgG and IgA levels might have a role in phenotypic features of AD in terms of severity and atopic sensitisation.

Published
2011

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