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Öğe THE ASSOCIATION BETWEEN HLA CLASS I AND II ALLELES AND THE OCCURRENCE OF INHIBITORS IN TURKISH PATIENTS WITH HEMOPHILIA A: A PILOT STUDY(Wiley, 2019) Patiroglu, Turkan; Cansever, Murat; Akbayram, Sinan; Gulen, Huseyin; Oncel, Kahraman; Borst, Ozcan; Oymak, Yesim; Aral, Yusuf Ziya; Ay, Yilmaz; Kilinc, Yurdanur; Oren, Hale; Kavakli, KaanÖğe The effect of virtual reality on pain, fear, and anxiety during access of a port with huber needle in pediatric hematology-oncology patients: Randomized controlled trial(Elsevier Sci Ltd, 2021) Gerceker, Gulcin Ozalp; Bektas, Murat; Aydinok, Yesim; Oren, Hale; Ellidokuz, Hulya; Olgun, NurPurpose: Port needle insertions are painful and distressing for Pediatric Hematology-Oncology patients. Virtual Reality (VR) can be used during needle-related procedures in these patients. This study aimed to investigate the effect of VR distraction during access to the venous port with a Huber needle in reducing needle-related pain, fear, and anxiety of children and adolescents with cancer. Methods: This randomized controlled study used a parallel trial design guided by the CONSORT checklist. The sample of children (n = 42) was allocated to the VR group (n = 21) and the control group (n = 21). Port needle related pain was assessed using the Wong-Baker Faces Pain Rating Scale after the procedure. Before and after the port needle insertion procedure, anxiety and fear assessed using self-and parent-report using the Children & rsquo;s Anxiety Meter and Child Fear Scale. The primary outcome was the patient-reported pain scores after the procedure and fear and anxiety scores before and after the procedure. Pain, anxiety, and fear scores of the two groups and within groups were analyzed and also Spearman correlation analysis was used. Results: Self-reported pain scores of patients in the VR and control group were 2.4 +/- 1.8 and 5.3 +/- 1.8, respectively. This study found a statistically significant difference between groups in pain scores (p < .001). A statistically significant difference was found between groups according to the self-and parent-reported fear and anxiety scores after the procedure. Self-reported fear scores in the VR and control group were 0.8 +/- 0.9, 2.0 +/- 1.0, self-reported anxiety scores were 2.9 +/- 2.0, 5.4 +/- 2.0, respectively (p < .001). Conclusion: Virtual reality is an effective distraction method in reducing port needle-related pain, fear, and anxiety in Pediatric Hematology-Oncology patients. ClinicalTrials.gov NCT04093154.Öğe Influence of Paroxysmal Nocturnal Hemoglobinuria Clone Positivity on Outcome of Childhood Acquired Aplastic Anemia: A Multicenter Center Study(Amer Soc Hematology, 2018) Cangul, Sule Unal; Karapinar, Deniz Yilmaz; Erdem, Arzu Yazal; Yarali, Husniye Nese; Ozdemir, Hamiyet Hekimci; Gumruk, Fatma; Cakmakli, Hasan Fatih; Ince, Elif Unal; Ozdemir, Gul Nihal Nihal; Gokce, Muge; Celkan, Tiraje; Bahadir, Aysenur; Bayhan, Turan; Oren, Hale; Gulen, Huseyin; Kupesiz, Funda Tayfun; Cetin, Mualla; Ozbek, NamikÖğe The investigation of relationship between joint findings and serum angiogenic and inflammatory factor levels in severe hemophilia A patients(Lippincott Williams & Wilkins, 2014) Karapinar, Tuba H.; Karadas, Nihal; Ozek, Gulcihan; Tufekci, Ozlem; Atabay, Berna; Turker, Meral; Yuksel, Faize; Karapinar, Deniz Y.; Vergin, Canan; Irken, Gulersu; Oren, HaleDespite the use of primary prophylactic Factor VIII replacement in severe hemophilia A patients, bleeding into joints cannot be prevented completely and early diagnosis and treatment of the joint bleedings are important for prevention of permanent joint damage. Recent studies have shown that neoangiogenesis plays important role in development of synovitis after recurrent joint bleedings. This study aimed to investigate the relationship between joint findings and levels of serum angiogenic and inflammatory factors in severe hemophilia A patients.The patient groups consisted of 10 severe hemophilia A patients with acute joint bleeding and 25 severe hemophilia A patients without acute joint bleeding. They were all inhibitor negative. The control group consisted of 22 healthy male children. Complete blood cell count analysis, C-reactive protein (CRP), serum ferritin, lactic acid, and ELISA-based detection of vascular endothelial growth factor (VEGF), intercellular adhesion molecule-1, thrombomodulin, macrophage migration inhibitory factor (Min, and endostatin were performed from peripheral blood of patient and the control groups. CRP and M IF levels were detected significantly higher in hemophilia patients with acute joint bleeding than patients without acute joint bleeding. There was a positive correlation between serum thrombomodulin, VEGF, and MIF levels. In this study, we demonstrated that serum CRP and MIF levels increases in acute bleeding period regardless of the presence of previous joint damage in children with severe hemophilia. CRP elevation may be a useful and rapid marker for acute bleeding in these patients. Blood Coagul Fibrinolysis 25:703-708 2014 (C) Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.Öğe Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients(Galenos Yayincilik, 2018) Tufekci, Ozlem; Kocak, Ulker; Kaya, Zuhre; Yenicesu, Idil; Albayrak, Canan; Albayrak, Davut; Bengoa, Sebnem Yilmaz; Patiroglu, Turkan; Karakukcu, Musa; Unal, Ekrem; Ince, Elif Unal; Ileri, Talia; Ertem, Mehmet; Celkan, Tiraje; Ozdemir, Gul Nihal; Sarper, Nazan; Kacar, Dilek; Yarali, Nese; Ozbek, Namik Yasar; Kupesiz, Alphan; Karapinar, Tuba; Vergin, Canan; Caliskan, Umran; Tokgoz, Huseyin; Evim, Melike Sezgin; Baytan, Birol; Gunes, Adalet Meral; Karapinar, Deniz Yilmaz; Karaman, Serap; Uygun, Vedat; Karasu, Gulsun; Yesilipek, Mehmet Akif; Koc, Ahmet; Erduran, Erol; Atabay, Berna; Oniz, Haldun; Oren, HaleObjective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x10(9)/L, 5.4x10(9)/L, and 58.3x10(9)/L, respectively. Monosomy 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 30 +/- 17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x10(9)/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results are not satisfactory and it is necessary to put more effort into this issue in Turkey.Öğe Long-term Dental Anomalies after Pediatric Cancer Treatment in Children(Galenos Yayincilik, 2019) Kilinc, Gulser; Bulut, Gulcin; Ertugrul, Fahinur; Oren, Hale; Demirag, Bengu; Demiral, Ayse; Aksoylar, Serap; Kernel, Emine Serra; Ellidokuz, Hulya; Olgun, NurObjective: The aim of this study is to determine the frequency of dental anomalies (DAs) (microdontia, hypodontia, hyperdontia, enamel defect, root malformation) in pediatric cancer patients at the ages <5 years and between 5 and 7 years, and understand their relationship with the received therapy. Materials and Methods: Pediatric patients who were diagnosed with cancer and treated before the age of 7 years were investigated in a case-control design. The study included 93 pediatric patients whose ages at diagnosis were between 9 months and 7 years and whose treatments were completed before 5-8 years. Group A consisted of patients in the age range of 9 months to 4 years and Group B consisted of patients in the age range of 5-7 years. Seventy-two siblings with compatible dental age ranges were included in the control group. For both groups, intraoral examinations were performed and panoramic radiographs were taken. Results: Among the 93 pediatric patients, the mean age was 9.54 +/- 1.25 (range: 8-13 years) and 48 (51.6%) patients were male. The most common diagnosis was hematologic malignancy with a rate of 65.5%. At least one DA was detected in 7 (9.7%) individuals of the control group and in 78 (83.9%) of the patient group. While the patients in the study group had all kinds of DAs, those in the control group had only enamel defects. The rates of microdontia (p=0.077) and hypodontia (p=0.058) were detected to be significantly higher in Group A than in Group B. Root malformation was more common in patients receiving chemotherapy and radiotherapy than in those receiving only chemotherapy (p=0.006). Conclusion: In this study it was found that the pediatric patients who received cancer treatment before the age of 7 years constituted a high-risk group for DAs. The frequencies of microdontia and hypodontia were increased even more when the patient was treated for cancer before 5 years of age.Öğe Prognostic Factors and a New Prognostic Index Model for Children and Adolescents with Hodgkin's Lymphoma Who Underwent Autologous Hematopoietic Stem Cell Transplantation: A Multicenter Study of the Turkish Pediatric Bone Marrow Transplantation Study Group(Galenos Yayincilik, 2016) Kesik, Vural; Atas, Erman; Karakukcu, Musa; Aksoylar, Serap; Erbey, Fatih; Tacyildiz, Nurdan; Kupesiz, Alphan; Oniz, Haldun; Unal, Ekrem; Kansoy, Savas; Ozturk, Guluz; Elli, Murat; Kaya, Zuhre; Unal, Emel; Hazar, Volkan; Bengoa, Sebnem Yilmaz; Karasu, Gulsun; Atay, Didem; Dagdemir, Ayhan; Oren, Hale; Kocak, Ulker; Yesilipek, M. AkifObjective: The prognostic factors and a new childhood prognostic index after autologous hematopoietic stem cell transplantation (AHSCT) in patients with relapsed/refractory Hodgkin's lymphoma (HL) were evaluated. Materials and Methods: The prognostic factors of 61 patients who underwent AHSCT between January 1990 and December 2014 were evaluated. In addition, the Age-Adjusted International Prognostic Index and the Childhood International Prognostic Index (CIPI) were evaluated for their impact on prognosis. Results: The median age of the 61 patients was 14.8 years (minimum-maximum: 5-20 years) at the time of AHSCT. There were single relapses in 28 patients, >= 2 relapses in eight patients, and refractory disease in 25 patients. The chemosensitivity/chemorefractory ratio was 36/25. No pretransplant radiotherapy, no remission at the time of transplantation, posttransplant white blood cell count over 10x10(3)/mu L, posttransplant positron emission tomography positivity at day 100, and serum albumin of < 2.5 g/dL at diagnosis were correlated with progression-free survival. No remission at the time of transplantation, bone marrow positivity at diagnosis, and relapse after AHSCT were significant parameters for overall survival. Conclusion: The major factors affecting the progression-free and overall survival were clearly demonstrated. A CIPI that uses a lactate dehydrogenase level of 500 IU/L worked well for estimating the prognosis. We recommend AHSCT at first complete remission for relapsed cases, and it should also be taken into consideration for patients with high prognostic scores at diagnosis.Öğe Rare Coagulation Disorders: A Retrospective Analysis of 156 Patients in Turkey(Galenos Yayincilik, 2012) Fisgin, Tunc; Balkan, Can; Celkan, Tiraje; Kilinc, Yurdanur; Turker, Meral; Timur, Cetin; Gursel, Turkiz; Kurekci, Emin; Duru, Feride; Kupesiz, Alphan; Olcay, Lale; Yilmaz, Sebnem; Ozgen, Unsal; Unuvar, Aysegul; Oren, Hale; Kavakli, KaanObjective: To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group of Turkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009. Material and Methods: The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasis subcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participating institutions. Results: In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDs were as follows: FVII (n = 53 [34%]), FY (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency The most common initial finding in the patients was epistaxis, followed by ecchymosis, and gingival bleeding. Conclusion: Initial symptoms were mucosal bleeding, and fresh frozen plasma (FFP) and tranexamic acid were the most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should be considered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and for those with a factor deficiency that can lead to severe bleeding.Öğe Timing of Central Venous Line Insertion During Induction in Children With Acute Lymphoblastic Leukemia(Lippincott Williams & Wilkins, 2023) Ataseven, Eda; Yilmaz, Sebnem; Tufekci, Ozlem; Oren, HalePurpose:Central venous lines (CVL) in children with acute lymphoblastic leukemia (ALL) provide comfortable administration of intensive chemotherapy and blood sampling. The optimal time for the insertion of CVL in patients with ALL during induction therapy is controversial. This study aimed to investigate the frequency of CVL-related complications in children with ALL concerning the time of CVL insertion. Patients and Methods:We reviewed the records of 52 pediatric ALL patients with CVL. CVL placement before or on treatment day 15 was defined as early insertion, and after treatment day 15 was defined as late insertion. Demographics, preoperative blood counts, type of central line, time of CVL placement, CVL-related complications, and blood counts during complications were all noted. All the data were collected from those with the first catheter use. Results:CVL was placed <= 15 days in 26 patients (50%) and after 15 days in 26 patients (50%). Regarding the infection rates, no statistical difference was found between early and late CVL-inserted groups (P=n.s.). Five patients developed thrombosis, and risk was found to be similar between early and late CVL-inserted groups (P=n.s.). Catheter-related mechanical complications were recorded in 7 patients (3 in early and 4 in late CVL-inserted group, (P=n.s.). Conclusion:The present study showed no relation between the timing of CVL placement during induction therapy and the occurrence of infection and thrombosis. Our results suggest that CVL can be placed safely at the time of diagnosis or early induction treatment to provide a comfortable administration of chemotherapy and decrease painful blood samplings.
