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1. Familial Mediterranean fever: current perspectives

Author

Sönmez HE, Batu ED, and Özen S

Subjects
familial Meditarranean fever, recommendation, child, Pathology, RB1-214, Therapeutics. Pharmacology, RM1-950
Abstract

Hafize Emine Sönmez,* Ezgi Deniz Batu,* Seza ÖzenDepartment of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey *These authors contributed equally to this workAbstract: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease, and it is characterized by recurrent attacks of fever and polyserositis. The disease is associated with mutations in the MEFV gene encoding pyrin, which causes exaggerated inflammatory response through uncontrolled production of interleukin 1. The major long-term complication of FMF is amyloidosis. Colchicine remains the principle therapy, and the aim of treatment is to prevent acute attacks and the consequences of chronic inflammation. With the evolution in the concepts about the etiopathogenesis and genetics of the disease, we have understood that FMF is more complicated than an ordinary autosomal recessive monogenic disorder. Recently, recommendation sets have been generated for interpretation of genetic testing and genetic diagnosis of FMF. Here, we have reviewed the current perspectives in FMF in light of recent recommendations.Keywords: familial Meditarranean fever, recommendation, child

Published
2016

4. Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

Author

Goilav, B., Goss, N., Oni, L., Marks, S.D., Smith, E.M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C.M., Kamphuis, S., Lambert, L., Levy, D.M., Lewandowski, L., Maxwell, N., Morand, E., Özen, S., Pain, C.E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., and Beresford, M.W.

Published
2024
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9. Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

Author

Smith, E.M.D., primary, Aggarwal, A., additional, Ainsworth, J., additional, Al-Abadi, E., additional, Avcin, T., additional, Bortey, L., additional, Burnham, J., additional, Ciurtin, C., additional, Hedrich, C.M., additional, Kamphuis, S., additional, Lambert, L., additional, Levy, D.M., additional, Lewandowski, L., additional, Maxwell, N., additional, Morand, E., additional, Özen, S., additional, Pain, C.E., additional, Ravelli, A., additional, Saad Magalhaes, C., additional, Pilkington, C., additional, Schonenberg-Meinema, D., additional, Scott, C., additional, Tullus, K., additional, Beresford, M.W., additional, Goilav, B., additional, Goss, N., additional, Oni, L., additional, and Marks, S.D., additional

Published
2024
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10. Defining remission in childhood-onset lupus:PReS-endorsed consensus definitions by an international task force

Author

Smith, E. M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C. M., Kamphuis, S., Lambert, L., Levy, D. M., Lewandowski, L., Maxwell, N., Morand, E., Özen, S., Pain, C. E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., Beresford, M. W., Goilav, B., Goss, N., Oni, L., Marks, S. D., Smith, E. M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C. M., Kamphuis, S., Lambert, L., Levy, D. M., Lewandowski, L., Maxwell, N., Morand, E., Özen, S., Pain, C. E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., Beresford, M. W., Goilav, B., Goss, N., Oni, L., and Marks, S. D.

Abstract

Objective: To derive childhood-onset SLE (cSLE) specific remission definitions for future treat-to-target (T2T) trials, observational studies, and clinical practice. Methods: The cSLE International T2T Task Force conducted Delphi surveys exploring paediatric perspectives on adult-onset SLE remission targets. A modified nominal group technique was used to discuss, refine, and agree on the cSLE remission target criteria.Results: The Task Force proposed two definitions of remission: ‘cSLE clinical remission on steroids (cCR)’ and ‘cSLE clinical remission off steroids (cCR-0)’. The common criteria are: (1) Clinical-SLEDAI-2 K = 0; (2) PGA score < 0.5 (0–3 scale); (4) stable antimalarials, immunosuppressive, and biologic therapy (changes due to side-effects, adherence, weight, or when building up to target dose allowed). Criterion (3) in cCR is the prednisolone dose ≤0.1 mg/kg/day (maximum 5 mg/day), whereas in cCR-0 it is zero. Conclusions: cSLE definitions of remission have been proposed, maintaining sufficient alignment with the adult-SLE definition to facilitate life-course research.

Published
2024

11. Genotype–phenotype correlation, gonadal malignancy risk, gender preference, and testosterone/dihydrotestosterone ratio in steroid 5-alpha-reductase type 2 deficiency: a multicenter study from Turkey

Author

Abacı, A., Çatlı, G., Kırbıyık, Ö., Şahin, N. M., Abalı, Z. Y., Ünal, E., Şıklar, Z., Mengen, E., Özen, S., Güran, T., Kara, C., Yıldız, M., Eren, E., Nalbantoğlu, Ö., Güven, A., Çayır, A., Akbaş, E. D., Kor, Y., Çürek, Y., Aycan, Z., Baş, F., Darcan, Ş., and Berberoğlu, M.

Published
2019
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12. POS0278 EFFICACY AND SAFETY OF SECUKINUMAB IN JUVENILE IDIOPATHIC ARTHRITIS: INTERIM RESULTS FROM THE EXTENSION OF THE JUNIPERA TRIAL

Author

Ruperto, N., primary, Foeldvari, I., additional, Alexeeva, E., additional, Aktay Ayaz, N., additional, Schulert, G., additional, Özen, S., additional, Popov, A., additional, Ramanan, A., additional, Scott, C., additional, Sozeri, B., additional, Zholobova, E., additional, Chakraborty, S., additional, Zhu, X., additional, Martin, R., additional, Whelan, S., additional, Kaur, S., additional, Pricop, L., additional, Lovell, D. J., additional, Martini, A., additional, and Brunner, H., additional

Published
2023
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13. POS0740 VALIDATION OF THE PEDIATRIC BEHÇET DISEASE CRITERIA (PEDBD): A REAL LIFE CONSENSUS-BASED APPROACH

Author

Matucci-Cerinic, C., primary, Palluy, H., additional, Al-Mayouf, S., additional, Brogan, P., additional, Cantarini, L., additional, Gül, A., additional, Kasapcopur, O., additional, Kuemmerle-Deschner, J. B., additional, Özen, S., additional, Saadoun, D., additional, Shahram, F., additional, Mosci, E., additional, Ruperto, N., additional, Gattorno, M., additional, and Koné-Paut, I., additional

Published
2023
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15. OP0167 EULAR POINTS TO CONSIDER FOR PATIENT EDUCATION, PAIN MANAGEMENT AND PHYSICAL ACTIVITY ADAPTED TO THE SELF-MANAGEMENT OF JUVENILE-ONSET RHEUMATIC AND MUSCULOSKELETAL DISEASES DURING TRANSITIONAL CARE. THE EULAR MOVE-UP PROJECT

Author

Prieto-Moreno, R., primary, Courel-Ibañez, J., additional, Briones-Vozmediano, E., additional, Angevare, S., additional, Anton, J., additional, Ariza-Vega, P., additional, Bini, I., additional, Clemente, D., additional, Correia, M., additional, Costello, W., additional, Diederik, D. C., additional, Domjan, A., additional, Leon Mateos, L., additional, Marques, A., additional, Minden, K., additional, Mourão, A. F., additional, Najm, A., additional, Özen, S., additional, Pimentel, G., additional, Saleem, Z., additional, Vetrovsky, T., additional, Wulffraat, N., additional, Zacarias, A., additional, Prior, Y., additional, Carmona, L., additional, and Estevez-Lopez, F., additional

Published
2023
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16. AB1697 COMPARISON OF DISEASE ACTIVITY, INFLAMMATORY BIOMARKER, FUNCTIONALITY, PARTICIPATION AND BIOPSYCHOSOCIAL STATUS OF INDIVIDUALS WITH JIA ACCORDING TO THE PRESENCE OF UPPER EXTREMITY INVOLVEMENT

Author

Buran, S., primary, Karaca, N. B., additional, Tüfekçi, M. O., additional, Atabey Gerlegiz, E. N., additional, Aliyev, E., additional, Bayindir, Y., additional, Yildiz Kabak, V., additional, Atasavun Uysal, S., additional, Bilginer, Y., additional, Ünal, E., additional, and Özen, S., additional

Published
2023
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20. POS1372 THE 2021 EULAR AND ACR POINTS TO CONSIDER FOR DIAGNOSIS, MANAGEMENT AND MONITORING OF THE IL 1 MEDIATED AUTOINFLAMMATORY DISEASES: CAPS, TRAPS, MKD, AND DIRA

Author

Romano, M., primary, Arici, Z. S., additional, Piskin, D., additional, Alehashemi, S., additional, Aletaha, D., additional, Barron, K., additional, Benseler, S., additional, Berard, R., additional, Broderick, L., additional, Dedeoglu, F., additional, Diebold, M., additional, Durrant, K., additional, Ferguson, P., additional, Foell, D., additional, Hausmann, J., additional, Jones, O., additional, Kastner, D., additional, Lachmann, H. J., additional, Laxer, R., additional, Rivera, D., additional, Ruperto, N., additional, Simon, A., additional, Twilt, M., additional, Frenkel, J., additional, Hoffman, H., additional, De Jesus, A., additional, Kuemmerle-Deschner, J., additional, Özen, S., additional, Gattorno, M., additional, Goldbach-Mansky, R., additional, and Demirkaya, E., additional

Published
2022
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21. AB0548 A COMPARATIVE STUDY BETWEEN THE NEUROPSYCHIATRIC INVOLVEMENTS IN ADULT-ONSET AND CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS

Author

Yardimci, G. K., primary, Sener, S., additional, Ayan, G., additional, Taghiyeva, A., additional, Acar Ozen, N. P., additional, Batu, E. D., additional, Kiliç, L., additional, Öncel, H. İ., additional, Tuncer, A., additional, Göçmen, R., additional, Karli Oğuz, K., additional, Bilginer, Y., additional, Kalyoncu, U., additional, Özen, S., additional, and Bilgen, Ş. A., additional

Published
2022
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22. Defining colchicine resistance/intolerance in patients with familial Mediterranean fever: a modified-Delphi consensus approach

Author

Özen, S. Sag, E. Ben-Chetrit, E. Gattorno, M. Gül, A. Hashkes, P.J. Kone-Paut, I. Lachmann, H.J. Tsitsami, E. Twilt, M. Benedetti, F. Kuemmerle-Deschner, J.B.

Subjects
education
Abstract

OBJECTIVES: Colchicine is the main treatment for FMF. Although a number of individuals with FMF are intolerant/resistant to colchicine, there is no standard definition of colchicine resistance/intolerance. We developed a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF that may serve as a guide for clinicians and health authorities. METHODS: A set of statements was identified using a modified-Delphi consensus-based approach. The process involved development of an initial colchicine resistance/intolerance-related questionnaire derived from a systematic literature review. The questionnaire, which was completed by an international panel of 11 adult and paediatric rheumatologists with expertise in FMF, was analysed anonymously. The results informed draft consensus statements that were discussed by a round-table expert panel, using a nominal group technique to agree on the selection and wording of the final statements. RESULTS: Consensus among the panel was achieved on eight core statements defining colchicine resistance/intolerance in patients with FMF. A definition of resistance was agreed upon that included recurrent clinical attacks (average one or more attacks per month over a 3-month period) or persistent laboratory inflammation in between attacks. Other core statements recognize the importance of assessing treatment adherence, and the impact of active disease and intolerance to colchicine on quality of life. CONCLUSION: Based on expert opinion, a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF were identified to help guide clinicians and health authorities in the management of patients with FMF. © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.

Published
2021

23. Cat allergy in Turkish children

Author

Kaya, M. S., Taşkırdı, I., Özen, S., Akay, Hacı, I., Çelebi, Çelik F., Soyoz, O., Seyhanli, D., Can, Demet, and Tıp Fakültesi

Subjects
Allergy, Immunology
Abstract

Can, Demet (Balikesir Author), Background: In recent years, pets keeping has become widespread in Turkey. In parallel, the prevalence of allergy to cat has increased. It is important to distinguish cat allergy from sensitization to cat al-lergen. Studies investigating cat allergy in children are rare. In our study, it was planned to investigate the clinical features of cat allergy and sensitization to cat allergen in children living in big cities. Method: Skin prick test (SPT) included cat allergen was applied to 3499 (16.8%) patients among 20717 patients who applied with any al-lergic complaints to two center of Pediatric Allergy Outpatient Clinics in İzmir and Balıkesir between 01.01.2019– 31.12.2020. Accepted as sensitization to cat allergen if any average wheal diameter was ≥3 mm bigger than the negative control. 140 (4%) children with sensitization to cat allergen were included in this study. Cat allergy was defined as the occurrence of reproducible symptoms or signs initiated by ex-posure to a cat. The patients were divided into groups first as mono-sensitized and polysensitized, and then as those with cat allergy and sensitization to cat allergen. Demographic data, allergic histories and laboratory results of the patients in each group were recorded. SPT threshold values of those with cat allergies were calculated, a ques-tionnaire was applied for the precautions they took for cat allergy. Results: 32% of the patients had sensitization to cat allergen, 68% had cat allergy and the most common symptom was sneezing. 57% of children with cat allergy were male and the mean age was 12.1 years. 17% of children with sensitization to cat allergen and 16% of children with cat allergy were monosensitized. It was found that the threshold value for SPT was 6.3 mm in monoosensitized patients (13.5%) with cat allergy, and the SPT was above 6.5 mm in polysen-sitized patients (86.5%). In the polysensitized group, the most com-mon aroallergens detected sensitivity other than cat allergen were grass and olive tree pollen, respectively. 50% of families to cat al-lergy in their children gave up their ownership of cats and sent them to a relative. 88% of children with cat allergy said that they would like to be allergen- specific immunotherapy (AIT). Conclusion: Cat allergy is more common in boys and the most com-mon complaint is sneezing. In patients with suspicious history, the SPT threshold value may be helpful to differentiate sensitization to cat allergen from cat allergy. Half of those with cat allergies do not give up their cats and prefer AIT.

Published
2021

25. Aromatase inhibitors: a useful additional therapeutic option for slowing down advanced bone age in boys with growth hormone deficiency

Author

Akın Kağızmanlı, G., Özalp Kızılay, D., Besci, Ö., Yüksek Acinikli, K., Özen, S., Demir, K., Gökşen Şimşek, R. D., Böber, E., Darcan, Ş., and Abacı, A.

Abstract

Introduction: Aromatase inhibitors (AIs) have been used to slow down estrogen-dependent skeletal maturation in pubertal boys with short stature. In the literature, few data evaluate the effectiveness and safety of AIs in boys with growth hormone deficiency (GHD). This study aimed to evaluate the auxologic effects and short-term laboratory profiles of combined AI and rhGH therapy for 1 year in adolescent males with GHD. Subjects and methods: Male subjects between the ages of 10 and 16 with GHD from two different centers were included in the study. Patients were divided into two groups: (i) those who only used recombinant human growth hormone (rhGH) therapy (Group I; G-I) and (ii) those who also used AI therapy (anastrozole or letrozole) along with rhGH (Group II; G-II). Results: Forty-one patients (G-I, 46%; G-II, 54%) were included in the study. All the subjects had isolated GHD. At the beginning of the treatment, the chronological ages (CAs) of the patients in the G-I and G-II groups were 11.8 (10.9–13.7) and 12.8 (12.0–14.3) years, respectively. The ratios of bone age (BA)/CA for the two groups were 0.8 (0.8–0.9) and 1.0 (0.9–1.1), respectively (p&lt; 0.001). After the treatment, the height standard deviation (SD) scores and predicted adult height (PAH) significantly increased from baseline in all subjects in the G-I and G-II groups (p&lt; 0.001; p&lt; 0.001, respectively). There was no significant change in the ratio of BA/CA post-therapy in the G-I group (p= 0.1), while there was a significant decrease in the G-II group (p&lt; 0.001). The growth velocities of the patients in the G-I and G-II groups were 9.1 (7.4–10.1) cm/year [1.5 (0.8–5.0) SD score] and 8.7 (7.5–9.9) cm/year [1.1 (0.3–3.1) SD score], respectively (p= 0.6). While post-therapy serum testosterone concentrations were seen to increase in the G-II group, none of the patients exhibited hematocrit above 50 percent, and the fasting glucose concentrations were normal. Conclusions: When used in addition to rhGH therapy in boys with GHD and advanced BA, AIs were observed to slow down the tempo of BA maturation after 1 year, compared to those who received rhGH treatment alone. AI therapy was found to be safe during the 1-year observation period and thus could be considered for preserving growth potential in these patients.

Published
2023
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28. POS1249 MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN DURING THE COVID-19 PANDEMIC IN TURKEY: FIRST REPORT FROM THE EASTERN MEDITERRANEAN

Author

Ozsurekci, Y., primary, Gürlevik, S., additional, Kesici, S., additional, Kaya Akca, U., additional, Oygar, P. D., additional, Aykac, K., additional, Karacanoglu, D., additional, Saritas Nakip, O., additional, Ilbay, S., additional, Katlan, B., additional, Cengiz, A. B., additional, Basaran, Ö., additional, Cura Yayla, B. C., additional, Karakaya, J., additional, Bilginer, Y., additional, Bayrakci, B., additional, Ceyhan, M., additional, and Özen, S., additional

Published
2021
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33. General gynecology

Author

Salfelder A., Lueken R. P., Bormann C., Gallinat A., Moeller C. P., Busche D., Nugent W., Krueger E., Nugent A., Caglar G., Tasci Y., Kayikcioglu F., Haberal A., Hasskamp Th., Krichbaum M., Aka N., Köse G., Sabah G., Sayharman E. S., Kumru P., Aka N., Karaca K., Köse G., Kumru P., Sayharman E. S., Haydardedeoglu B., Simsek E., Kilicdag E., Tarim E., Bagis T., Dede F. S., Dilbaz B., Dede H., Ilhan A. K., Haberal A., Dede F. S., Dilbaz B., Oral S., Erten A., Ilhan A. K., Haberal A., Ertas I. E., Kahyaoglu S., Turgay I., Tug M., Kalyoncu S., Batioglu S., Zorlu G., Arici C., Akar M. E., Ari E. S., Ari E., Erbay O. U., Caliskan M. O., Akar M. E., Simsek M., Taskm O., Gümüs Il., Turhan N. O., Arikan G., Giuliani A., Kelekci S., Yorgancioglu Z., Yilmaz B., Yasar L., Savan K., Sonmez S., Kart C., Vural M., Tanriverdi H. A., Cinar E., Barut A., Özbay K., Yardim T., Demir B., Kilinc N., Gul T., Erden A. C., Turgay I., Kahyaoglu S., Kokanali M. K., Batioglu S., Haydardedeoglu B., Simsek E., Kilicdag E. B., Tarim E., Aslan E., Bagis T., Seval M., Taskin S., Özmen B., Kahraman K., Yarci A., Tasci T., Unlü C., Taskin S., Seval M., Özmen B., Kahraman K., Gözükücük M., Kurt S., Unlü C., Taskin S., Özmen B., Bozaci E. A., Seval M., Ortac F., Yasar L., Sönmez A. S., Zebitay A. G., Gezer N., Yazicioglu H. F., Mehmetoglu G., Dede F. S., Dilbaz B., Kocak M., Dede H., Haberal A., Erten A., Ilhan A. K., Algül Y. L., Erden A. C., Yasar L., Zebitay A. G., Ozcan J., Duman O., Sonmez S., Yazicioglu F., Sensoy Y., Koc S., Cebi Z., Yasar L., Zebitay A. G., Özcan J., Duman O., Sönmez S., Yazicioglu F., Sensoy Y., Cebi Z., Zebitay A. G., Yasar L., Özcan J., Duman O., Sönmez S., Yazicioglu F., Sensoy Y., Koc S., Cebi Z., Zebitay A. G., Yasar L., Özcan J., Duman O., Sönmez S., Yazicioglu F., Sensoy Y., Cebi Z., Simsek M., Mendilcioglu I., Özekinci M., Ulukus M., Ulukus E. C., Seval Y., Cinar O., Zheng W., Arici A., Erkan L., Soylu F., Tatli O., Ozkent V., Dilbaz B., Ilhan A. K., Oral S., Dede H., Dogan A. R., Gün I., Erdemoglu E., Sargin H., Kamaci M., Dede F. S., Erten A., Sendag F., Akman L., Yucebilgin S., Karadadas N., Oztekin K., Bilgin O., Topuz S., Cigerli E., Iyibozkurt C. A., Akhan E. S., Saygili H., Berkman S., Bezircioglu I., Karakaya E., Baran N., Baloglu A., Aydin C., Hizli N., Cetinkaya B., Kavas A., Baloglu A., Cukurova K., Köksal A., Yetimalar H., Yildiz A., Ivit H., Keklik A., Pinar F., Aka N., Köse G., Tosun N., Kumru P., Tuncel T., Boynukalin K., Salman M. C., Ozyuncu O., Bozdag G., Ayhan A., Ates U., Usta T., Seyhan A., Ata B., Sidal B., Guler O. T., Salman M. C., Bozdag G., Ozyuncu O., Esin S., Ozyuncu O., Salman M. C., Bozdag G., Guven S., Gürban A., Gürban G., Özen S., Kirecci A., Özkesici B., Yücel S., Süer N., Erdemoglu E., Gün I., Sargin H., Erdemoglu C. E., Kamaci M., Akhan S. E., Citil I., Topuz S., Iyibozkurt C., Kesim M. D., Atis A., Aydin Y., Özpak D., Tashan F., Zeteroglu S., Kolusari A., Altunay H., Sahin H. G., Kamaci M., Kayikcioglu F., Erol O., Sarici S., Haberal A., Dingiloglu B. S., Güngör T., Özdal B., Cavkaytar S., Bilge Ü., Mollamahmutoglu L., Toprak Konca M., Özsoy S., Hekim N., Özel E., Senates M., Yener C., Göker N., Caliskan E., Filiz T., Yucesoy G., Coskun E., Vural B., Corakci A., Narin M. A., Caliskan E., Kayikcioglu F., Haberal A., Meydanli M. M., Kamaci M., Sahin H. G., Kolusari A., Yildizbas B., Bolluk G., Ates U., Usta T., Ata B., Seyhan A., Ozdemir B., Sidal B., Ünlü B. S., Aytan H., Evsen S., Tapisiz Ö L., Zergeroglu S., Zeteroglu S., Sahin H. G., Guler A., Kolusari A., Kamaci M., Altay M. M., Can A., Ungormus A., Polat A., and Haberal A.

Published
2005
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36. SAT0243 SUBPHENOTYPES IN POLYARTERITIS NODOSA (PAN): TARGET ORGAN ASSOCIATIONS OF A WORLDWIDE COLLABORATION STUDY

Author

Karadag, O., primary, Bolek, E. C., additional, Furuta, S., additional, Emmi, G., additional, Hocevar, A., additional, Hinojosa-Azaola, A., additional, Mohammad, A. J., additional, Ugurlu, S., additional, Alibaz-Oner, F., additional, Yazici, A., additional, Quartuccio, L., additional, Bozzolo, E., additional, Dagna, L., additional, Ramirez, G. A., additional, Cantarini, L., additional, Gregorini, G., additional, Guido, J., additional, Monti, S., additional, Martin-Nares, E., additional, Schiavon, F., additional, Padoan, R., additional, Kono, H., additional, Vaglio, A., additional, Kiliçkap, S., additional, Ertenli, A. İ., additional, Direskeneli, H., additional, Özen, S., additional, and Jayne, D., additional

Published
2020
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37. OP0272 LONG-TERM EFFICACY AND SAFETY OF CANAKINUMAB IN PATIENTS WITH COLCHICINE-RESISTANT FAMILIAL MEDITERRANEAN FEVER: RESULTS FROM THE RANDOMISED PHASE 3 CLUSTER TRIAL

Author

Özen, S., primary, Ben-Chetrit, E., additional, Foeldvari, I., additional, Amarilyo, G., additional, Ozdogan, H., additional, Vanderschueren, S., additional, Marzan, K., additional, Kahlenberg, J. M., additional, Dekker, E., additional, De Benedetti, F., additional, and Koné-Paut, I., additional

Published
2020
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39. OP0197 THE INITIAL TREATMENT OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS: AN INTERNATIONAL COLLABORATION AMONG 10 REGISTRIES

Author

Son, M. B., primary, Kimura, Y., additional, Aalto, K., additional, Berntson, L., additional, Dallas, J., additional, Duffy, C., additional, Glerup, M., additional, Guzman, J., additional, Herlin, T., additional, Hovi, P., additional, Hyrich, K., additional, Klotsche, J., additional, Magnusson, B., additional, Mcityre, V., additional, Nordal, E., additional, Özen, S., additional, Santos, M. J., additional, Sözeri, B., additional, and Beukelman, T., additional

Published
2020
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41. New features for child metrics: Further growth references and blood pressure calculations

Author

Demir, K., Konakçı, E., Demir, BK., Özen, S., Aydın, M., Darendeliler, F., Bursa Uludağ Üniversitesi/Tıp Fakültesi/Biyoistatik., Özkaya, Güven, and A-4421-2016

Subjects
Male, Turkey, Down syndrome, Guideline, Adolescents, Procedures, Pediatrics, Blood pressure monitoring, ambulatory, Congenital abnormalities, Infant, newborn, Turkey (bird), Congenital disorder, Devices, Growth Hormone, Body Height, Pituitary Dwarfism, Noonan syndrome, Child, Genetic disorder, Endocrinology & metabolism, AAP, Prader Willi syndrome, Standard, Blood pressure monitoring, Protein tyrosine phosphatase SHP 2, Body mass, Hypertension, Systolic blood pressure, Noonan, Blood pressure, Female, Prematurity, Genetic diseases, Human, Practice guidelines as topic, Practice guideline, Child, preschool, Genotype, Application, Turner syndrome, Societies, medical, Growth chart, Mobile, Article, Achondroplasia, Diastolic blood pressure, Humans, Comorbidity assessment, Calculator, Gene mutation, Morphometry, Infant, Weight, Infant, premature, Newborn, Medical society, Short stature, Body height, Inborn, Preschool child, Reference standards, Body weights and measures
Abstract

Many new features have recently been incorporated to CEDD Cozum/Child Metrics, an online and freely accessible scientific toolset. Various auxological assessments can now be made with data of children with genetic diseases (Prader Willi syndrome, Noonan syndrome, Turner syndrome, Down syndrome, and Achondroplasia) and preterm and term newborns. More detailed reports for height, weight, and body mass index data of a given child are now available. Last but not least, office and 24-hour ambulatory blood pressure values can be analyzed according to normative data.

Published
2019

42. A destructive ulcer

Author

Akdeniz, N., Çalka, Ö., Metin, A., Yuca, K., and Özen, S.

Published
2005

46. Diagnosis and treatment approach in newborn infants with ambiguous genitalia with sex development disorder: Turkish neonatal and pediatric endocrinology and diabetes societies consensus report [Cinsiyet gelişim bozukluğu olan ambiguous genitalyalı yenidoğan bebeklerde tanı ve tedavi yaklaşımı: Türk neonatoloji ve çocuk endokrinoloji ve diyabet dernekleri uzlaşı raporu]

Author

Çetinkaya M., Özen S., Uslu S., Gönç N., Sevinir B., Akıncı A., Berberoğlu M., and Çukurova Üniversitesi

Subjects
Atypical genitalia, Disorder of sex development, Ambiguous genitalia, Intersex, Newborn
Abstract

2-s2.0-85065768185, Disorders of sex development are defined as conditions in which the chromosomal, gonadal, and anatomic sex is discordant. Patients usually present with atypical appearing genitals. In the assessment of neonates with disorders of sex development, first, it is important to determine whether this situation requires prompt evaluation, and then the karyotype, hormone levels, and underlying etiology should be determined as soon as possible. All these procedures should be performed in the guidance of a multidisciplinary team in reference centers. The physician should suspect and then perform a detailed history and physical examination because the physical examination of the infant is very important, and lastly plan the required laboratory and imaging procedures for the exact diagnosis. It is important not to be hurried in the choice of sex. The aim of this article, which includes the diagnostic and therapeutic approaches in infants with ambiguous genitalia, was to provide a common practice for all pediatricians. © Copyright 2018 by Turkish Pediatric Association.

Published
2018

47. Evaluation of Physiological Effect of Audiological Test based on Galvanic Skin Response

Author

Polat L. Nurel Özdinç and Özen Şükrü

Subjects
audiological test, galvanic skin response (gsr), physiological effect. root mean square (rms), Mathematics, QA1-939
Abstract

The aim of this study was to determine the physiological effects of the audiological test procedure on individuals and the changes in Galvanic Skin Response (GSR). GSR data from 39 volunteers at rest and during the audiological testing were analyzed and the effects of the audiological testing procedure were evaluated. It was observed that the audiological test showed significant differences according to the resting status in terms of mean value, mean power, Root Mean Square (RMS), Kurtosis, and Skewness. The results obtained in the study show that these differences in GSR can be evaluated according to the physiological effect reflections of the emotional changes created on individuals by the audiological test.

Published
2023
Full Text
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48. In silico validation of the autoinflammatory disease damage index

Author

ter Haar, Nm, van Delft, Alj, Annink, Kv, van Stel, H, Al-Mayouf, Sm, Amaryan, G, Anton, J, Barron, K, Benseler, S, Brogan, Pa, Cantarini, L, Cattalini, M, Cochino, Av, De Benedetti, F, Dedeoglu, F, de Jesus, Aa, Demirkaya, E, Dolezalova, P, Durrant, Kl, Fabio, G, Gallizzi, R, Goldbach-Mansky, R, Hachulla, E, Hentgen, V, Herlin, T, Hofer, M, Hoffman, Hm, Insalaco, A, Jansson, Af, Kallinich, T, Koné-Paut, I, Kozlova, A, Kuemmerle-Deschner, Jb, Lachmann, Hj, Laxer, Rm, Martini, A, Nielsen, S, Nikishina, I, Ombrello, Ak, Özen, S, Papadopoulou-Alataki, E, Quartier, P, Rigante, Donato, Russo, R, Simon, A, Trachana, M, Uziel, Y, Ravelli, A, Schulert, G, Gattorno, M, Frenkel, J, Rigante D (ORCID:0000-0001-7032-7779), ter Haar, Nm, van Delft, Alj, Annink, Kv, van Stel, H, Al-Mayouf, Sm, Amaryan, G, Anton, J, Barron, K, Benseler, S, Brogan, Pa, Cantarini, L, Cattalini, M, Cochino, Av, De Benedetti, F, Dedeoglu, F, de Jesus, Aa, Demirkaya, E, Dolezalova, P, Durrant, Kl, Fabio, G, Gallizzi, R, Goldbach-Mansky, R, Hachulla, E, Hentgen, V, Herlin, T, Hofer, M, Hoffman, Hm, Insalaco, A, Jansson, Af, Kallinich, T, Koné-Paut, I, Kozlova, A, Kuemmerle-Deschner, Jb, Lachmann, Hj, Laxer, Rm, Martini, A, Nielsen, S, Nikishina, I, Ombrello, Ak, Özen, S, Papadopoulou-Alataki, E, Quartier, P, Rigante, Donato, Russo, R, Simon, A, Trachana, M, Uziel, Y, Ravelli, A, Schulert, G, Gattorno, M, Frenkel, J, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

INTRODUCTION: Autoinflammatory diseases can cause irreversible tissue damage due to systemic inflammation. Recently, the Autoinflammatory Disease Damage Index (ADDI) was developed. The ADDI is the first instrument to quantify damage in familial Mediterranean fever, cryopyrin-associated periodic syndromes, mevalonate kinase deficiency and tumour necrosis factor receptor-associated periodic syndrome. The aim of this study was to validate this tool for its intended use in a clinical/research setting. METHODS: The ADDI was scored on paper clinical cases by at least three physicians per case, independently of each other. Face and content validity were assessed by requesting comments on the ADDI. Reliability was tested by calculating the intraclass correlation coefficient (ICC) using an 'observer-nested-within-subject' design. Construct validity was determined by correlating the ADDI score to the Physician Global Assessment (PGA) of damage and disease activity. Redundancy of individual items was determined with Cronbach's alpha. RESULTS: The ADDI was validated on a total of 110 paper clinical cases by 37 experts in autoinflammatory diseases. This yielded an ICC of 0.84 (95% CI 0.78 to 0.89). The ADDI score correlated strongly with PGA-damage (r=0.92, 95% CI 0.88 to 0.95) and was not strongly influenced by disease activity (r=0.395, 95% CI 0.21 to 0.55). After comments from disease experts, some item definitions were refined. The interitem correlation in all different categories was lower than 0.7, indicating that there was no redundancy between individual damage items. CONCLUSION: The ADDI is a reliable and valid instrument to quantify damage in individual patients and can be used to compare disease outcomes in clinical studies.

Published
2018

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