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1. Prediction of factor VIII inhibitor development in the SIPPET cohort by mutational analysis and factor VIII antigen measurement

Author

Spena, S., Garagiola, I., Cannavò, A., Mortarino, M., Mannucci, P.M., Rosendaal, F.R., Peyvandi, F., El‐Beshlawy, A., Elalfy, M., Ramanan, V., Eshghi, P., Hanagavadi, S., Varadarajan, R., Karimi, M., Manglani, M.V., Ross, C., Young, G., Seth, T., Apte, S., Nayak, D.M., Santagostino, E., Mancuso, M.E., Sandoval Gonzalez, A.C., Mahlangu, J.N., Bonanad Boix, S., Cerqueira, M., Ewing, N.P., Male, C., Owaidah, T., Soto Arellano, V., Kobrinsky, N.L., Majumdar, S., Perez Garrido, R., Sachdeva, A., Simpson, M., Thomas, M., Zanon, E., Antmen, B., Kavakli, K., Manco‐Johnson, M.J., Martinez, M., Marzouka, E., Mazzucconi, M.G., Neme, D., Palomo Bravo, A., Paredes Aguilera, R., Prezotti, A., Schmitt, K., Wicklund, B.M., and Zulfikar, B.

Published
2018
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2. Timing and severity of inhibitor development in recombinant versus plasma‐derived factor VIII concentrates: a SIPPET analysis

Author

Peyvandi, F., Cannavò, A., Garagiola, I., Palla, R., Mannucci, P.M., Rosendaal, F.R., El‐Beshlawy, A., Elalfy, M., Ramanan, V., Eshghi, P., Hanagavadi, S., Varadarajan, R., Karimi, M., Manglani, M.V., Ross, C., Young, G., Seth, T., Apte, S., Nayak, D.M., Santagostino, E., Elisa Mancuso, M., Sandoval Gonzalez, A.C., Mahlangu, J.N., Bonanad Boix, S., Cerqueira, M., Ewing, N.P., Male, C., Owaidah, T., Soto Arellano, V., Kobrinsky, N.L., Majumdar, S., Perez Garrido, R., Sachdeva, A., Simpson, M., Thomas, M., Zanon, E., Antmen, B., Kavakl, K., Manco‐Johnson, M.J., Martinez, M., Marzouka, E., Mazzucconi, M.G., Neme, D., Palomo Bravo, A., Paredes Aguilera, R., Prezotti, A., Schmitt, K., Wicklund, B.M., and Zulfikar, B.

Published
2018
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6. PHARMACOKINETIC-GUIDED PROPHYLAXIS BASED ON BAYESIAN MODEL WITH MYPKFIT (R) IN HEMOPHILIA A: TURKISH EXPERIENCE

Author

Zulfikar, B., Albayrak, D., Sasmaz, I., Celkan, T., Ozbek, N. Y., Turkkan, E., Canbolat, A., Karaman, S., Yagci, M., Evim, M., Ozdemir, G. N., Aytac, S., Koc, B., Albayrak, C., Balkan, C., Gulen, H., Ay, Y., Okan, V., Aksu, S., Ileri, T., Andic, N., Oymak, Y., Patiroglu, T., Sahin, F., Erduran, E., Kavakli, K., Kaya, Z., Yilmaz, B., Antmen, B., Soker, M., Zengin, E., Kupesiz, A., Oren, H., and Umit, E.

Published
2020

18. Pharmacokinetic-guided prophylaxis based on bayesian model with myPKFiT (R) in hemophilia A: Turkish experience

Author

Balkan, C., Albayrak, C., Ozbek, N. Y., Kaya, Z., Sasmaz, I., Celkan, T., Yagci, M., Karaman, S., Erduran, E., Sahin, F., Ozdemir, G. N., Patiroglu, T., Oymak, Y., Turkkan, E., Evim, M. S., Gulen, H., Ay, Y., Okan, V., Aksu, S., Ileri, T., Salcioglu, Z., Andic, N., Yilmaz, B., Antmen, B., Albayrak, D., Oren, H., Kupesiz, A., Zengin, E., Soker, M., Ar, C., and Kavakli, K.

Published
2019

20. Deferasirox in children with transfusion-dependent thalassemia or sickle

Author

Antmen, B, Karakas, Z, Yesilipek, MA, Kupesiz, OA, Sasmaz, I, Uygun, V, Kurtoglu, E, Oktay, G, Aydogan, G, Akin, M, Salcioglu, Z, Vergin, C, Kazanci, EG, Unal, S, Caliskan, U, Aral, YZ, Turkkan, E, Gunes, AM, Tunc, B, Gumruk, F, Ayhan, AC, Soker, M, Koc, A, Oymak, Y, Ertem, M, Timur, C, Yildirmak, Y, Irken, G, Apak, H, Biner, B, Eren, TG, Balci, YI, Kocak, U, Karasu, G, Akkaynak, D, and Patiroglu, T

Subjects
hemoglobinopathy, iron chelation, iron overload, pediatric, transfusion
Abstract

Objectives To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (>= 100 mL/kg of pRBC or a serum ferritin [SF] level >1000 mu g/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 mu g/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 mu g/L), SCA (1655.5 to 1260 mu g/L), and across age groups of 2-6 years (1971.5 to 1499 mu g/L), 7-12 years (1688.5 to 1159.8 mu g/L), and 13-18 years (1496.5 to 1107 mu g/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses >= 30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. Conclusions Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (>= 30 mg/kg/d) may be required to achieve iron balance. C1 [Antmen, Bulent] Acibadem Hosp, Adana, Turkey. [Karakas, Zeynep] Istanbul Univ, Med Fac, Istanbul, Turkey. [Yesilipek, Mehmet Akif; Kupesiz, Osman Alphan] Akdeniz Univ, Med Fac, Antalya, Turkey. [Sasmaz, Ilgen] Cukurova Univ, Med Fac, Adana, Turkey. [Uygun, Vedat; Kurtoglu, Erdal] Antalya Training & Res Hosp, Antalya, Turkey. [Oktay, Gonul] Antakya State Hosp, Antakya, Turkey. [Aydogan, Gonul; Salcioglu, Zafer] Kanuni Sultan Suleyman Training & Res Hosp, Istanbul, Turkey. [Akin, Mehmet] Denizli State Hosp, Denizli, Turkey. [Vergin, Canan] Dr Behcet Uz Child Dis Surg Training & Res Hosp, Izmir, Turkey. [Kazanci, Elif Guler] Dortcelik Child Dis Hosp, Bursa, Turkey. [Unal, Selma] Mersin Univ, Med Fac, Mersin, Turkey. [Caliskan, Umran] Necmettin Erbakan Univ, Meram Med Fac, Konya, Turkey. [Aral, Yusuf Ziya] Adnan Menderes Univ, Med Fac, Aydin, Turkey. [Turkkan, Emine] Okmeydani Training & Res Hosp, Istanbul, Turkey. [Gunes, Adalet Meral] Uludag Univ, Med Fac, Bursa, Turkey. [Tunc, Bahattin] Hematol Oncol Training & Res Hosp, Ankara Child Hlth & Dis, Ankara, Turkey. [Gumruk, Fatma] Hacettepe Univ, Med Fac, Ankara, Turkey. [Ayhan, Aylin Canbolat; Timur, Cetin] Goztepe Training & Res Hosp, Istanbul, Turkey. [Soker, Murat] Dicle Univ, Med Fac, Diyarbakir, Turkey. [Koc, Ahmet; Oymak, Yesim] Harran Univ, Med Fac, Sanliurfa, Turkey. [Ertem, Mehmet] Ankara Univ, Fac Med, Ankara, Turkey. [Yildirmak, Yildiz] Sisli Etfal Training & Res Hosp, Istanbul, Turkey. [Irken, Gulersu] Dokuz Eylul Univ, Med Fac, Izmir, Turkey. [Apak, Hilmi] Istanbul Univ, Cerrahpasa Med Fac, Istanbul, Turkey. [Biner, Betul; Eren, Tugba Gurleyen] Trakya Univ, Med Fac, Edirne, Turkey. [Balci, Yasemin Isik] Pamukkale Univ, Med Fac, Denizli, Turkey. [Kocak, Ulker] Gazi Univ, Med Fac, Ankara, Turkey. [Karasu, Gulsun] Istanbul Zeynep Kamil Women & Childrens Dis Raini, Istanbul, Turkey. [Akkaynak, Diyar] Novartis Saglik Gida & Tarim Urunleri San & Tic A, Istanbul, Turkey. [Patiroglu, Turkan] Erciyes Univ, Med Fac, Kayseri, Turkey.

Published
2019

31. Oral mucormycosis in children with leukemia: report of 2 cases.

Author

Dogan MC, Leblebisatan G, Haytac MC, Antmen B, and Surmegozler O

Abstract

Mucormycosis is a rare acute opportunistic infection caused by a saprophytic fungus, which belongs to the order Mucorales. This report describes intraoral mucormycosis in 2 children with acute leukemia who were undergoing chemotherapy and had febrile neutropenia. A 7-year-old boy with acute myeloid leukemia and a 9-year-old boy with acute lymphoblastic leukemia were referred to the Department of Pediatric Dentistry at Cukurova University for their intraoral soft tissue lesions, which were diagnosed as mucormycosis by histologic examination. While, for the first case, the lesion was debrided under general anesthesia and medical antifungal therapy was performed, only medical management was done, without any debridement, for the second case. Early recognition of mucormycosis is necessary to limit the spread of infection, which can lead to high morbidity and mortality. Therefore, health practitioners should be familiar with the signs and symptoms of the disease. [ABSTRACT FROM AUTHOR]

Published
2007

46. National pediatric hematopoietic stem cell transplantation activity in Turkey

Author

Kansoy, S., Hazar, V., Kupesiz, A., Uckan, D., Kuskonmaz, B., Tezcan, I., Aksoylar, S., Tezcan, G., Kilic, S. Caki, Ertem, M., Unal, E., Ikinciogullari, A., Uygun, V., Ozturk, G., Erbey, F., Gedikoglu, G., Kurekci, E., Kesik, V., Musa Karakukcu, Albayrak, D., Oniz, H., Azik, F., Tanyeli, A., Anak, S., Kocak, U., Oren, H., Antmen, B., Fisgin, T., Ozyurek, E., Atay, D., Gozmen, S., and Yesilipek, A.

Subjects
Transplantation, surgical procedures, operative, National Pediatric Hematopoietic, Activity
Abstract

European Society Blood and Marrow Transplantation

Published
2015

47. Efficacy and safety of prophylaxis with once-weekly BAY 79-4980 compared with thrice-weekly rFVIII-FS in haemophilia A patients: A randomised, active-controlled, double-blind study

Author

Powell, Jerry, Martinowitz, Uri, Windyga, Jerzy, Hellmann, Andrzej, Pabinger, Ingrid, Enriquez, Monika Maas, Schwartz, Lawrence, Ingerslev, Jørgen, Group Author: Pabinger Fasching I, Hermans C, Poon MC, Ritchie B, Tinmouth A, Teitel J, Zupancic Salek S, Ingerslev J, Chambos H, Schved JF, Guillet B, Oldenburg J, Klamroth R, Martinowitz U, Santagostino E, Schinco P, Musso R, Morfini M, Rocin A, Meijer K, Larosvan B, Holme P, Windyga J, Skotnicki A, Hellmann A, Zawilska K, Robak T, Soto I, Haya S, Kavakli K, Antmen B, Yesilipek MA, Hay C, Wilde J, Rangarajan S, Bernstein J, Damon L, Gill J, Gruppo R, Cuker A, Kuriakose P, Lin J, Manco Johnson M, Mathew P, Neufeld E, Soni A, Powell J, Kulkarni R, Wicklund B., DI MINNO, GIOVANNI, DI MINNO, MATTEO, Powell, Jerry, Martinowitz, Uri, Windyga, Jerzy, DI MINNO, Giovanni, Hellmann, Andrzej, Pabinger, Ingrid, Enriquez, Monika Maa, Schwartz, Lawrence, Ingerslev, Jørgen, Group Author: Pabinger Fasching, I, Hermans, C, Poon, Mc, Ritchie, B, Tinmouth, A, Teitel, J, Zupancic Salek, S, Ingerslev, J, Chambos, H, Schved, Jf, Guillet, B, Oldenburg, J, Klamroth, R, Martinowitz, U, DI MINNO, Matteo, Santagostino, E, Schinco, P, Musso, R, Morfini, M, Rocin, A, Meijer, K, Larosvan, B, Holme, P, Windyga, J, Skotnicki, A, Hellmann, A, Zawilska, K, Robak, T, Soto, I, Haya, S, Kavakli, K, Antmen, B, Yesilipek, Ma, Hay, C, Wilde, J, Rangarajan, S, Bernstein, J, Damon, L, Gill, J, Gruppo, R, Cuker, A, Kuriakose, P, Lin, J, Manco Johnson, M, Mathew, P, Neufeld, E, Soni, A, Powell, J, Kulkarni, R, and Wicklund, B.

Subjects
0301 basic medicine, Male, Sucrose, rFVIII-FS, Chemistry, Pharmaceutical, 030204 cardiovascular system & hematology, Polyethylene Glycol, Polyethylene Glycols, law.invention, 0302 clinical medicine, Randomized controlled trial, law, Delayed-Action Preparation, Clinical endpoint, Medicine, Prophylaxi, Hematology, Middle Aged, Liposome, PEGylated liposome, Treatment Outcome, Tolerability, Solvent, Human, Adult, medicine.medical_specialty, Haemophilia, Adolescent, Haemophilia A, Hemorrhage, Hemophilia A, Drug Administration Schedule, 03 medical and health sciences, Young Adult, Double-Blind Method, Internal medicine, Humans, Dosing, Factor VIII, business.industry, medicine.disease, Surgery, Regimen, 030104 developmental biology, Delayed-Action Preparations, Liposomes, Solvents, business, Bay
Abstract

SummaryThe benefits of prophylaxis of haemophilia A patients regarding joint health and quality-of-life are well established. However, adherence to an up to every-other-day infusion regimen is a barrier to widespread adoption of prophylaxis. BAY 79–4980 is an investigational drug consisting of rFVIII-FS (sucrose-formulated recombinant FVIII) reconstituted with liposome solvent. Previous clinical studies showed extended protection from bleeding after a single injection of BAY 79–4980 (13.3 ± 6.2 days) compared with rFVIII-FS (7.2 ± 1.7 days). The effect of once-a-week prophylaxis with BAY 79–4980 (35 IU/kg) compared with three times-per-week rFVIII-FS (25 IU/kg) in previously treated, severe haemophilia A patients was evaluated in a 52-week, double-blind, two-arm, randomised, controlled study. The primary and secondary endpoints were protection from total bleeds and joint bleeds, respectively. Short- and long-term safety and tolerability of BAY 79–4980 including effects on lipid levels were assessed. A total of 139 and 131 subjects were evaluable for safety and efficacy analyses, respectively. A large difference in efficacy between treatment groups was observed with 72.1% (49/68) in the rFVIII-FS control group demonstrating

Published
2012

50. OUTCOMES OF A COHORT OF CHILDREN WITH HEMOGLOBINOPATHY RECEIVING ORAL IRON CHELATORS FOR TREATMENT OF TRANSFUSIONAL IRON OVERLOAD IN TURKEY: 24-MONTH FOLLOW-UP

Author

Antmen, B., Karakas, Z., Kupesiz, O. A., Sasmaz, I., Kurtoglu, E., Aydogan, G., Salcioglu, Z., and Selçuk Üniversitesi

Subjects
education, social sciences, humanities, geographic locations, health care economics and organizations
Abstract

19th Congress of the European-Hematology-Association -- JUN 12-15, 2014 -- Milan, ITALY, WOS: 000342830901013, …, European Hematol Assoc

Published
2014

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