41 results on '"Antmen B"'
Search Results
2. Prediction of factor VIII inhibitor development in the SIPPET cohort by mutational analysis and factor VIII antigen measurement
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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.
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- 2018
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3. Timing and severity of inhibitor development in recombinant versus plasma‐derived factor VIII concentrates: a SIPPET analysis
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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.
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- 2018
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4. Intracranial hemorrhage in hereditary bleeding disorders: the experience of Çukurova University Hemophilia Center: PO 163
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Sasmaz, I, Antmen, B, Karagun, B S, Leblebisatan, G, Yilmaz, M, and Kilinc, Y
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- 2013
5. The effect of water exercise on atrophic muscles associated with limited range of motion in severe haemophilia A patients: PO 100
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Antmen, B
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- 2013
6. Iliopsoas hemorrhage in congenital factor deficiencies: the experience of Çukurova University, Adana, Turkey: PO 099
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Antmen, B, Sasmaz, I, Karagun, B S, Leblebisatan, G, and Kilinc, Y
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- 2013
7. PRO45 NATIONAL BURDEN OF HEMOPHILIA A IN TURKEY
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Malhan, S., primary, Oksuz, E., additional, Antmen, B., additional, Ar, C., additional, Balkan, C., additional, and Kavakli, K., additional
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- 2019
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8. Deferasirox in children with transfusion-dependent thalassemia or sickle
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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
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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.
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- 2019
9. THE 12 MONTHS FOLLOW-UP RESULTS FROM A COHORT OF PEDIATRIC PATIENTS WITH HEMOGLOBINOPATHY AND USING ORAL IRON CHELATOR FOR TRANSFUSIONAL HEMOSIDEROSIS IN TURKEY
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Antmen, B., Karakas, Z., Oktay, G., Kurtoglu, E., Aydogan, G., Akin, M., Salcioglu, Z., Antmen, B, Karakas, Z, Oktay, G, Kurtoglu, E, Aydogan, G, Akin, M, Yesilipek, M, Yeditepe Üniversitesi, and Selçuk Üniversitesi
- Abstract
WOS: 000445782800866, …
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- 2013
10. A COHORT STUDY TO ASSESS THE CONTRIBUTION OF PATIENT COMPLIANCE PROGRAM ON PERSISTENCE TO DEFERASIROX IN PATIENTS WITH CHRONIC IRON OVERLOAD IN TURKEY (EX-PAT PROGRAM)
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Antmen, B, Organ, K, Sasmaz, I, Berktas, M, Kilinc, Y, Çukurova Üniversitesi, Antmen, B, Organ, K, Sasmaz, I, Berktas, M, Kilinc, Y, and Yeditepe Üniversitesi
- Abstract
WOS: 000445782800868 …
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- 2013
11. OUTCOMES OF A COHORT OF CHILDREN WITH HEMOGLOBINOPATHY RECEIVING ORAL IRON CHELATORS FOR TREATMENT OF TRANSFUSIONAL IRON OVERLOAD IN TURKEY: RESULTS OF 3-YEAR FOLLOW-UP STUDY
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Antmen, B., Karakas, Z., Kupesiz, O. A., Sasmaz, I., Kurtoglu, E., Aydogan, G., Salcioglu, Z., and Çukurova Üniversitesi
- Abstract
20th Congress of European-Hematology-Association -- JUN 11-14, 2015 -- Vienna, AUSTRIA WOS: 000361204902241 …
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- 2015
12. CIRCUMCISION AND COMPLICATIONS IN ADOLESCENT AND ADULT PATIENTS WIITH HEMOPHILIA IN SOUTHERN PART OF TURKEY
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Sasmaz, I., Antmen, B., Guvenc, B., Karagun, B., Kilinc, Y., Aridogan, A., and Çukurova Üniversitesi
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20th Congress of European-Hematology-Association -- JUN 11-14, 2015 -- Vienna, AUSTRIA WOS: 000361204904224 …
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- 2015
13. MICROPARTICLE PROFILE DURING PAINFUL CRISIS AND NON-CRISIS PERIOD IN SICKLE CELL ANEMIA
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Atmis, A., Sasmaz, I., Antmen, B., Karagun, B., Kilinc, Y., and Çukurova Üniversitesi
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20th Congress of European-Hematology-Association -- JUN 11-14, 2015 -- Vienna, AUSTRIA WOS: 000361204905004 …
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- 2015
14. OUTCOMES OF A COHORT OF CHILDREN WITH HEMOGLOBINOPATHY RECEIVING ORAL IRON CHELATORS FOR TREATMENT OF TRANSFUSIONAL IRON OVERLOAD IN TURKEY: 24-MONTH FOLLOW-UP
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Antmen, B., Karakas, Z., Kupesiz, O. A., Sasmaz, I., Kurtoglu, E., Aydogan, G., Salcioglu, Z., and Selçuk Üniversitesi
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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
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- 2014
15. INTRACRANIAL HEMORRHAGE IN HEREDITARY BLEEDING DISORDERS: THE EXPERIENCE OF CUKUROVA
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Sasmaz, I., Antmen, B., Karagun, B., Leblebisatan, G., Yilmaz, D., Kilinc, Y., and Çukurova Üniversitesi
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WOS: 000445783600028 …
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- 2013
16. THE SERUM TRAIL AND TRAIL LIGAND ON THE APOPITOTIC PATHWAY IN CHILDHOOD ACUTE LEUKEMIAS
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Antmen, B., Haytoglu, Z., Sasmaz, I., Karagun, B., Kilinc, Y., and Çukurova Üniversitesi
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WOS: 000445782800252 …
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- 2013
17. Circumcision in patients with hemophilia: Ten years experience in Adana, Turkey
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Sasmaz, I., Antmen, B., Leblebisatan, G., Tuncer, R., Kilinc, Y., and Çukurova Üniversitesi
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12th Congress of the European-Hematology-Association -- JUN 07-10, 2007 -- Vienna, AUSTRIA WOS: 000247176901474 … European Hematol Assoc
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- 2007
18. Congenital Factor X deficiency: Case report
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Antmen, B., Sasmaz, I., Leblebisatan, G., Aydogan, B., Kilinc, Y., and Çukurova Üniversitesi
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12th Congress of the European-Hematology-Association -- JUN 07-10, 2007 -- Vienna, AUSTRIA WOS: 000247176901486 … European Hematol Assoc
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- 2007
19. Erythrocyte pyruvate kinase activity during chemotherapy in children with leukemia and lymphoma
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Hayri Levent YILMAZ, Tanyeli, A., Ozüsaglam, H., Kayrin, L., Antmen, B., Sasmaz, H. I., and Çukurova Üniversitesi
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Adult ,Male ,Erythrocytes ,Adolescent ,Lymphoma ,Pyruvate Kinase ,Infant ,Precursor Cell Lymphoblastic Leukemia-Lymphoma ,Recurrence ,Child, Preschool ,Biomarkers, Tumor ,Humans ,Female ,Longitudinal Studies ,Prospective Studies ,Child - Abstract
PubMedID: 12518995 OBJECTIVES: To determine both erythrocyte pyruvate kinase activity (ePKA) at the time of diagnosis of patients with acute leukemia or lymphoma and the differences in the ePKA profiles during the malignant disease and its chemotherapy. METHODS: A prospective, longitudinal clinical study was performed involving 57 patients, 10 were the ones with relapse of acute lymphoblastic leukemia, 32 were the ones with acute lymphoblastic leukemia (ALL) and 15 were the ones with lymphoma. None of the subjects in this study group received treatment or blood transfusion before the study, except the ones diagnosed with relapse of ALL. Forty two healthy children were also selected to form the control group. In order to measure ePKA, blood samples were taken for five times, with 1.5 months apart between each other during the study. Statistical analysis were done by using Wilcoxon's signed rank test, Kruskall-Wallis with Mann-Whitney U Test and Spearman rank correlation coefficient test. RESULTS: The ePKA of the patients with relapse of ALL, and ALL, but not the patients with lymphoma, at the time of diagnosis were found to be lower compared to the one's in the control group (respectively p = 0.001, p = 0.003). The comparison between the first ePKA samples and the third ePKA samples of the patients with both ALL and lymphoma showed a significant increase (respectively p = 0.006, and p = 0.047). CONCLUSION: The measurement of ePKA can be considered for follow-up the neoplastic treatment due to the fact that it is detected to be low in leukemia and relapse of ALL and in normal values after chemotherapy. However, more long-term studies, including more number of cases, are required to be carried out in order to prove the accuracy of this hypothesis. (Tab. 2, Fig. 1, Ref. 28.)
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- 2003
20. Health-related quality of life in patients with haemophilia and inhibitors on prophylaxis with anti-inhibitor complex concentrate: Results from the Pro-FEIBA study
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Gringeri, A, Leissinger, C, Cortesi, P, Jo, H, Fusco, F, Riva, S, Antmen, B, Berntorp, E, Biasoli, C, Carpenter, S, Kavakli, K, Morfini, M, Négrier, C, Rocino, A, Schramm, W, Windyga, J, Zülfikar, B, Mantovani, L, Cortesi, PA, Mantovani LG, Gringeri, A, Leissinger, C, Cortesi, P, Jo, H, Fusco, F, Riva, S, Antmen, B, Berntorp, E, Biasoli, C, Carpenter, S, Kavakli, K, Morfini, M, Négrier, C, Rocino, A, Schramm, W, Windyga, J, Zülfikar, B, Mantovani, L, Cortesi, PA, and Mantovani LG
- Abstract
Patients with haemophilia A and inhibitors are at high risk for severe bleeding, progression of joint disease and deterioration of health-related quality of life (HRQoL). To determine the impact of prophylaxis with an activated prothrombin complex concentrate (aPCC) on HRQoL, HRQoL was assessed using the Short-Form (SF)-36 Health Survey and the EQ-5D questionnaire in subjects ≥14 years participating in a prospective, randomized, crossover study comparing 6 months of aPCC prophylaxis with 6 months of on-demand therapy. Eighteen of 19 patients completed the survey or questionnaire before and after the on-demand therapy and prophylaxis periods. A general trend towards improved HRQoL after prophylaxis was observed for the 18 evaluable patients in all SF-36 dimensions except for vitality/energy and physical functioning. After prophylaxis, 'good responders,' defined as patients experiencing ≥50% reduction in bleeding, exhibited statistically and clinically significant differences in the physical component score (P = 0.021), role - physical (P = 0.042), bodily pain (P = 0.015), and social functioning (P = 0.036). Similarly, the EQ-5D health profile showed a trend towards improvement after prophylaxis in all evaluable patients. Among the good responders, improvements did not differ from those observed after on-demand treatment. EQ visual analogue scale values were slightly improved following prophylaxis for all evaluable patients and the EQ-5D utility index improved in the good responders only. During prophylaxis, patients missed significantly fewer days from school or work because of bleeding than during on-demand treatment (P = 0.01). In conclusion, by significantly reducing bleeding frequency in good responders, aPCC prophylaxis improved HRQoL compared with on-demand treatment.
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- 2013
21. Anti-inhibitor coagulant complex prophylaxis in hemophilia with inhibitors
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Leissinger, C, Gringeri, A, Antmen, B, Berntorp, E, Biasoli, C, Carpenter, S, Cortesi, P, Jo, H, Kavakli, K, Lassila, R, Morfini, M, Neǵrier, C, Rocino, A, Schramm, W, Serban, M, Uscatescu, M, Windyga, J, Zul̈fikar, B, Mantovani, L, CORTESI, PAOLO ANGELO, MANTOVANI, LORENZO GIOVANNI, Leissinger, C, Gringeri, A, Antmen, B, Berntorp, E, Biasoli, C, Carpenter, S, Cortesi, P, Jo, H, Kavakli, K, Lassila, R, Morfini, M, Neǵrier, C, Rocino, A, Schramm, W, Serban, M, Uscatescu, M, Windyga, J, Zul̈fikar, B, Mantovani, L, CORTESI, PAOLO ANGELO, and MANTOVANI, LORENZO GIOVANNI
- Abstract
BACKGROUND: Patients with severe hemophilia A and factor VIII inhibitors are at increased risk for serious bleeding complications and progression to end-stage joint disease. Effective strategies to prevent bleeding in such patients have not yet been established. METHODS: We enrolled patients with hemophilia A who were older than 2 years of age, had high-titer inhibitors, and used concentrates known as bypassing agents for bleeding in a prospective, randomized, crossover study comparing 6 months of anti-inhibitor coagulant complex (AICC), infused prophylactically at a target dose of 85 U per kilogram of body weight (±15%) on 3 nonconsecutive days per week, with 6 months of on-demand therapy (AICC at a target dose of 85 U per kilogram [±15%] used for bleeding episodes). The two treatment periods were separated by a 3-month washout period, during which patients received on-demand therapy for bleeding. The primary outcome was the number of bleeding episodes during each 6-month treatment period. RESULTS: Thirty-four patients underwent randomization; 26 patients completed both treatment periods and could be evaluated per protocol for the efficacy analysis. As compared with on-demand therapy, prophylaxis was associated with a 62% reduction in all bleeding episodes (P<0.001), a 61% reduction in hemarthroses (P<0.001), and a 72% reduction in target-joint bleeding (≥3 hemarthroses in a single joint during a 6-month treatment period) (P<0.001). Thirty-three randomly assigned patients received at least one infusion of the study drug and were evaluated for safety. One patient had an allergic reaction to the study drug. CONCLUSIONS: AICC prophylaxis at the dosage evaluated significantly and safely decreased the frequency of joint and other bleeding events in patients with severe hemophilia A and factor VIII inhibitors. (Funded by Baxter BioScience; Pro-FEIBA ClinicalTrials.gov number, NCT00221195.) Copyright © 2011 Massachusetts Medical Society
- Published
- 2011
22. ZINC LEVELS IN SERUM AND HAIR, PARASITOSIS AND ANEMIA IN SCHOOL-CHILDREN IN SOUTHERN TURKEY 112
- Author
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Kilinç, Y, primary, Antmen, B, additional, Tanyeli, A, additional, and Kümi, M, additional
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- 1997
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23. GLUTATHONE PEROXIDASE, GLUTATHINE REDUCTASE AND VITAMIN E LEVELS IN GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY 129
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öztürk, S, primary, Kilinç, Y, additional, Tanyeli, A, additional, and Antmen, B, additional
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- 1997
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24. Effect of the radiographic contrast material lopamidol on hemostasis: an observational study in thirty cardiac patients.
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Kilinç Y, Sasmaz I, Bozkurt A, Antmen B, and Acartürk E
- Abstract
Background. In vitro studies have shown that nonionic radiographic contrast material may induce the generation of thrombin in blood, whereas ionic contrast agents, such as iohexol, do not. However, knowledge of the effects of contrast material on coagulation and fibrinolytic systems in vivo is limited.Objective: This study was designed to assess the effects of the nonionic radiographic contrast material iopamidol on hemostasis in patients undergoing coronary angiography or cardiac catheterization.Methods: Patients aged >/= 18 years with chest pain and/or dyspnea who underwent coronary angiography or cardiac catheterization with intra-arterial contrast material were assessed for hemostasis. Blood samples were drawn before and 3 minutes after injection of iopamidol. Complete blood count and coagulation profile (bleeding time, clotting time, clot retraction time, euglobulin lysis time [ELT], prothrombin and partial thromboplastin times, coagulation factor I [CFI] level, and platelet factor 3 [PF-3] availability) were assessed. The natural coagulation inhibitors protein C, protein S, and antithrombin III (AT-III) also were measured.Results: Thirty patients (7 males, 23 females; mean [SD] age, 51.3 [20.2] years; range, 17-79 years) were included in this single-center study. All hematologic variables (hemoglobin, white blood cell count, and platelet count) decreased significantly (P < 0.001, P < 0.001, and P < 0.05, respectively) after administration of iopamidol but remained within normal limits. Mean levels of protein C, protein S, and AT-III did not change significantly after administration of iopamidol. Bleeding time was not changed significantly, and PF-3 availability was prolonged in both groups, but the changes were not statistically significant.Conclusions: In this study population, although hemostasis remained grossly intact after injection of nonionic contrast material, the coagulation system may have been affected by the accelerated consumption of CFI and platelets. The affected variables were platelets, clot retraction time, ELT, and natural coagulation inhibitors (protein C, protein S, and AT-III). Although the natural coagulation inhibitors remained within the normal range, the correlations were found significant. These changes in hemostasis affected the vascular phase. If the vascular compartment, especially the endothelium, remained intact, the infusion of nonionic agents in low concentrations might be safe for angiography and other procedures; however, more studies are needed. [ABSTRACT FROM AUTHOR]
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- 2003
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25. A Randomized Trial of Factor VIII and Neutralizing Antibodies in Hemophilia A
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Kaan Kavakli, Mamta Manglani, Ezio Zanon, Christoph Male, Tarek Owaidah, Johnny Mahlangu, Ramabadran Varadarajan, Veronica Soto Arellano, Flora Peyvandi, Alessandra Nunes Loureiro Prezotti, Monica Martinez, Frits R. Rosendaal, Cecil Ross, Suresh Hanagavadi, Suvankar Majumdar, Bulent Zulfikar, Anupam Sachdeva, Brian M. Wicklund, Amal El-Beshlawy, Marilyn J. Manco-Johnson, M. Cerqueira, Dinesh M Nayak, Pier Mannuccio Mannucci, Santiago Bonanad Boix, Nadia P. Ewing, Klaus Schmitt, Angeles Palomo Bravo, Nathan L Kobrinsky, Maria Elisa Mancuso, Shashikant Apte, Mathew Thomas, Isabella Garagiola, Rogelio Paredes Aguilera, Guy Young, Maria Gabriella Mazzucconi, Esperanza Marzouka, Rosario Perez Garrido, Tulika Seth, Bülent Antmen, Mohsen Saleh Elalfy, Peyman Eshghi, E. Santagostino, Mindy L. Simpson, Vijay Ramanan, Daniela Neme, Mehran Karimi, Adriana C Sandoval Gonzalez, Çukurova Üniversitesi, Ege Üniversitesi, [Peyvandi,F, Mannucci,PM, Santagostino,E, Mancuso,ME] the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca’ Granda Ospedale Maggiore Policlinico, Italy. [Peyvandi,F, and Garagiola,I] Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Italy. [Zanon,E] Milan, Clinica Medica II, Azienda Ospedaliera di Padova, Centro Emofilia, Padua. Italy. [Mazzucconi,MG] Ematologia, Unità Operativa Diagnostica Speciale e Terapia delle Malattie dell’Emostasi e della Trombosi, Università Sapienza, Policlinico Umberto I, Rome, Italy. [El-Beshlawy,A] The Pediatric Hematology Department, Cairo University Pediatric Hospital, Cairo. [Elalfy,M] Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo. [Ramanan,V] Jehangir Clinical Development Center, Department of Hematology, Jehangir Hospital Premises, India. , [Apte,S] Sahyadri Speciality Hospita, l India. [Hanagavadi,S] Pune, Jagadguru Jayadeva Murugarajendra Medical College, Davangere, India. [Baradajaran,P] Center for Blood Disorders, Chennai, India. [Manglani,MV] Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, India. [Ross,C] St. John’s Medical College Hospital, Bangalore India. [Seth,T] India Institute of Medical Sciences, Department of Hematology, India. [Sachdeva,A] Pediatric Hematology Oncology and Bone Marrow Transplantation, Institute for Child Health, Sir Ganga Ram Hospital, India. [Nayak,DM] New Delhi, Melaka-Manipal Medical College, Manipal University, Manipal, India. [Thomas,M] Kerala Institute of Medical Science, Trivandrum, India. [Eshghi,P] The Congenital Pediatric Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. [Karimi,M] Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. [Young,G] Children’s Hospital Los Angeles, Los Angeles,California. [Ewing,NP] City of Hope National Medical Center, Duarte, California. [Sandoval Gonzalez, AC] Hospital de Especialidades Unidad Médica de Alta Especialidad, Instituto Mexicano del Seguro Social, Monterrey, Mexico. [Paredes Aguilera,R] Instituto Nacional de Pediatria, Mexico City, Mexico. [Mahlangu,JN] Faculty of Health Sciences, School of Pathology, University of the Witwatersrand, National Health Laboratory Service and Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg.[Bonanad Boix,S] Hospital Universitario La Fe, Unidad Coagulopatias Congenitas, Valencia, Spain. [Perez Garrido, R] Hospital Universitario Virgen del Rocío, Unidad de Hemofilia, Seville, Spain. [Palomo Bravo,A] Hospital Regional Universitario Carlos Haya, Malaga, Spain. [Cerqueira,M] Centro de Pesquisa Clinica Hemorio–Instituto Estadual de Hematologia Arthur de Siqueira Cavalcanti, Rio de Janeiro, Brazil. [Prezotti,A] Centro de Hematologia e Hemoterapia do Espírito Santo, Vitoria, Brazil. [Male,C] Medizinische Universität Wien, Department of Pediatrics, Vienna, Austria. [Schmitt,K] Department of Pediatric and Adolescent Medicine, Kepler University Clinic, Linz, Austria. [Owaidah,T] King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. [Soto Arellano,V] Centro de Hemofílicos del Hospital de Niños Dr. Roberto del Río, Chile. [Marzouka,E] Hospital de Niños Dr. Luis Calvo Mackenna, Centro Hemofílico, Santiago, Chile. [Kobrinsky, NL] Sanford Roger Maris Cancer Center, Fargo, ND. [Majundar,S] University of Mississippi Medical Center, Division of Pediatric Hematology–Oncology, Jackson. [Simpson,M] Rush Hemophilia and Thrombophilia Center, Rush University Medical Center, Chicago. [Antmen,B] Cukurova Universitesi, Tip Fakultesi Pediatrik Hematoloji Bilim Dali, Adana, Turkey. [Kabakli,K] Ege Universitesi Tip Fakultesi Cocuk Sagligi ve Hastalikari Anabilim Dali, Pediatrik Hematoloji Bilim Dali, Turkey. [Zulfikar,B] Istanbul Universitesi Cerrahpasa Tip Fakultesi, Pediatrik Hematoloji Bilim Dali, Istanbul,Turkey. [Manco-Johnson,MJ] Hemophilia and Thrombosis Center, University of Colorado Denver, Aurora. [Martinez,M] Hospital de Niños Sor María Ludovica La Plata, Servicio de Hematología, Buenos Aires. [Neme,D] Fundación de la Hemofilia, Buenos Aires.[Wicklund,BM] Children’s Mercy Hospital, Kansas City, MO, The Netherlands. [Rosendaal,FR] he Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.
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Male ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Models, Statistical::Proportional Hazards Models [Medical Subject Headings] ,Named Groups::Persons::Age Groups::Adult::Middle Aged [Medical Subject Headings] ,Adulto joven ,030204 cardiovascular system & hematology ,Gastroenterology ,law.invention ,Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings] ,0302 clinical medicine ,Randomized controlled trial ,Isoantibodies ,law ,Hemorragia ,hemic and lymphatic diseases ,Medicine ,Child ,Masculino ,Adolescente ,Named Groups::Persons::Age Groups::Child::Child, Preschool [Medical Subject Headings] ,biology ,Adulto ,Incidence ,Hemofilia A ,General Medicine ,Middle Aged ,Modelos de riesgos proporcionales ,Humanos ,Diseases::Hemic and Lymphatic Diseases::Hematologic Diseases::Hemorrhagic Disorders::Hemophilia A [Medical Subject Headings] ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Drug Therapy::Drug Administration Routes::Injections::Injections, Subcutaneous [Medical Subject Headings] ,Child, Preschool ,Niño ,Named Groups::Persons::Age Groups::Adolescent [Medical Subject Headings] ,Drug Therapy, Combination ,Named Groups::Persons::Age Groups::Infant [Medical Subject Headings] ,Antibody ,Incidencia ,Niño preescolar ,Adult ,medicine.medical_specialty ,Randomization ,Adolescent ,Named Groups::Persons::Age Groups::Adult::Young Adult [Medical Subject Headings] ,Injections, Subcutaneous ,Anciano ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Drug Therapy::Drug Therapy, Combination [Medical Subject Headings] ,Check Tags::Male [Medical Subject Headings] ,Hemorrhage ,Hemophilia A ,Isoanticuerpos ,Bethesda unit ,Anticuerpos neutralizantes ,Diseases::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Hemorrhage [Medical Subject Headings] ,Young Adult ,03 medical and health sciences ,Pharmacotherapy ,Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Morbidity::Incidence [Medical Subject Headings] ,Von Willebrand factor ,Internal medicine ,von Willebrand Factor ,Named Groups::Persons::Age Groups::Adult [Medical Subject Headings] ,Humans ,Factor de von Willebrand ,Named Groups::Persons::Age Groups::Adult::Aged [Medical Subject Headings] ,Named Groups::Persons::Age Groups::Child [Medical Subject Headings] ,Aged ,Proportional Hazards Models ,Emicizumab ,Mediana edad ,Factor VIII ,Dose-Response Relationship, Drug ,business.industry ,Phenomena and Processes::Physiological Phenomena::Pharmacological Phenomena::Dose-Response Relationship, Drug [Medical Subject Headings] ,Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Antibodies::Isoantibodies [Medical Subject Headings] ,Infant ,Inyecciones subcutáneas ,Lactante ,Antibodies, Neutralizing ,Relación dosis-respuesta de medicamentos ,Chemicals and Drugs::Biological Factors::Blood Coagulation Factors::von Willebrand Factor [Medical Subject Headings] ,Surgery ,Farmacoterapia combinada ,Chemicals and Drugs::Biological Factors::Blood Coagulation Factors::Factor VIII [Medical Subject Headings] ,biology.protein ,Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Globulins::Serum Globulins::Immunoglobulins::Antibodies::Antibodies, Neutralizing [Medical Subject Headings] ,business ,030215 immunology - Abstract
WOS: 000376443500008, PubMed ID: 27223147, BACKGROUND The development of neutralizing anti-factor VIII alloantibodies (inhibitors) in patients with severe hemophilia A may depend on the concentrate used for replacement therapy. METHODS We conducted a randomized trial to assess the incidence of factor VIII inhibitors among patients treated with plasma-derived factor VIII containing von Willebrand factor or recombinant factor VIII. Patients who met the eligibility criteria (male sex, age = 5 Bethesda units). Inhibitors developed in 29 of the 125 patients treated with plasma-derived factor VIII (20 patients had high-titer inhibitors) and in 47 of the 126 patients treated with recombinant factor VIII (30 patients had high-titer inhibitors). The cumulative incidence of all inhibitors was 26.8% (95% confidence interval [CI], 18.4 to 35.2) with plasma-derived factor VIII and 44.5% (95% CI, 34.7 to 54.3) with recombinant factor VIII; the cumulative incidence of high-titer inhibitors was 18.6% (95% CI, 11.2 to 26.0) and 28.4% (95% CI, 19.6 to 37.2), respectively. In Cox regression models for the primary end point of all inhibitors, recombinant factor VIII was associated with an 87% higher incidence than plasma-derived factor VIII (hazard ratio, 1.87; 95% CI, 1.17 to 2.96). This association did not change in multivariable analysis. For high-titer inhibitors, the hazard ratio was 1.69 (95% CI, 0.96 to 2.98). When the analysis was restricted to recombinant factor VIII products other than second-generation full-length recombinant factor VIII, effect estimates remained similar for all inhibitors (hazard ratio, 1.98; 95% CI, 0.99 to 3.97) and high-titer inhibitors (hazard ratio, 2.59; 95% CI, 1.11 to 6.00). CONCLUSIONS Patients treated with plasma-derived factor VIII containing von Willebrand factor had a lower incidence of inhibitors than those treated with recombinant factor VIII. (Funded by the Angelo Bianchi Bonomi Foundation and others; ClinicalTrials.gov number, NCT01064284; EudraCT number, 2009-011186-88.), Angelo Bianchi Bonomi Foundation, Funded by the Angelo Bianchi Bonomi Foundation and others
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- 2016
26. Anti-inhibitor coagulant complex prophylaxis in hemophilia with inhibitors
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Alessandro Gringeri, Erik Berntorp, Kaan Kavakli, Riitta Lassila, Hyejin Jo, Angiola Rocino, Bulent Zulfikar, Jerzy Windyga, Marusia Valentina Uscatescu, Claude Negrier, Paolo Cortesi, Bülent Antmen, Cindy A. Leissinger, Chiara Biasoli, Lorenzo G. Mantovani, Massimo Morfini, Wolfgang Schramm, Margit Serban, Shannon L. Carpenter, Çukurova Üniversitesi, Leissinger, C, Gringeri, A, Antmen, B, Berntorp, E, Biasoli, C, Carpenter, S, Cortesi, P, Jo, H, Kavakli, K, Lassila, R, Morfini, M, Neǵrier, C, Rocino, A, Schramm, W, Serban, M, Uscatescu, M, Windyga, J, Zul̈fikar, B, and Mantovani, L
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Adult ,Male ,Health Services Research, Adolescent ,medicine.medical_specialty ,Adolescent ,MED/42 - IGIENE GENERALE E APPLICATA ,Hemorrhage ,Hemophilia A ,Drug Administration Schedule ,Statistics, Nonparametric ,Joint disease ,Young Adult ,Anti-Inhibitor Coagulant Complex ,Internal medicine ,medicine ,Humans ,Prospective Studies ,Young adult ,Prospective cohort study ,Child ,Aged ,Bleeding episodes ,Cross-Over Studies ,Factor VIII ,business.industry ,Medicine (all) ,General Medicine ,Cross-Over Studie ,Middle Aged ,Crossover study ,Treatment period ,Blood Coagulation Factors ,Surgery ,Target dose ,Prospective Studie ,Child, Preschool ,Female ,business ,Blood Coagulation Factor ,Human - Abstract
BACKGROUND: Patients with severe hemophilia A and factor VIII inhibitors are at increased risk for serious bleeding complications and progression to end-stage joint disease. Effective strategies to prevent bleeding in such patients have not yet been established. METHODS: We enrolled patients with hemophilia A who were older than 2 years of age, had high-titer inhibitors, and used concentrates known as bypassing agents for bleeding in a prospective, randomized, crossover study comparing 6 months of anti-inhibitor coagulant complex (AICC), infused prophylactically at a target dose of 85 U per kilogram of body weight (±15%) on 3 nonconsecutive days per week, with 6 months of on-demand therapy (AICC at a target dose of 85 U per kilogram [±15%] used for bleeding episodes). The two treatment periods were separated by a 3-month washout period, during which patients received on-demand therapy for bleeding. The primary outcome was the number of bleeding episodes during each 6-month treatment period. RESULTS: Thirty-four patients underwent randomization; 26 patients completed both treatment periods and could be evaluated per protocol for the efficacy analysis. As compared with on-demand therapy, prophylaxis was associated with a 62% reduction in all bleeding episodes (P
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- 2011
27. Busulfan-Based and Treosulfan-Based Myeloablative Conditioning for Allogeneic Transplantation in Children with Thalassemia Major: a Single-Center Experience From Southern Turkey.
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Aygüneş U, Karagun BS, Ay Tuncel D, Sasmaz HI, and Antmen B
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- Humans, Child, Busulfan adverse effects, Retrospective Studies, Quality of Life, Turkey, Transplantation, Homologous adverse effects, Transplantation Conditioning adverse effects, Vidarabine, beta-Thalassemia diagnosis, beta-Thalassemia therapy, beta-Thalassemia complications, Hematopoietic Stem Cell Transplantation adverse effects, Graft vs Host Disease etiology
- Abstract
Objectives: Allogeneic hematopoietic stem cell transplant is the only curative treatment for patients with transfusion-dependent thalassemia major. In recent years, a number of novel approaches have improved patient outcomes and quality of life by minimizing the toxicity of conditioning regimens. The objective of this study was to compare the role of treosulfan- and busulfan-based conditioning in transfusion-dependent thalassemia., Materials and Methods: Data were collected retrospectively on 121 children with beta thalassemia major who underwent hematopoietic stem cell transplant using treosulfan-based (n = 37) or busulfan-based (n = 84) conditioning regimens between 2012 and 2022., Results: Two-year overall survival was 87.5% in the busulfan-based conditioning group and 91.1% in the treosulfan-based conditioning group.The group given the busulfan regimen compared with treosulfan regimen had significantly increased number of side effects (58.3% vs 21.6%, respectively; P < .001). When the busulfan-based regimen by level was evaluated, we observed no significant differences between the frequency of side effects according to drug serum levels. In addition, no significant differences were shown between the 2 regimen groups for cumulative incidence of acute and chronic graft-versus-host disease., Conclusions: The safety and effectiveness of a treosulfan-based myeloablative conditioning regimen has been confirmed by ourretrospective investigation of pediatric patients with beta thalassemia.
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- 2023
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28. Pneumatosis cystoides intestinalis mimicking free intraabdominal air following chemotherapy for relapsed acute myeloblastic leukemia in a transplanted neutropenic child: a case report.
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Aygüneş U, Karagün BŞ, Şaşmaz İ, Tutuş K, Özden Ö, and Antmen B
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- Animals, Female, Humans, Child, Child, Preschool, Tissue Donors, Anti-Bacterial Agents, Pneumatosis Cystoides Intestinalis chemically induced, Pneumatosis Cystoides Intestinalis diagnostic imaging, Leukemia, Myeloid, Acute therapy, Hematopoietic Stem Cell Transplantation
- Abstract
Background: Pneumatosis cystoides intestinalis (PI) is a rare but important condition in which widespread air sacs are found in the submucosa, and subserosa of the bowel wall. Although it has several etiologies, children receiving chemotherapy are at risk for PI. Preferred imaging tools for the diagnosis are abdominal direct radiography and computed tomography. In patients with PI, rupture of intramural air sacs is the source of benign pneumoperitoneum, causing free air without true intestinal perforation. Intestinal perforation or obstruction are indications for surgical intervention., Case: Here, we present a 4-year-old patient diagnosed with acute myeloblastic leukemia (AML), who underwent allogeneic hematopoietic stem cell transplantation (HSCT) from a matched sibling donor (MSD) and developed PI after HSCT. The patient was consulted to the pediatric surgery department, and her oral feeding was stopped. Broad spectrum antibiotics (teicoplanin, metronidazol and vancomycin) were initiated. Her fever increased during the 24-hour monitoring, there was no stool passage, CRP ( > 25 mg/dL, normal value < 1 mg/dL) and abdominal distension increased and there was prolonged neutropenia and radiologic investigations could not rule out intestinal perforation, so the patient underwent exploratory laparotomy. No intestinal perforation was found. There was no sign in the intestinal wall and numerous gas-filled cysts of various sizes., Conclusions: PI is an uncommon complication, and direct radiography/computed tomography scans are very helpful in making the diagnosis in suspicious cases. PI, should be kept in mind, especially in transplanted or relapsed leukemia patients receiving intensive chemotherapy.
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- 2023
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29. Gene therapy in haemophilia: literature review and regional perspectives for Turkey.
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Kavaklı K, Antmen B, Okan V, Şahin F, Aytaç S, Balkan C, Berber E, Kaya Z, Küpesiz A, and Zülfikar B
- Abstract
Haemophilia is an X-linked lifelong congenital bleeding disorder that is caused by insufficient levels of factor VIII (FVIII; haemophilia A) or factor IX (FIX; haemophilia B) and characterized by spontaneous and trauma-related bleeding episodes. The cornerstone of the treatment, factor replacement, constitutes several difficulties, including frequent injections due to the short half-life of recombinant factors, intravenous administration and the risk of inhibitor development. While extended half-life factors and subcutaneous novel molecules enhanced the quality of life, initial successes with gene therapy offer a significant hope for cure. Although adeno-associated viral (AAV)-based gene therapy is one of the most emerging approaches for treatment of haemophilia, there are still challenges in vector immunogenicity, potency and efficacy, genotoxicity and persistence. As the approval for the first gene therapy product is coming closer, eligibility criteria for patient selection, multidisciplinary approach for optimal delivery and follow-up and development of new pricing policies and reimbursement models should be concerned. Therefore, this review addresses the unmet needs of current haemophilia treatment and explains the rationale and principles of gene therapy. Limitations and challenges are discussed from a global and national perspective and recommendations are provided to adopt the gene therapies faster and more sufficient for the haemophilia patients in developing countries like Turkey., Competing Interests: Competing interests: The authors declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: Project and authors’ coordination was provided by Dr Ayse Ozlem Yilmaz and Feride Uçar at Remedium Consulting Group and was funded by Pfizer., (© The Author(s), 2022.)
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- 2022
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30. Different Kinetics and Risk Factors for Isolated Extramedullary Relapse after Allogeneic Hematopoietic Stem Cell Transplantation in Children with Acute Leukemia.
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Hazar V, Öztürk G, Yalçın K, Uygun V, Aksoylar S, Küpesiz A, Ok Bozkaya İ, Karagün BŞ, Bozkurt C, İleri T, Atay D, Koçak Ü, Karasu GT, Yeşilipek A, Gökçe M, Kansoy S, Kintrup GT, Karakükcü M, Okur FV, Ertem M, Kaya Z, Gürsel O, Yaman Y, Özbek N, Antmen B, Tüfekçi Ö, Albayrak C, Adaklı Aksoy B, Sezgin G, Albayrak D, Evim MS, Zengin E, and Pekpak E
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- Child, Humans, Kinetics, Recurrence, Retrospective Studies, Risk Factors, Hematopoietic Stem Cell Transplantation, Leukemia, Myeloid, Acute therapy
- Abstract
Relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the most frequent cause of post-transplantation mortality. Isolated extramedullary (EM) relapse (iEMR) after HSCT is relatively rare and not well characterized, particularly in pediatric patients. We retrospectively analyzed 1527 consecutive pediatric patients with acute leukemia after allo-HSCT to study the incidence, risk factors, and outcome of iEMR compared with systemic relapse. The 5-year cumulative incidence of systemic relapse (either bone marrow [BM] only or BM combined with EMR) was 24.8%, and that of iEMR was 5.5%. The onset of relapse after allo-HSCT was significantly longer in EM sites than in BM sites (7.19 and 5.58 months, respectively; P = .013). Complete response (CR) 2+/active disease at transplantation (hazard ratio [HR], 3.1; P < .001) and prior EM disease (HR, 2.3; P = .007) were independent risk factors for iEMR. Chronic graft-versus-host disease reduced the risk of systemic relapse (HR, 0.5; P = .043) but did not protect against iEMR. The prognosis of patients who developed iEMR remained poor but was slightly better than that of patients who developed systemic relapse (3-year overall survival, 16.5% versus 15.3%; P = .089). Patients experiencing their first systemic relapse continued to have further systemic relapse, but only a minority progressed to iEMR, whereas those experiencing their iEMR at first relapse developed further systemic relapse and iEMR at approximately similar frequencies. A second iEMR was more common after a first iEMR than after a first systemic relapse (58.8% versus 13.0%; P = .001) and was associated with poor outcome. iEMR has a poor prognosis, particularly after a second relapse, and effective strategies are needed to improve outcomes., (Copyright © 2021 The American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. All rights reserved.)
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- 2021
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31. Risk Assessment for BK Virus-Associated Hemorrhagic Cystitis After Pediatric Hematopoietic Stem Cell Transplant: A Single-Center Retrospective Cross-Sectional Study.
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Karagun BS, Aygunes U, Eken A, Akbas T, Melek E, and Antmen B
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Objectives: BK virus-associated hemorrhagic cystitis is a common complication of allogeneic hematopoietic stem cell transplant. It is known to be associated with cyclophosphamide therapy and the intensity of the conditioning regimen as well as infection with the BK virus. Data are limited for BK virus-associated hemorrhagic cystitis in pediatric recipients of allogeneic hematopoietic stem cell transplant. Therefore, we aimed to identify the risk factors and etiology of BK virus-associated hemorrhagic cystitis and determine the factors that may improve the treatment efficacy., Materials and Methods: Data from recipients of allogeneic hematopoietic stem cell transplant were retrospectively analyzed. These data included information about age, sex, underlying disease, the details of ablative conditioning, graft-versus-host disease prophylaxis, donor type, stem cell source, history of acute graft-versus-host disease, and cytomegalovirus reactivation., Results: A total of 50 patients developed BK virusassociated hemorrhagic cystitis among 334 patients. Symptoms associated with BK virus-associated hemorrhagic cystitis manifested an average of 45.3 days after transplant. Most of the patients had grade 2 and grade 3 hemorrhagic cystitis. Risk factor analysis revealed that haploidentical donor type, treatment with busulfan and cyclophosphamide as part of conditioning regimen, and history of total body irradiation increased the risk of BK virus-associated hemorrhagic cystitis in the pediatric recipient population., Conclusions: We found that, despite current conditioning regimens, BK virus-associated infection still leads to a considerable incidence rate of hemorrhagic cystitis in pediatric recipients of allogeneic hematopoietic stem cell transplant. Patients with a haploidentical donor and a history of busulfan and cyclophosphamide treatment or total body irradiation had a higher risk of BK virus-associated hemorrhagic cystitis. Thus, we suggest that patients with these factors should be followed closely after allogeneic hematopoietic stem cell transplant.
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- 2021
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32. Cost of hemophilia A in Turkey: an economic disease burden analysis.
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Malhan S, Öksüz E, Antmen B, Ar MC, Balkan C, and Kavaklı K
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- Health Care Costs, Health Expenditures, Humans, Turkey epidemiology, Cost of Illness, Hemophilia A drug therapy, Hemophilia A epidemiology
- Abstract
Objective: Hemophilia A is the second most common bleeding disorder causing patients to have lifelong follow-up and treatment. Despite being a rare disease, hemophilia A has a high economic burden on individuals and the public. The purpose of this study was to estimate the total disease cost of hemophilia A in Turkey., Materials and Methods: Data used in this analysis were collected through literature review, including studies conducted in Turkey in December 2018. A disease burden analysis was performed by modeling hemophilia A-related costs among patients, their relatives, and the social security system. Two expert panels were held to evaluate real-world data sources and to provide further information. All direct medical and non-medical costs were calculated annually from the Social Security Institution of the Republic of Turkey perspective, while indirect costs were estimated from the patient and community perspective., Results: For the calendar year of 2018, the number of hemophilia A patients in Turkey were estimated to be 5,055, with an average weight of 64.7 kg. The average annual direct medical, direct non-medical, and indirect costs of hemophilia A were calculated as €93,268 ($109,286; ₺502,717), €2,533 ($2,968; ₺13,655), and €7,957 ($9,323; ₺42,888) per patient, respectively, with a total annual cost of €103,759 ($121,578; ₺559,259). For the management of patients with inhibitors (4.9%), the average annual total cost was calculated to be €325,439 ($381,330; ₺1,754,117) per patient. The total annual disease burden of hemophilia A in 2018 was estimated to be about €524 million ($614 million; ₺2.82 billion), which corresponded to 1.6% of the total health expenditure in Turkey., Conclusion: The most important reason hemophilia A has a significant economic burden in Turkey is that replacement therapy is expensive. The major cost contributor was identified as factor replacement therapy. With inhibitor development, the average annual cost increased more than 3-fold.
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- 2021
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33. Muscle strength and joint health in children with hemophilia: a cross-sectional study.
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Tat NM, Tat AM, Can F, Antmen B, and Öner AF
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- Adolescent, Ankle Joint, Child, Cross-Sectional Studies, Hemarthrosis diagnosis, Hemarthrosis epidemiology, Hemarthrosis etiology, Humans, Muscle Strength, Hemophilia A complications
- Abstract
Background and Objectives: We aimed to evaluate joint health in children with hemophilia (CwH) and to investigate the effects of hemarthrosis on the musculoskeletal system., Method: Forty-one CwH aged between 6-18 years participated in the study. Joint health status was evaluated according to Hemophilia Joint Health Score (HJHS). Pain intensity level was assessed in resting and in activity using Visual Analog Scale. Range of motion was measured with goniometer and muscle strength was assessed with digital dynamometer. Arthropathic joints were examined in three groups named knee, elbow and ankle., Results: Physical examination revealed arthropathy findings to be found in 29 knee, 19 elbow and 18 ankle joints. The median of flexion angle of the affected side were 120°, 122° and 12° for the knee, elbow and ankle and extension losses of these joints were 5°, 7° and 0, respectively. In CwH having knee and elbow arthropathy, index joint HJHS was found to be significantly higher than those with ankle arthropathy (p < 0.01). The flexor and extensor muscle strength significantly decreased in 11 CwH with unilateral elbow arthropathy compared to the non-arthropatic side (p < 0.05). In 15 CwH with unilateral ankle arthropathy decreased in the extensor muscle strength (plantarflexors) (p < 0.05). Extension loss showed a good correlation with index HJHS of elbow, knee and ankle joints, respectively. (rs= 0.599, 0.576, 0.606, p < 0.01). We observed that the muscle strength of elbow flexors/extensors and ankle extensors were significantly decreased compared to the non-arthropathic side. However this situation was not detected in knee joint despite having highest index HJHS., Conclusion: Our findings indicate that hemarthrosis may cause more muscle strength loss in the upper extremity than the lower extremity. Furthermore, extension loss was found to be an important parameter in physical examination of hemophilic arthropathy. Musculoskeletal system should be evaluated comprehensively at regular intervals and when necessary rehabilitative treatment should be planned.
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- 2020
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34. Bilateral recurrent external obturator muscle hematoma: An unusual cause of pelvic pain in hemophilia.
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Arpaci T, Sasmaz I, Akbas T, Eken A, Ozgur A, and Antmen B
- Abstract
Following joint hemorrhages, intramuscular hemorrhages are the second most prevalent bleeding pattern in hemophiliac patients. Hematomas of the iliopsoas muscle are a well-known complication of hemophilia; however, obturator muscle hematomas are rare. We herein report a case of spontaneous bleeding of the bilateral external obturator muscles, which occured three times within a period of 9 months in a hemophilia patient with factor VIII inhibitors. To the best of our knowledge, this is the first published case of an obturator externus muscle hematoma in hemophilia. In addition to hip hemarthrosis, iliopsoas hematomas and acute appendicitis, obturator muscle hematoma should be considered as one of the diagnostic alternatives for pelvic pain in hemophiliaψ patients. Magnetic resonance imaging enables rapid diagnosis of obturator muscle hematoma.
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- 2016
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35. Hepatitis A-associated immune thrombocytopenia.
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Leblebısatan G, Tümgör G, Saşmaz I, Ozgür O, and Antmen B
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- Child, Child, Preschool, Glucocorticoids therapeutic use, Humans, Immunoglobulins, Intravenous therapeutic use, Male, Methylprednisolone therapeutic use, Purpura, Thrombocytopenic, Idiopathic drug therapy, Hepatitis A diagnosis, Purpura, Thrombocytopenic, Idiopathic virology
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- 2012
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36. Vascular endothelial growth factor levels in childhood acute lymphoblastic and myeloblastic leukemia.
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Leblebisatan G, Antmen B, Saşmaz I, and Kilinç Y
- Abstract
Angiogenesis has been associated with the growth, dissemination and metastasis and has been shown to be a prognostic. Although there are some data suggesting that angiogenesis may have a role in the pathophysiology of leukemia, its role in patient prognosis is yet to be defined. We analyzed the expression level of vascular endothelial growth factor (VEGF), an angiogenesis promoter and its possible- prognostic value in bone marrow samples at the time of diagnosis and remission of acute childhood leukemia patients. Besides 46 patients diagnosed as ALL or AML, 16 children were also included as a control group in the study. Our data have demonstrated that VEGF levels of AML patients were found higher than the control group statistically (P = 0.022). However we could not find any significant difference between VEGF levels of diagnosis and remission in both AML and ALL groups by blastic VEGF expression (P > 0.05). In this study the higher levels of VEGF in AML patients is one of the main findings although we were not able to assess any role of VEGF in predicting prognosis in pediatric leukemia patients by evaluating blastic cell VEGF expression. These results have demonstrated that the relationship between angiogenesis or angiogenesis promoters and hematological malignancies is not clear and simple as different methods or different cells beside different angiogenesis promotors are involved to these studies. So that not only tumor cells and their cytokines but also surrounding cells and their cytokines must be taken into consideration with the standardized study methods in the further studies to obtain a promising treatment approach.
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- 2012
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37. Concomitance of idiopathic myelofibrosis and amyloidosis.
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Leblebisatan G, Sasmaz I, Antmen B, Ergin M, and Kilinc Y
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- Bone Marrow pathology, Child, Fibrinogen analysis, Humans, Kidney pathology, Male, Proteinuria diagnosis, Amyloidosis complications, Amyloidosis pathology, Primary Myelofibrosis complications, Primary Myelofibrosis pathology
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- 2010
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38. The results of treatment with idarubicin in childhood acute nonlymphoblastic leukemia.
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Saşmaz I, Tanyeli A, Bayram I, Antmen B, Yilmaz L, Küçükosmanoğlu O, and Kilinç Y
- Subjects
- Adolescent, Antineoplastic Combined Chemotherapy Protocols administration & dosage, Bone Marrow Examination, Child, Child, Preschool, Cytarabine administration & dosage, Disease-Free Survival, Etoposide administration & dosage, Female, Humans, Idarubicin administration & dosage, Infant, Leukemia, Myeloid, Acute pathology, Male, Methotrexate administration & dosage, Retrospective Studies, Treatment Outcome, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Leukemia, Myeloid, Acute drug therapy
- Abstract
Anthracycline and cytosine arabinoside are used in combination as the standard therapy for remission induction of acute nonlymphoblastic leukemia. Idarubicin, a synthetic daunorubicin analogue, shows an improved spectrum activity and diminishes acute or chronic toxicity when compared with the other anthracyclines. This study has been carried out in our clinic in order to evaluate the efficiency of the acute nonlymphoblastic leukemia protocol which includes idarubicin. Thirty-eight patients admitted to our Department between 1992-1999 and diagnosed as acute nonlymphoblastic leukemia (ANLL) were included in the study. Their median age was 7 years 6 months (range, 8 months to 14 years). Induction therapy consisted of idarubicin plus cytosine arabinoside and etoposide. Consolidation therapy consisted of two courses, followed by maintenance therapy with thioguanine, cytosine arabinoside, vincristine and cyclophoshamide. The complete remission rate was found to be 71%. The overall survival estimate was found to be 40% for one year and 23% for three years. We established that the protocol with idarubicin reached a higher remission ratio when compared with the other protocols with anthracycline. However, the degree of the hematologic toxicity ratios related to the therapy increased the complication ratios, which affected the long-term life analyses directly. Therefore this protocol may be revised according to socioeconomical conditions, especially in the developing countries.
- Published
- 2004
39. Klippel-trenaunay-Weber syndrome with hydronephrosis and vesicoureteral reflux: an unusual association.
- Author
-
Yildizdaş D, Antmen B, Bayram I, and Yapicioğlu H
- Subjects
- Child, Preschool, Humans, Hydronephrosis diagnosis, Klippel-Trenaunay-Weber Syndrome diagnosis, Male, Urography, Vesico-Ureteral Reflux diagnosis, Hydronephrosis complications, Klippel-Trenaunay-Weber Syndrome complications, Vesico-Ureteral Reflux complications
- Abstract
The Klippel-Trenaunay-Weber syndrome is a rare disorder characterized by congenital vascular hamartomas, limb hypertrophy, cutaneous manifestations, lymphangiomas and atresia of lymph vessels with non-pitting edema. A three-year-old boy was referred to our clinic for progressive hypertrophy of leg and feet with 32-month history. We diagnosed Klippel-Trenaunay-Weber syndrome, and determined vesicoureteral reflux in our patient. To our knowledge, hydronephrosis and vesicoureteral reflux have not been described previously in the KTWS.
- Published
- 2002
40. Erythrocyte pyruvate kinase activity during chemotherapy in children with leukemia and lymphoma.
- Author
-
Yilmaz HL, Tanyeli A, Ozüsaglam H, Kayrin L, Antmen B, and Sasmaz HI
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Infant, Longitudinal Studies, Lymphoma drug therapy, Lymphoma enzymology, Male, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy, Precursor Cell Lymphoblastic Leukemia-Lymphoma enzymology, Prospective Studies, Recurrence, Biomarkers, Tumor blood, Erythrocytes enzymology, Lymphoma diagnosis, Precursor Cell Lymphoblastic Leukemia-Lymphoma diagnosis, Pyruvate Kinase blood
- Abstract
Objectives: To determine both erythrocyte pyruvate kinase activity (ePKA) at the time of diagnosis of patients with acute leukemia or lymphoma and the differences in the ePKA profiles during the malignant disease and its chemotherapy., Methods: A prospective, longitudinal clinical study was performed involving 57 patients, 10 were the ones with relapse of acute lymphoblastic leukemia, 32 were the ones with acute lymphoblastic leukemia (ALL) and 15 were the ones with lymphoma. None of the subjects in this study group received treatment or blood transfusion before the study, except the ones diagnosed with relapse of ALL. Forty two healthy children were also selected to form the control group. In order to measure ePKA, blood samples were taken for five times, with 1.5 months apart between each other during the study. Statistical analysis were done by using Wilcoxon's signed rank test, Kruskall-Wallis with Mann-Whitney U Test and Spearman rank correlation coefficient test., Results: The ePKA of the patients with relapse of ALL, and ALL, but not the patients with lymphoma, at the time of diagnosis were found to be lower compared to the one's in the control group (respectively p = 0.001, p = 0.003). The comparison between the first ePKA samples and the third ePKA samples of the patients with both ALL and lymphoma showed a significant increase (respectively p = 0.006, and p = 0.047)., Conclusion: The measurement of ePKA can be considered for follow-up the neoplastic treatment due to the fact that it is detected to be low in leukemia and relapse of ALL and in normal values after chemotherapy. However, more long-term studies, including more number of cases, are required to be carried out in order to prove the accuracy of this hypothesis. (Tab. 2, Fig. 1, Ref. 28.)
- Published
- 2002
41. Oral Health Status in Children with Acute Lymphoblastic Leukemia and Lymphoma.
- Author
-
Doğan C, Haytaç C, Antmen B, Şaşmaz İ, and Tanyeli A
- Abstract
We evaluated the oral health status of 85 acute lymphoblastic leukemia (ALL)/lymphoma pediatric patients who received remission-induction and maintenance chemotherapy and 85 age and sex-matched healthy children with the criteria of World Health Organization (WHO) and to determine the prevalence and distribution of dental problems in order to constitute preventive dentistry precautions in this study. The gingival tissues were scored with Community Index of Periodontal Treatment Necessity (CPITN) and dmf-t and DMF-T indices were used for caries evaluation. In the study group, malocclusion was found in 24 patients (28.2%). CPITN was scored as follows in the study group; 11% of the patients had healthy gingiva (Grade 0), the presence of plaque (Grade I) 79% of the patients, the presence of calculus (Grade II) 10% of patients were observed. Nevertheless, mucositis was found with various grades in 9 patients who received chemotherapy. Decayed teeth were found in the 76 patients and in 45 healthy children. 91.7% of patients and 52.9% of children needed dental treatment were determined. The DMF-T and dmf-t scores showed that ALL/lymphoma patients had more decayed and needed more dental treatment, missing or filled teeth both in their deciduous (p< 0.001) and permanent (p< 0.05) dentition when compared to systemically healthy children.
- Published
- 2001
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