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    Akut İshali Olan Hastalarda Rotavirüs ve Norovirüs Sıklığının Araştırılması
    (2019) Arslan, Ayşe; Çiçek, Candan; Aksoylar, Serap; Saz, Eylem Ulaş; Taşbakan, Meltem Işıkgöz
    Giriş: Bu çalışmada, yaklaşık bir yıllık süreçte bir üniversite hastanesine akut ishal şikayetiyle ayaktan başvuran veya akut gastroenterit ön tanısıyla hastanede yatarak izlenen hastalardaki rotavirüs ve norovirüs sıklığının araştırılması amaçlanmıştır. Materyal ve Metod: Eylül 2016-Ağustos 2017 tarihleri arasında akut gastroenterit ön tanısıyla hastaneye başvuran hastalardan laboratuvara gönderilen 408 dışkı örneği incelenmiştir. Bu örnekler, yaş aralığı bir ay ile 84 yaş arasında değişen (medyan: 3.0 yaş, ortalama: 10.25 yaş), 341 (%83.5)’i çocuk, 67 (%16.4)’si yetişkin hastalardan toplanmıştır. Dışkı örneklerinin 206 (%50.5)’sı polikliniğe başvuran, 202 (%49.5)’si yatan hastalardan elde edilmiştir. Akut gastroenterit ön tanılı hastalardan toplanan dışkı örnekleri norovirüs (genotip I ve II) ve rotavirüs türlerini kalitatif olarak saptayan otomatize ve kapalı bir sistem olan multipleks gerçek zamanlı polimeraz zincir reaksiyonu (rt-PCR) testi (BD MAX™ Enteric Viral Panel, BD Diagnostics, Baltimore, MD, USA) ile incelenmiştir. Bu multipleks PCR sisteminde örnekte bulunan viral nükleik asitler kapalı ve otomatize şekilde ekstrakte edilip, revers transkripsiyonla komplementer DNA (cDNA) sentezlenip, hedef cDNA rt-PCR yöntemiyle amplifiye edildikten sonra virüsler kalitatif olarak saptanmıştır. Bulgular: Akut gastroenterit şikayetiyle başvuran 408 hastanın 223 (%54.7)’ü en az bir virüs açısından pozitif bulunmuştur. Pozitif örneklerin 184 (%45.1)’ünde rotavirüs, 26 (%6.4)’sında norovirüs genotip II, 1 (%0.2)’inde norovirüs genotip I ve 12 (%2.9)’sinde rotavirüs ve norovirüs genotip II birlikte saptanmıştır. Pozitif bulunan örneklerin 121 (%54.3)’i poliklinik hastalarından, 102 (%45.7)’si yatan hastalardan gönderilmiştir (p= 0.094). Rotavirüs için pozitiflik oranı pediatrik ve yetişkin hasta grupları için sırasıyla %48.4 (165/341) ve %46.3 (31/67) olarak saptanmıştır (p= 0.751). Rotavirüs pozitifliğinin en çok mart (%75), şubat (%70) ve ocak (%60) aylarında olduğu görülmüştür. Sonuç: Akut viral gastroenterit ön tanısıyla laboratuvara gönderilen dışkı örneklerinde özellikle kış aylarında rotavirüs pozitifliği yaklaşık %48 oranında, norovirüs pozitifliği ise yaklaşık %10 oranında bulunmuştur. Yatan ya da ayaktan hastaların pozitiflik oranlarında ve çocuk ya da erişkin hastaların pozitiflik oranlarında anlamlı bir fark bulunamamıştır
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    ASSESSMENT OF SCHOOL-RELATED PROBLEMS OF CHILDHOOD CANCER PATIENTS: A STUDY FROM WEST OF TURKEY
    (Wiley Periodicals, Inc, 2012) Yilmaz, Medine; Sari, Hatice Yildirim; Kantar, Mehmet; Aksoylar, Serap; Cetingul, Nazan; Erermis, Serpil
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    The Association of Minor Congenital Anomalies and Childhood Cancer
    (Wiley, 2011) Durmaz, Asude; Durmaz, Burak; Kadioglu, Bengu; Aksoylar, Serap; Karapinar, Deniz; Koturoglu, Guldane; Orman, Mehmet N.; Özkınay, Ferda; Cogulu, Ozgur
    Background. Although the association of some congenital malformations and specific genetic syndromes is well understood, the association between minor anomalies and cancer is not well known. In recent years some researchers have reported studies establishing this association in different types of cancer. In this study, we aimed to investigate the prevalence and patterns of age-independent minor anomalies in childhood cancer patients. Procedure. Two hundred patients with various types of cancer and 200 healthy controls were examined by two different medical geneticists for minor anomalies who evaluated all the cases and controls simultaneously. Besides minor anomalies, information on the consanguinity between the parents and occurrence of cancer in relatives were also recorded. The types of minor anomalies in different types of cancer, the number of minor anomalies in patients and controls, the association between cancer and the occurrence of different types of minor anomalies were also evaluated. Results. The consanguinity and the history of cancer in relatives were significantly more prevalent in patients (P = 0.04 and P < 0.001, respectively). The number of minor anomalies in patients were significantly higher compared to the controls (P < 0.01). Particularly, the presence of hypertelorism, high-arched palate (approximately 40-fold higher, 95% CI: 12.895-125.037) and hand-foot anomalies were found to be more prevalent in patients having cancer compared to the controls. Conclusion. The common pathways during the embryogenesis may play a role in the development of cancer. The presence and the combination of minor anomalies seem to be associated with a higher prevalence of cancer. Pediatr Blood Cancer 2011;56:1098-1102. (C) 2011 Wiley-Liss, Inc.
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    Bone mineral density in survivors of childhood acute lymphoblastic Leukemia and risk factors
    (Karger, 2008) Goksen, Damla; Aksoylar, Serap; Ozen, Samim; Demirag, Bengu; Cetingul, Nazan; Darcan, Sukran
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    A BRIEF OVERVIEW TO PEDIATRIC GRAY ZONE LYMPHOMAS
    (Wiley, 2022) Ataseven, Eda; Ozek, Gulcihan; Ozsan, Nazan; Kamer, Serra; Anacak, Yavuz; Aksoylar, Serap; Kantar, Mehmet
    [No Abstract Available]
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    A Case of Allergic Broncopulmonary Aspergillosis Associated With Hematopoietic Stem Cell Transplantation Due to Chronic Granulomatous Disease
    (Lippincott Williams & Wilkins, 2019) Malbora, Baris; Aksoylar, Serap; Ozdemir, Hamiyet H.; Ozdemir, Sinem; Kansoy, Savas
    Allergic bronchopulmonary aspergillosis is an immunologic pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus. This disorder is most commonly seen in patients with poorly controlled asthma and cystic fibrosis. It is rarely reported in chronic granulomatous disease patients; however, there are no cases reported with hematopoietic stem cell transplantation in the English literature. Herein, we report a patient with chronic granulomatous disease who had hematopoietic stem cell transplantation and subsequently developed allergic bronchopulmonary aspergillosis.
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    Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome
    (2019) Toret, Ersin; Ay, Yılmaz; Aksoylar, Serap; Karapınar, Tuba Hilkay; Oymak, Yeşim
    Type II Griscelli Syndrome (GS) is caused by a mutation in the RAB27A gene and usually manifests with silvery-gray hair, immune deficiency and the development of hemophagocytic lymphohistiocytosis (HLH). A hematopoietic stem cell transplantation is the curative treatment for HLH and reduced-intensity conditioning prevents the morbidity/mortality in the transplantation related to myeloablative conditioning. We report on a 21-month old boy with cerebral involvement of HLH related to GS.
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    Chimerism Analysis of Children with Allogeneic Stem-Cell transplantation and Its Effect on Survival
    (Galenos Publ House, 2022) Tekguc, Hakan; Aksoylar, Serap; Tekguc, Doga Ceren; Kansoy, Savas
    BACKGROUND/AIMS: Allogeneic hematopoietic stem cell transplantation (Allo-HSCT) is an important and usually the only curative clinical tool for treating pediatric patients with many hereditary and acquired diseases. Although complete donor stem cell engraftment is the desired result of Allo-HSCT, patients do not always have a definite engraftment and end up with mixed chimerism. Many factors both related to patient and transplantation can affect chimerism levels. Additionally, mixed chimerism levels may affect the event free survival (EFS) differently in distinct diseases. The major goals of this study were to determine the first 100-day donor chimerism ratios and to search for a relationship between donor chimerism success (CS) (for malignant diseases, hematopoietic donor chimerism >95%; for non-malignant diseases, >70%) and EFS for pediatric patients.MATERIALS AND METHODS: We collected data from 95 pediatric patients who underwent Allo-HSCT between March, 2005 and April, 2010 at Ege University Hospital with at least one chimerism result obtained within the first 100 days.RESULTS: After checking for all other factors, CS in the first 100 days increases the chance of post-transplant EFS by-3.04 (-4.00 to-2.08) [hazard ratio (HR): 0.05 (p<0.001)]. Neutrophil engraftment was the other factor which was positively correlated with EFS (HR p-value: 0.05)CONCLUSION: There is a positive correlation between CS in the first 100 days and EFS for both malignant and non-malignant diseases.
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    Clinical Features and HSCT Outcome for SCID in Turkey
    (Springer/Plenum Publishers, 2019) Ikinciogullari, Aydan; Cagdas, Deniz; Dogu, Figen; Tugrul, Tuba; Karasu, Gulsum; Haskologlu, Sule; Aksoylar, Serap; Uygun, Vedat; Kupesiz, Alphan; Yildiran, Alisan; Gursel, Orhan; Ates, Can; Elhan, Atilla; Kansoy, Savas; Yesilipek, Akif; Tezcan, Ilhan
    Severe combined immunodeficiency (SCID) is the most serious PID, characterized by T cell lymphopenia and lack of antigen-specific T cell and B cell immune responses, inevitably leading to death within the first year of life if hematopoietic stem cell transplantation (HSCT) is not performed. Purpose and Methods Since SCID is a common type of PID with an estimated incidence of 1/10.000 in Turkey, a retrospective analysis of HSCT characteristics, survival, immune recovery, and the major clinical features of SCID prior to HSCT is the aim of this multi-transplant center-based analysis. Results A total of 234 SCID patients transplanted between the years 1994 and 2014 were included in the study. Median age at diagnosis was 5 months, at transplantation, 7 months, B- phenotype and RAGs were the most common defects among others. Immune phenotype did not seem to have an effect on survival rate (p > 0.05), Immunoglobulin (Ig) requirement following HSCT did not differ between B+ and B- phenotypes (p > 0.05). Overall survival rate was 65.7% over a period of 20 years. It increased from 54% (1994-2004) to 69% (p = 0.052) during the last 10 years (2005-2014). Ten-year survival after HSCT has improved over time although the difference was not significant. Infection at the time of transplantation (p = 0.006), mismatched related donor (MMRD) (haploidentical parents), and matched unrelated donor (MUD) donor transplants p < 0.001 were the most important factors, significantly affecting the outcome. Conclusions This is the first multicenter study with the largest data obtained from transplanted SCID patients in Turkey. Early diagnosis with newborn screening (NBS) together with emerging referrals, treatment by transplantation centers, and specialized teams are mandatory in countries with high parental consanguinity such as Turkey.
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    Çocukluk çağı beyin tümörlerinden medulloblastom ve ependimal tümorlerin oluşumunda etkili olduğu düşünülen onkogen ve tümör supresör genlerindeki delesyon ve duplikasyon durumlarının MLPA yöntemi ile araştırılması
    (Ege Üniversitesi, 2016) Aksoylar, Serap; Özkınay, Ferda
    Beyin tümörleri çocukluk çağının en sık görülen solid tümörüdür ve çocuk kanserlerinin yaklaşık olarak %20' sini oluşturmaktadır. En sık görülen tipi glial orijinli olanlar ve bunlar arasında astrositomalardır. Daha sonra ise medulloblastoma ve ependimal türrıörler gelir. Bu tümör grubunda hastalığın moleküler genetik nedenleri hala aydınlatılamamıştır. Tümör gelişiminde onkogen ve tumor supresör genlerin rolü bilinmektedir. MLPA yöntemi çok sayıda genin kopya tekrar sayısındaki (delesyon, duplikasyon) değişiklikleri tek seferde gösterebilen yeni bir moleküler genetik yöntemdir. Bu araştırmada; çocukluk çağı beyin tümörlerinden medulloblastoma ve ependimal tümörlerin oluşumunda etkili olduğu düşünülen onkogen ve tümör supresör genlerdeki moleküler değişikliklerin birçoğunun MLPA yöntemi ile aynı anda tümör dokusunda araştırılması amaçlanmıştır. Ege Üniversitesi Tıp Fakültesi'nde SSS tümörü tanısı almış 20 tane çocuk hastanın parafıne gömülü tümör dokusu ve kontrol olarak 10 normal beyin dokusu örnekleri, MLPA yöntemi ile 5 farklı gendeki delesyon ve duplikasyonlar açısından araştırıldı. Beş örnek yetersiz DNA kalitesi nedeniyle değerlendirilemedi. Geriye kalan 15 örneğin 2'sinde normal DNA, 13 tanesinde çeşitli delesyon ve duplikasyonlar saptandı. TP53 geninde delesyon ya da duplikasyon (6 örnek), EGFR geninde delesyon ya da duplikasyon (9 örnek), CDKN2A geninde duplikasyon (3 örnek), PTEN geninde duplikasyon veya delesyon (4 örnek), ERBB2 geninde duplikasyon (1 örnek) gösterildi. Sonuç olarak; bulunan genetik değişiklikler hem ependimom hemde medulloblastomda etkilenebileceğini bildiğimiz yolaklardadır ve gösterilmesi önmlidir. Bu spesifik hasta grubunda çok sayıda örnekle sonuçların doğrulanması önemlidir.;Childhood, ependimoma, medulloblastoma, MLPA, molecular characterisation.;Çocukluk çağı, Ependimom, Medulloblastom, MLPA, Moleküler özellikler.
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    Çocukluk çağı hastalıklarında kemik iliği ve kök hücre nakli sonuçları
    (2001) Kansoy, Savaş; Aksoylar, Serap; Kantar, Mehmet
    Kemik iliği Transplantasyonu (KİT), kemoterapötik ajanları çok yüksek dozlarda, çoğu zaman radyoterapi ile birlikte, nonhematolojik toksisiteler sınırında hastaya vermek ve böylece maksimal antitümör etki veya eradikasyonu sağlayıp, oluşan kemik iliği supresyonunu da allojenik, sinjeneik veya otolog koşullarda, transplantasyonla desteklemek esasına dayanan bir uygulamadır. Başlangıçta maliyn hastalıklarda uygulanan KİT'nun bir başka uygulama alanı ise bazı doğmalık hastalıklarda, Kİ hücrelerinin sağlıklı olanlarla değiştirilmesi olmaktadır. Bu çalışmada, pediatrik yaş grubundaki maliyn ve maliyn olmayan hastalıklara sahip çocuklarda, kemik iliği ve periferik kan kök hücre naklinden oluşan "Kök Hücre Nakli" uygulandı. Çalışma, Sosyal Sigortalar Kurumu (SSK) Tepecik Eğitim Hastanesi ve Ege Üniversitesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı Çocuk Onkoloji ve Kemik iliği Transplantasyonu Merkezleri'nde yapıldı. Kasım 1994 - Haziran 2000 yılları arasında toplam 38 olguya otolog veya allojenik transplantasyon uygulandı. Transplantasyon işlemi için maliyn hastalık gurubunda 9 Akut Myeloblastik Lösemi (AML), 5 Akut Lenfoblastik Lösemi (ALL), 3 Kronik Myelositer Lösemi, 3 Hodgkin Hastalığı (HD) ve 5 Solid Tümör olgusu, maliyn olmayan grupta ise 10 Talasemi Majör (TM), bir Aplastik Anemi (AA)'Iİ ve 2 ağır kombine yetmezliği (SCID) olan hastalar seçildi. Üç TM'lu ve bir HD'lı olguya iki kez allojenik veya otolog TX (ikincil Transplantasyon / Second TX) uygulandı. Olguların 20'sı (%52.6) kız, 18'i (%47.4) erkek, ilk tanı anında yaş ortalaması 94.1±67.3 ay (3 - 200), ortanca değer 113.5 ay idi. Transplant zamanındaki yaş ortalaması 119.2 ± 55.7 (8 - 207), ortanca değer 125.0 ay idi. Otolog transplantasyon grubunda 16 olguya periferik kök hücre (OPKH) uygulandı. Allojenik transplantasyon gurubunda toplam 22 olgunun 16'sına kemik iliği transplantasyonu (AKİT), 6 hastaya ise periferik kök hücre nakli (APKH) yapıldı. Allojenik transplantasyonların biri hariç tümünde HLA doku gurupları tam uygun olan kardeş donörler seçildi. Tüm olgular birlikte değerlendirildiğinde; TX yaşı 119.3 ± 55.7 ay (8 ay - 17 yaş), Kök hücre miktarı ortanca 4.46 x 108 (1.50 - 13.2), engraftment günü ortalama 16.4 ± 5.3 gün (7 - 28 gün), lökosit düzelmesi 22.3 ± 17.9 gün (12 - 66 gün), platelet düzelmesi ortalama 26.9 ± 27.3 gün (13 - 67 gün), Post TX DFS 30.7±18.1 ay (5 - 66 ay), Post TX OS ortalama 60.5 ± 52.2 ay (4.5 - 238 ay) bulundu. Sonuç olarak, maliyn ve bazı maliyn olmayan hastalıklarda "Kemik iliği" ve "Kan Kök Hücre" nakilleri ile kür şansı elde edilebilmektedir. Maliyn hastalığı olanlar primer hastalık ile beraber kemoterapi ve radyoterapinin erken ve geç etkilerinden uzaklaşmaktadır. Bazı doğmalık kan hastalıklarında, hasta transfüzyon ihtiyacından kurtulmakta ve geç endokrin etkilere maruz kalmamakta, normal bir yaşam süresine kavuşabilmektedir.
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    Combined cord blood and bone marrow transplantation from the same human leucocyte antigen-identical sibling donor for children with malignant and non-malignant diseases
    (Wiley-Blackwell, 2015) Tucunduva, Luciana; Volt, Fernanda; Cunha, Renato; Locatelli, Franco; Zecca, Marco; Yesilipek, Akif; Caniglia, Maurizio; Guengoer, Tayfun; Aksoylar, Serap; Fagioli, Franca; Bertrand, Yves; Addari, Maria Carmen; de la Fuente, Josu; Winiarski, Jacek; Biondi, Andrea; Sengeloev, Henrik; Badell, Isabel; Mellgren, Karin; de Heredia, Cristina Diaz; Sedlacek, Petr; Vora, Ajay; Rocha, Vanderson; Ruggeri, Annalisa; Gluckman, Eliane
    Umbilical cord blood (UCB) from an human leucocyte antigen (HLA)-identical sibling can be used for transplantation of patients with malignant and non-malignant diseases. However, the low cellular content of most UCB units represents a limitation to this approach. An option to increase cell dose is to harvest bone marrow (BM) cells from the same donor and infuse them along with the UCB. We studied 156 children who received such a combined graft between 1992 and 2011. Median age was 7years and 78% of patients (n=122) were transplanted for non-malignant diseases, mainly haemoglobinopathies. Acute leukaemia (n=26) was the most frequent malignant diagnosis. Most patients (91%) received myeloablative conditioning. Median donor age was 17years, median infused nucleated cell dose was 244x10(7)/kg and median follow-up was 41months. Sixty-days neutrophil recovery occurred in 96% of patients at a median of 17d. The probabilities of grade-II-IV acute and chronic graft-versus-host disease (GVHD) were 19% and 10%, respectively. Four-year overall survival was 90% (68% malignant; 97% non-malignant diseases) with 3% probability of death. In conclusion, combined UCB and BM transplantation from an HLA-identical sibling donor is an effective treatment for children with malignant and non-malignant disorders with high overall survival and low incidence of GVHD.
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    Combined cord blood and bone marrow transplantation from the same human leucocyte antigen-identical sibling donor for children with malignant and non-malignant diseases
    (Wiley-Blackwell, 2015) Tucunduva, Luciana; Volt, Fernanda; Cunha, Renato; Locatelli, Franco; Zecca, Marco; Yesilipek, Akif; Caniglia, Maurizio; Guengoer, Tayfun; Aksoylar, Serap; Fagioli, Franca; Bertrand, Yves; Addari, Maria Carmen; de la Fuente, Josu; Winiarski, Jacek; Biondi, Andrea; Sengeloev, Henrik; Badell, Isabel; Mellgren, Karin; de Heredia, Cristina Diaz; Sedlacek, Petr; Vora, Ajay; Rocha, Vanderson; Ruggeri, Annalisa; Gluckman, Eliane
    Umbilical cord blood (UCB) from an human leucocyte antigen (HLA)-identical sibling can be used for transplantation of patients with malignant and non-malignant diseases. However, the low cellular content of most UCB units represents a limitation to this approach. An option to increase cell dose is to harvest bone marrow (BM) cells from the same donor and infuse them along with the UCB. We studied 156 children who received such a combined graft between 1992 and 2011. Median age was 7years and 78% of patients (n=122) were transplanted for non-malignant diseases, mainly haemoglobinopathies. Acute leukaemia (n=26) was the most frequent malignant diagnosis. Most patients (91%) received myeloablative conditioning. Median donor age was 17years, median infused nucleated cell dose was 244x10(7)/kg and median follow-up was 41months. Sixty-days neutrophil recovery occurred in 96% of patients at a median of 17d. The probabilities of grade-II-IV acute and chronic graft-versus-host disease (GVHD) were 19% and 10%, respectively. Four-year overall survival was 90% (68% malignant; 97% non-malignant diseases) with 3% probability of death. In conclusion, combined UCB and BM transplantation from an HLA-identical sibling donor is an effective treatment for children with malignant and non-malignant disorders with high overall survival and low incidence of GVHD.
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    Combined cord blood and bone marrow transplantation from the same human leucocyte antigen-identical sibling donor for children with malignant and non-malignant diseases
    (Wiley-Blackwell, 2015) Tucunduva, Luciana; Volt, Fernanda; Cunha, Renato; Locatelli, Franco; Zecca, Marco; Yesilipek, Akif; Caniglia, Maurizio; Guengoer, Tayfun; Aksoylar, Serap; Fagioli, Franca; Bertrand, Yves; Addari, Maria Carmen; de la Fuente, Josu; Winiarski, Jacek; Biondi, Andrea; Sengeloev, Henrik; Badell, Isabel; Mellgren, Karin; de Heredia, Cristina Diaz; Sedlacek, Petr; Vora, Ajay; Rocha, Vanderson; Ruggeri, Annalisa; Gluckman, Eliane
    Umbilical cord blood (UCB) from an human leucocyte antigen (HLA)-identical sibling can be used for transplantation of patients with malignant and non-malignant diseases. However, the low cellular content of most UCB units represents a limitation to this approach. An option to increase cell dose is to harvest bone marrow (BM) cells from the same donor and infuse them along with the UCB. We studied 156 children who received such a combined graft between 1992 and 2011. Median age was 7years and 78% of patients (n=122) were transplanted for non-malignant diseases, mainly haemoglobinopathies. Acute leukaemia (n=26) was the most frequent malignant diagnosis. Most patients (91%) received myeloablative conditioning. Median donor age was 17years, median infused nucleated cell dose was 244x10(7)/kg and median follow-up was 41months. Sixty-days neutrophil recovery occurred in 96% of patients at a median of 17d. The probabilities of grade-II-IV acute and chronic graft-versus-host disease (GVHD) were 19% and 10%, respectively. Four-year overall survival was 90% (68% malignant; 97% non-malignant diseases) with 3% probability of death. In conclusion, combined UCB and BM transplantation from an HLA-identical sibling donor is an effective treatment for children with malignant and non-malignant disorders with high overall survival and low incidence of GVHD.
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    Cutaneous Side Effects of Chemotherapy in Pediatric Oncology Patients
    (Quadrant Healthcom Inc, 2015) Ceylan, Can; Kantar, Mehmet; Tuna, Arzu; Ertam, Ilgen; Aksoylar, Serap; Gunaydin, Asli; Cetingul, Nazan
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    Cytoprotective effect of amifostine in the treatment of childhood malignancies
    (Wiley-Liss, 2007) Midvat, Levent; Cetingul, Nazan; Kantar, Mehmet; Demirao, Bengu; Aksoylar, Serap; Kansoy, Savap
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    Cytoprotective Effects of Amifostine in the Treatment of Childhood Malignancies
    (Wiley, 2009) Cetinguel, Nazan; Midyat, Levent; Kantar, Mehmet; Demirag, Bengue; Aksoylar, Serap; Kansoy, Savas
    Background. Multi-systemic acute side effects occur, in response to intensive therapies that have been applied in childhood malignancies in recent years. Amifostine has rarely been used in the childhood cancers as a multisystemic protective agent for minimizing these side effects. Procedure. In this Study, the effectiveness of amifostine in combination with chemotherapy for childhood cancer treatment has been researched. Of 11 Subjects (2.5 months-17 years) 4 Subjects had leukemia, 4 had solid tumor, and 3 1 ad lymphoma. For these 11 subjects, 29 chemotherapy Courses were given in combination with amifostine, and 20 without amifostine. Their hematological, gastrointestinal and hepatic toxicity were evaluated according to the WHO toxicity criteria. Amifostine was given intravenously in a dose of 740 mg/m(2), one to three consecutive days depending on the chemotherapy regimen. Results. The hemoglobin, leukocyte, and platelet levels of the two groups were not statistically different. However, when comparing the Courses of the patients receiving the same medications at the same closes, in the group with amifostine, mean erythrocyte transfusion requirement was significantly reduced (P=0.025). In 31%, of the Courses with amifostine and 50% of the Courses Without amifostine, febrile neutropenia developed. Gastrointestinal system and hepatic toxicity Was Significantly reduced in the Courses with amifostine with respect to those without it (P=0.001). Vomiting, hypotension and nausea were the only side effects related to amifostine. Conclusion. Use of amifostine during the treatment of childhood cancers with intensive chemotherapy and/or radiotherapy significantly reduced the erythrocyte transfusion requirements of the patients as well as gastrointestinal and hepatic toxicity. Pediatr Blood Cancel 2009;52:829-833. (C) 2009 Wiley-Liss, Inc.
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    Determination of School-Related Problems in Children Treated for Cancer
    (Sage Publications Inc, 2014) Yilmaz, Medine C.; Sari, Hatice Yildirim; Cetingul, Nazan; Kantar, Mehmet; Erermis, Serpil; Aksoylar, Serap
    This descriptive and case-control study was carried out in a pediatric oncology outpatient clinic to determine the school-related physical, social, and psychological problems and problems experienced in academic achievement of children treated for cancer. The sample of the study consisted of 56 Turkish patients with cancer, aged 7-18 years, who were in remission and attending school as well as their parents, a control group of patients who did not have cancer, and their teachers. A Child Information Form, a Child Health Questionnaire Parent's Form of 50 questions, a Behavior Evaluation Scale for Children, and Young People and a Teacher's Report Form were used as data collection tools in the study. Of the children, 30.3% experienced various physical difficulties stemming from cancer therapy that affected their school life. The number of late enrollments, the number of children repeating a grade, and the rates of school absenteeism were also found to be higher in the survivors than in the controls.
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    Different Kinetics and Risk Factors for Isolated Extramedullary Relapse after Allogeneic Hematopoietic Stem Cell Transplantation in Children with Acute Leukemia
    (Elsevier Science Inc, 2021) Hazar, Volkan; Ozturk, Gulyuz; Yalcin, Koray; Uygun, Vedat; Aksoylar, Serap; Kupesiz, A.; Bozkaya, Ikbal Ok
    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. (C) 2021 The American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. All rights reserved.
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    Diverse phenotypic and genotypic presentation of RAG1 mutations in two cases with SCID
    (Springer, 2009) Karaca, Neslihan Edeer; Aksu, Guzide; Genel, Ferah; Gulez, Nesrin; Can, Sema; Aydinok, Yesim; Aksoylar, Serap; Karaca, Emin; Altuglu, Imren; Kutukculer, Necil
    Severe combined immunodeficiencies (SCID) comprise a spectrum of genetic defects that involve both humoral and cellular immunities. Defects in recombinating activating gene 1 (RAG1), RAG2, Artemis, or LIG4 can disrupt V(D)J recombination. Defective V(D)J recombination of the T and B cell receptors is responsible for T(-)B(-)NK(+)SCID. Amorphic mutations in RAG1 and RAG2 cause T(-)B(-)NK(+)SCID, whereas hypomorphic mutations cause an immunodeficency characterized by oligoclonal expansion of TCR gamma delta T cells, severe CMV infection and autoimmunity. First patient is a typical T(-)B(-)NK(+)SCID with clinical and immunologic findings while the second is atypical with normal immunoglobulin levels, CD4 lymphopenia, elevated TCR gamma delta T cells, persistent CMV infection, and autoimmune hemolytic anemia. These cases are presented to emphasize that mutations in RAG1 gene may lead to a diverse spectrum of clinical and immunologic findings while hypomorphic mutations may be related with autoimmunity and refractory CMV infection during infancy.